Jo Shapiro, MD, FACS, Steven M. Steinberg, MD, FACS, and Wiley W. Souba MD, ScD, MBA, FACS
Over the past decade, the American health care system has had to cope with and manage an unprecedented amount of change. As
a consequence, the medical profession has been challenged along the entire range of its cultural values and its traditional
roles and responsibilities. It would be difficult, if not impossible, to find another social issue directly affecting all
Americans that has undergone as rapid and remarkable a transformation—and oddly, a transformation in which the most important
protagonists (i.e., the patients and the doctors) remain dissatisfied.1
Nowhere is this metamorphosis more evident than in the field of surgery. Marked reductions in reimbursement, explosions in
surgical device biotechnology, a national medical malpractice crisis, and the disturbing emphasis on commercialized medicine
have forever changed the surgical landscape, or so it seems. The very foundation of patient care—the doctor-patient relationship—is
in jeopardy. Surgeons find it increasingly difficult to meet their responsibilities to patients and to society as a whole.
In these circumstances, it is critical for us to reaffirm our commitment to the fundamental and universal principles and values
of medical professionalism.
The concept of medicine as a profession grounded in compassion and sympathy for the sick has come under serious challenge.2 One eroding force has been the growth and sovereignty of biomedical research. Given the high position of science and technology
in our societal hierarchy, we may be headed for a form of medicine that includes little caring but becomes exclusively focused
on the mechanics of treatment, so that we deal with sick patients much as we would a flat tire or a leaky faucet. In such
a form of medicine, healing becomes little more than a technical exercise, and any talk of morality that is unsubstantiated
by hard facts is considered mere opinion and therefore carries little weight.
The rise of entrepreneurialism and the growing corporatization of medicine also challenge the traditions of virtue-based medical
care. When these processes are allowed to dominate medicine, health care becomes a commodity. As Pellegrino and Thomasma remark,
“When economics and entrepreneurism drive the professions, they admit only self-interest and the working of the marketplace
as the motives for professional activity. In a free-market economy, effacement of self-interest, or any conduct shaped primarily
by the idea of altruism or virtue, is simply inconsistent with survival.”2
Another profound change in the practice of medicine is the shift from a largely autonomous focus, with the surgeon both shouldering
tremendous personal responsibility and wielding considerable control and independence, to a complex, team-based focus. Within
this new paradigm, leadership of a surgical team requires vastly different competencies than were previously required or even
valued. Many surgeons have flourished in this new environment: leading a cohesive team dedicated to excellent outcomes is
highly rewarding. Unfortunately, however, as a profession, we have not explicitly taught these teamwork and leadership skills,
nor have we always helped our colleagues remediate deficiencies in such skills.
The Meaning of Professionalism
Professionalism is the basis of our contract with society. A profession is a collegial discipline that regulates itself by
means of mandatory, systematic training. It has a base in a body of technical and specialized knowledge that it both teaches
and advances; it sets and enforces its own standards; and it has a service orientation, rather than a profit orientation,
enshrined in a code of ethics.3–5 To put it more succinctly, a profession has cognitive, collegial, and moral attributes. These qualities are well expressed
in the familiar sentence from the Hippocratic oath: “I will practice my art with purity and holiness and for the benefit of
the sick.”
Historically, the legitimacy of medical authority is based on three distinct claims2,6: first, that the knowledge and competence of the professional have been validated by a community of peers; second, that this
knowledge has a scientific basis; and third, that the professional's judgment and advice are oriented toward a set of values.
These aspects of legitimacy correspond to the collegial, cognitive, and moral attributes that define a profession.
The American College of Surgeons (ACS) Task Force on Professionalism has developed a Code of Professional Conduct,7 which emphasizes the following four aspects of professionalism:
1. A competent surgeon is more than a competent technician.
2. Whereas ethical practice and professionalism are closely related, professionalism also incorporates surgeons' relationships
with patients and society.
3. Unprofessional behavior must have consequences.
4. Professional organizations are responsible for fostering professionalism in their membership.
Specifically, the ACS Code of Professional Conduct includes tenets of professionalism that relate to both our care of individual
patients and our role in society [see Table 1].
The Accreditation Council on Graduate Medical Education (ACGME) has identified six competencies that must be demonstrated
by the surgeon: (1) patient care; (2) medical knowledge; (3) practice-based learning and improvement; (4) interpersonal and
communication skills; (5) professionalism; and (6) systems-based practice. These competencies are now being integrated into
the training programs of all accredited surgical residencies.
Being a professional demands unwavering personal integrity and a commitment to lifelong learning and improvement. It places
the responsibility to serve (care for) others above self-interest and reward [see Elizabeth Blackwell: A Model of Professionalism8].
Regrettably, examples of unprofessional behavior exist. An excerpt from a note from a third-year medical student to the core
clerkship director reads as follows: “I have seen attendings make sexist, racist jokes or remarks during surgery. I have met
residents who joke about deaf patients and female patients with facial hair. [I have encountered] teams joking and counting
down the days until patients die” (personal communication, 2004). This kind of exposure to unprofessional conduct and language
can influence young people negatively, and it must change.
Most of us went to medical school because we wanted to help and care for people who are ill. This genuine desire to care is
unambiguously apparent in the vast majority of personal statements that medical students prepare as part of their application
process. To quote William Osler, “You are in this profession as a calling, not as a business; as a calling which extracts
from you at every turn self-sacrifice, devotion, love and tenderness to your fellow man. We must work in the missionary spirit
with a breath of charity that raises you far above the petty jealousies of life.”9 To keep medicine a calling, we must explicitly incorporate into the meaning of professionalism those nontechnical practices,
habits, and attributes that the compassionate, caring, and competent physician exemplifies. We must remind ourselves that
a true professional places service to the patient above self-interest and above reward.
One of the core tenets of professionalism is being consistently respectful not only toward our patients and their families
but also toward our colleagues and other health care team members. Although no one would argue with this in principle, there
are many factors that challenge our ability to hold true to this precept, including poor interpersonal communication skills,
lack of training in conflict resolution, resource constraints, and cultural tradition.
Given that professionalism is so multifaceted, it is not surprising that various important professional values may conflict
with one another, creating tension sometimes internally and at other times between various groups. For example, the patient
may want a specific treatment that is not yet supported by evidence but may be of benefit. Meeting the patient's expectations
and needs may directly conflict with the expectation that we are advocates for “efficient distribution of health care resources.”
Another direct challenge to several professionalism obligations is trying to balance the important adherence to the duty hours
mandate with the equally important value of continuity of care.10
The underpinning of medicine as a compassionate, caring profession is the doctor-patient relationship, a relationship that
has become jeopardized and sometimes fractured over the past decade. Our individual perceptions of what this relationship
is and how it should work will inevitably have a great impact on how we approach the care of our patients.2
The view of the physician-patient relationship as a covenant does not demand devotion to medicine to the exclusion of other
responsibilities and is not inconsistent with the fact that medicine is also a science, an art, and a business.2 Nevertheless, in our struggle to remain viable in a health care environment that has become a commercial enterprise, efforts
to preserve market share cannot take precedence over the provision of care that is grounded in charity and compassion. It
is exactly for this reason that medicine always will be, and should be, a relationship between people. To fracture that relationship
by exchanging a covenant based on charity and compassion for a contract based solely on the delivery of goods and services
is something none of us would want for ourselves. The nature of the healing relationship is itself the foundation of the special
obligations of physicians as physicians.2
Translation of Theory into Practice
It is encouraging to note that many instances of unprofessional conduct that once were routinely overlooked—such as mistreating
medical students, speaking disrespectfully to coworkers, and fraudulent behavior—are now being dealt with. Still, from time
to time, an incident is made public that makes us all feel shame. In March 2003, the Seattle Times carried a story about the chief of neurosurgery at the University of Washington, who pleaded guilty to a felony charge of
obstructing the government's investigation and admitted that he asked others to lie for him and created an atmosphere of fear
in the neurosurgery department.11 According to the US Attorney in Seattle, University of Washington employees destroyed reports revealing that university doctors
submitted inflated billings to Medicare and Medicaid. The department chair lost his job, was barred from participation in
Medicare, and, as part of his plea bargain, had to pay a $500,000 fine, perform 1,000 hours of community service, and write
an article in a medical journal about billing errors. The university spent many millions in legal fees and eventually settled
the billing issues with the federal government for one of the highest Physicians at Teaching Hospitals (PATH) settlements
ever.
Fortunately, such extreme cases of unprofessionalism are quite uncommon. Nevertheless, there are numerous reports in the literature
of other types of unprofessional behavior, such as disruptive behavior,7 that can lead to patient safety risks.12–14 To this end, The Joint Commission has mandated that all of our hospitals have zero tolerance for disruptive behavior. Regardless
of the practice setting, it remains our responsibility as professionals to prevent such behaviors from developing and from
being reinforced. A study published in 2004 demonstrated an association between displays of unprofessional behavior in medical
school and subsequent disciplinary action by a state medical board.15 The authors concluded that professionalism is an essential competency that students must demonstrate to graduate from medical
school. Who could disagree? Yet we know that throughout our careers, there will be challenges, both personal and external,
to our consistently behaving professionally.
In addition to the reports recounting acts of unprofessional behavior, various publications describing methods of teaching
and assessing professionalism have begun to appear in the past few years.16,17 As an example, Kumar and colleagues found that using ACS case-based multimedia materials enhanced the ability of residents
to recognize and discuss matters related to professional behavior.18 Surgical residents who viewed these materials scored higher on an assessment tool than did residents with the same level
of experience who did not use the materials. An additional encouraging finding was that residents of all years were able to
define the components of professionalism. In another publication, Gauger and colleagues described an evaluation instrument
used to evaluate residents with respect to the aspects of professionalism.19 They divided the concept of professionalism into 15 domains and modified a standard resident evaluation form to assess the
faculty's perception of resident performance in each domain. This evaluation tool proved to be internally consistent, but
in the absence of any other gold standard tools with which to compare it, its validity could not be determined.
Hickson developed a system to monitor patient complaint data and use this to improve the behavior of individual physicians.20 Others have developed 360° assessment tools to give feedback to physicians regarding how their professionalism skills are
perceived by their team members.21
Assessment tools and formal courses alone are not enough to support the significant adaptive challenge22 that is involved in maintaining a culture of professionalism.10 Several authors have argued for a nuanced approach to supporting professional behaviors—one that acknowledges that professionalism
is not a fixed trait but rather a set of developmental skills that need to be continuously taught, role-modeled, and reflected
on throughout one's career.23 Understanding that professional behavior is contextual and situation dependent is crucial to developing programs to support
professionalism.
Elizabeth Blackwell: A Model of Professionalism
Elizabeth Blackwell was born in England in 1821, the daughter of a sugar refiner. When she was 10 years old, her family emigrated
to New York City. Discovering in herself a strong desire to practice medicine and care for the underserved, she took up residence
in a physician's household, using her time there to study using books in the family's medical library.
As a young woman, Blackwell applied to several prominent medical schools but was snubbed by all of them. After 29 rejections,
she sent her second round of applications to smaller colleges, including Geneva College in New York. She was accepted at Geneva—according
to an anecdote, because the faculty put the matter to a student vote, and the students thought her application a hoax. She
braved the prejudice of some of the professors and students to complete her training, eventually ranking first in her class.
On January 23, 1849, at the age of 27, Elizabeth Blackwell became the first woman to earn a medical degree in the United States.
Her goal was to become a surgeon.
After several months in Pennsylvania, during which time she became a naturalized citizen of the United States, Blackwell traveled
to Paris, where she hoped to study with one of the leading French surgeons. Denied access to Parisian hospitals because of
her gender, she enrolled instead at La Maternité, a highly regarded midwifery school, in the summer of 1849. While attending
to a child some 4 months after enrolling, Blackwell inadvertently spattered some pus from the child's eyes into her own left
eye. The child was infected with gonorrhea, and Blackwell contracted a severe case of ophthalmia neonatorum, which later necessitated
the removal of the infected eye. Although the loss of an eye made it impossible for her to become a surgeon, it did not dampen
her passion for becoming a practicing physician.
By mid-1851, when Blackwell returned to the United States, she was well prepared for private practice. However, no male doctor
would even consider the idea of a female associate, no matter how well trained. Barred from practice in most hospitals, Blackwell
founded her own infirmary, the New York Infirmary for Indigent Women and Children, in 1857. When the American Civil War began,
Blackwell trained nurses, and in 1868 she founded a women's medical college at the Infirmary so that women could be formally
trained as physicians. In 1869, she returned to England and, with Florence Nightingale, opened the Women's Medical College.
Blackwell taught at the newly created London School of Medicine for Women and became the first female physician in the United
Kingdom Medical Register. She set up a private practice in her own home, where she saw women and children, many of whom were
of lesser means and were unable to pay. In addition, Blackwell mentored other women who subsequently pursued careers in medicine.
She retired at the age of 86.
In short, Elizabeth Blackwell embodied professionalism in her work. In 1889 she wrote, "There is no career nobler than that
of the physician. The progress and welfare of society is more intimately bound up with the prevailing tone and influence of
the medical profession than with the status of any other class."
The Future of Surgical Professionalism
It is often subtly implied—or even candidly stated—that no matter how well we adjust to the changing health care environment,
the practice of surgery will never again be quite as rewarding as it once was. This need not be the case. The ongoing advances
in surgical technology, the increasing opportunities for community-based surgeons to enrol their patients into clinical trials,
the growing emphasis on lifelong learning, and the increased focus on teamwork and shared responsibility are factors that
not only help satisfy social and organizational demands for quality care but also are in the best interest of our patients.
In the near future, maintenance of certification for surgeons will involve much more than taking an examination every decade.
The ACS is taking the lead in helping to develop new measures of competence. Whatever specific form such measures may take,
displaying professionalism and living up to a set of uncompromisable core values24 will always be central indicators of the performance of the individual surgeon and the integrity of the discipline of surgery
as a whole.
Although surgeons vary enormously with respect to personality, practice preferences, areas of specialization, and style of
relating to others, they all have one role in common: that of healer. Indeed, it is the highest of privileges to be able to
care for the sick. As the playwright Howard Sackler once wrote, “To intervene, even briefly, between our fellow creatures
and their suffering or death, is our most authentic answer to the question of our humanity.”25 Inseparable from this privilege is a set of responsibilities that are not to be taken lightly: a pledge to offer our patients
the best care possible and a commitment to teach and advance the science and practice of medicine. Commitment to the practice
of patient-centered, high-quality, cost-effective care is what gives our work meaning and provides us with a sense of purpose.26 As surgeons, we must participate actively in the current evolution of integrated health care. By doing so, we help build
our own future.
Jo Shapiro, MD, FACS, or their significant other, has disclosed a potential conflict of interest as follows: grants and/or
salary by Health Dialog
References
1. Fein R. The HMO revolution. Dissent 1998;Spring:29.
2. Pellegrino ED, Thomasma DC. Helping and healing. Washington (DC): Georgetown University Press; 1997.
3. Brandeis LD. Familiar medical quotations. In: Strauss M, editor. Business—a profession. Boston: Little Brown & Co; 1986.
p. 17.
4. Cogan ML. Toward a definition of profession. Harv Educ Rev 1953;23:33.
5. Greenwood E. Attributes of a profession. Social Work 1957;22:44.
6. Starr PD. The social transformation of American medicine. New York: Basic Books; 1982.
7. Gruen RI, Arya J, Cosgrove EM, et al. Professionalism in surgery. J Am Coll Surg 2003;197:605.
9. Hinohara S, Niki H, editors. Osler's “Way of Life” and other addresses, with commentary and annotations. Durham (NC): Duke
University Press; 2001.
10. Lucey C, Souba W. The problem with the problem of professionalism. Acad Med 2010;85:1018–24.
11. Ostrom CM. A neaurosurgeon ‘crisis’: Insurer drops doctors' group. The Seattle Times 2003 June 7.
12. Rosenstein AH, O' Daniel M. A survey of the impact of disruptive behaviors and communication defects on patient safety. Jt
Comm J Qual Patient Saf 2008;34:464–71.
13. Rosenstein AH, O'Daniel M. Disruptive behavior and clinical outcomes. Am J Nurs 2005;105:54–64.
14. The Joint Commission. Behaviors that undermine a culture of safety. Sentinel Event Alert 2008;40:1–3.
15. Papadakis M, Hodgson C, Teherani A, Kohatsu ND. Unprofessional behavior in medical school is associated with subsequent disciplinary
action by a state medical board. Acad Med 2004;79:244.
16. Papadakis MA, Osborn EH, Cooke M, Healy K. A strategy for the detection and evaluation of unprofessional behavior in medical
students. University of California, San Francisco School of Medicine Clinical Clerkships Operation Committee. Acad Med 1999;74:980–90.
17. Yao D, Wright S. National survey of internal medicine residency program directors regarding problem residents. JAMA 2000;284:1099–104.
18. Kumar A, Shibru D, Bullard K, et al. Case-based multimedia program enhances the maturation of surgical residents regarding
the concepts of professionalism. J Surg Educ 2007;64:194.
19. Gauger P, Gruppen L, Minter R, et al. Initial use of a novel instrument to measure professionalism in surgical residents.
Am J Surg 2005;189:479.
20. Hickson G, Pichert JW, Webb LE, Gabbe SG. A complementary approach to promoting professionalism: identifying, measuring and
addressing unprofessionalism behaviors. Acad Med 2007;82:1040–8.
21. Harmon L, Gregory P, Hiller N, Batista L. PULSE (Physicians Universal Leadership Skills Education Survey) technical report
executive summary. Physicians Development Program, Miami, FL; 2010 (Unpublished proprietary manuscript).
22. Heifetz R, Linsky M. Leadership on the line. Boston: Harvard Business Press; 2002.
23. Lesser C, Lucey C, Egener B, et al. A behavioral and systems view of professionalism. JAMA 2010;304:2732–7.
24. Souba W. Academic medicine's core values: what do they mean? J Surg Res 2003;115:171.
25. Rosenow EC. The art of living...the art of medicine: the wit and wisdom of life and medicine: a physician's perspective.
Victoria BC: Trafford; 2003. p. 128.
26. Souba W. Academic medicine and our search for meaning and purpose. Acad Med 2002;77:139.
Surgical care of neonates, infants, and children differs in many respects from that of adults.1 Accordingly, it is essential that surgeons caring for pediatric patients be capable of recognizing and managing certain clinical
problems that occur frequently in this population. To this end, this chapter begins by discussing several basic considerations
related to pediatric physiology, which is markedly different from adult physiology. With this general discussion as a background,
an overview of specific surgical problems commonly encountered in pediatric patients is provided.
Management of fluids and electrolytes in neonates and infants requires a thorough understanding of the changes in body fluid
compartments that occur during development [see Figure 1 ].2–7 At birth, total body water accounts for roughly 75% of total body weight (TBW), but this percentage decreases rapidly in
the first few days, falling slowly toward 60% over the first year. Similarly, extracellular fluid volume accounts for 45%
of TBW at birth but only 20% by the end of the first year. Newborns have lower glomerular filtration rates and reduced renal
concentrating ability8, 9; consequently, they tolerate dehydration poorly and cannot excrete a water load as effectively as older persons with mature
kidneys can. For this reason, adequate fluid management is especially challenging in these patients.
Measurement of urine output is a useful guide to fluid management provided that accurate urine collections can be obtained.
In critically ill infants and children, a urinary catheter should be inserted to ensure accurate urine collections. Catheterization
of male neonates and infants carries a significant risk of trauma to the small urethra; in these patients, the use of properly
secured collection bags may allow accurate measurement of urine output without the need for catheter insertion. An appropriate
urine output is 1 to 2 mL/kg/hr.
Insensible water loss results from continuous evaporative loss of water through the respiratory tract and the skin.10 In premature infants, a large proportion of insensible water loss occurs transcutaneously. Insensible water loss from the
skin can be minimized by using incubators in the neonatal intensive care unit (NICU) and extremity coverings in the operating
room (OR). Insensible water loss from the lungs can be decreased by humidifying the inspired gases.
In general, the maintenance fluid requirement of a neonate is considered to be 70 mL/kg/24 hr initially; this figure rises
to 100 mL/kg/24 hr after a few days of life. Neonates with surgical problems may require dramatically increased amounts of
fluid.
The sodium requirement of a full-term infant is, on average, 2 mEq/kg/24 hr. Conditions such as intestinal obstruction [see Common Surgical Problems of Newborns, Intestinal Obstruction in the Newborn, below] and peritonitis increase sodium loss and therefore increase sodium requirements. Although full-term infants can retain sodium
as well as adults do in the face of a sodium deficit, they are unable to excrete excess sodium as effectively. As a result,
excessive infusion of sodium can rapidly result in hypernatremia.
The generally accepted potassium requirement is 2 mEq/kg/24 hr after the first 2 to 3 days of life. However, the need for
potassium replacement can be significant in the first few days of life as well, especially after a major surgical intervention.
Thus, potassium should be administered in the first 1 or 2 days of life after an operation once urine output has been established.
In view of the various fluid and electrolyte requirements of neonates and infants, the initial fluid used in surgical management
of these patients, both preoperatively and postoperatively, should be 5 or 10% dextrose in 0.2% saline at a dosage of 100
to 150 mL/kg/24 hr [see Table 1].
Acid-Base Status
Metabolic alkalosis caused by loss of electrolytes (specifically, chloride) may occur with prolonged gastric suction or vomiting;
usually, it is easily corrected by replacing the lost electrolytes (e.g., by administering potassium chloride). Metabolic
acidosis, on the other hand, is usually the result of poor tissue perfusion and lactic acidosis; it is best corrected by treating
the underlying cause of the poor perfusion and by temporarily administering buffers (e.g., sodium bicarbonate). It should
be noted that standard sodium bicarbonate solutions are extremely hypertonic and should be diluted before administration,
especially when they are being given to neonates.
Temperature
In neonates, strict regulation of the environmental temperature is critical for preventing hypothermia. Compared with older
children and adults, infants have a higher ratio of body surface area to weight, less subcutaneous fat (and therefore poorer
thermal insulation), and less lean body mass (which is required for generating and retaining heat).
Maintaining the body temperature of neonates and young infants is critical in both the NICU and the OR. In the NICU, the infant
may be placed in an incubator (which is designed to reduce airflow across the skin and provide a tightly regulated, thermally
neutral environment) or on a bed with an overhead radiant heater. In the OR, the ambient room temperature may be raised, overhead
radiant warmers may be used, circulatory warm air blankets may be applied, and the head and extremities may be wrapped in
plastic. Ventilatory gases are warmed and humidified, and warm intravenous (IV) and irrigation fluids are used.
Nutrition
Whereas the nutritional requirements of children and teenagers do not differ significantly from those of adults, the requirements
of infants do. Not only must the metabolic demands that a major illness or operation imposes on all patients be taken into
account, but special consideration must also be given to the smaller body size of infants, their rapid growth, their highly
variable fluid requirements, and the immaturity of most organ systems. These characteristics, coupled with the low caloric
reserves present if the infant is premature or sick, make adequate nutritional intake particularly important. Consequently,
infants whose nutritional needs are not met as the result of a functional or organic disorder of the gastrointestinal (GI)
tract can rapidly acquire protein-calorie malnutrition. Even a relatively short period of inadequate nutrition can lead to
impaired host resistance, an increased risk of infection, and poor wound healing, all of which contribute appreciably to morbidity
and mortality in infants and children with surgical disease.
Nutritional Requirements
Infants have higher weight-adjusted caloric requirements than older children and adults do [see Table 2], and these requirements are further increased by periods of active growth and extreme physical activity.11, 12 Major illness or surgical trauma may raise caloric requirements even further. However, measured energy expenditures do not
appear to be elevated in neonatal surgical patients and critically ill premature infants.13–15 In general, calories should be provided in the proportions found in a well-balanced diet: 50% carbohydrate, 35% fat, and
15% protein.
The protein needs of infants are based on the combined requirements for maintenance and growth. Most of the increase in body
protein occurs during the first year of life, which explains why protein requirements are highest in infancy and decrease
with age. Of the 20 essential amino acids from which proteins are synthesized, eight are essential in adults, but it is believed
that in addition, histidine is essential in infants and cysteine and tyrosine in premature infants.
In general, infants require more vitamins and minerals than adults do. Increased amounts of calcium and phosphorus are particularly
important because of the rapid growth rate of the infant’s skeleton.
Nutritional Assessment
In many cases, a sick infant’s history and overall appearance provide sufficient grounds for initiating nutritional support.
For example, a preterm infant with respiratory distress who is small for his or her gestational age clearly requires parenteral
nutrition, as does a newborn with gastroschisis. Physical variables that should be considered in nutritional assessment include
weight, length, head circumference, chest circumference, and triceps skinfold thickness. No blood test reflects changes in
the patient’s nutritional status with ideal accuracy, but serum albumin, prealbumin, and transferrin levels are frequently
used as markers.
Enteral Nutrition
The type of nutritional support to be employed depends on the disease affecting the patient and on the patient’s overall health
status. From a physiologic standpoint, enteral nutrition is preferable and is the first choice for patients whose GI tract
is functioning adequately.16 For infants younger than 1 year, breast milk or a standard infant formula is delivered orally or via a tube.17 For older children who are unable or unwilling to eat, a liquid diet consisting of either blenderized food or liquid formula
may be employed. A number of nutritionally complete liquid formulas are commercially available. Specialized formulas are available
for use in patients who have lactose intolerance or protein sensitivity or who are experiencing renal or hepatic failure.
The use of predigested or elemental diets may allow patients with injured intestine or inadequate intestinal length to absorb
enteral feedings. In children who cannot manage oral feedings, nasogastric and nasojejunal tubes are used. If the child’s
condition necessitates long-term tube feeding, a gastrostomy may be necessary.
Postoperative Feeding
Infants have more difficulty in feeding during the early months of life. This is especially true of premature infants, in
whom the complex physiology of sucking and swallowing is not yet fully developed. In addition, the work of feeding accounts
for most of an infant’s caloric expenditure in the early months, and a stressed infant tires easily. For this reason, gavage
or gastrostomy tube feedings are often employed for the early stages of postoperative feeding in infants. Feeding is begun
after the resolution of postoperative ileus has been demonstrated by the passage of meconium or stool. Further evidence that
the bowel is beginning to function is the disappearance of the bilious green color of the gastric aspirate and the decrease
in the volume of the aspirate from the nasogastric or gastrostomy tube. Initially, small volumes of rehydration fluid are
given orally or via a gastrostomy tube. If these are tolerated, the feedings are increased incrementally until the nutritional
goals for the patient have been reached.
Infants tolerate increases in volume more readily than increases in osmolarity. Accordingly, it is often best to start with
standard strength breast milk or formula (20 kcal/30 mL); then, if nutritional or physiologic requirements dictate increased
calorie density, breast milk and formulas can be fortified up to a maximum of 30 kcal/30 mL.
Elemental or chemically defined diets require a minimum of digestive work and are free of residual bulk. They may be accepted
by infants if given orally; however, they are unpalatable and thus are usually given by tube. Because of the high osmolality
of these diets, constant infusion with a pump may be necessary to prevent the development of dumping syndrome. The ease of
GI absorption and the minimal residue make elemental diets useful as an intermediate step between parenteral nutrition and
regular feedings. In infants, whenever possible, oral feedings or oral stimulation should accompany tube feedings.
Parenteral Nutrition
The concept of total parenteral nutrition (TPN) had been entertained for decades before its initial development, but it was
not until the 1960s that the major breakthrough occurred, when Dudrick and colleagues successfully administered a hypertonic
glucose solution into a central vein.18, 19 In the early years after the group’s initial success, this approach to TPN began to be widely used in newborns with GI anomalies.20, 21 Since then, the technique has been applied to the care of patients of all age groups, with dramatic results. Less concentrated
solutions are available for administration into peripheral veins.
Generally, TPN is reserved for infants and children who are threatened by catabolic or nutritional deficits because feeding
via the GI tract is hazardous, inadequate, or impossible. Conditions that may necessitate TPN include intestinal obstruction
from congenital disorders, prolonged postoperative ileus, peritonitis, intestinal fistulas, chronic nonspecific diarrhea,
necrotizing enterocolitis, short bowel syndrome, extensive burns, and abdominal neoplasms treated with surgery, chemotherapy,
and radiation. Besides being used for nutritional repletion of malnourished children, TPN may also be employed prophylactically
when prolonged starvation is expected, as in cases of gastroschisis. In infants, TPN is indicated if nutrition is inadequate
for longer than 3 days; however, in older children and adults, a longer period of inadequate nutrition may be tolerated, depending
on patients’ nutritional status before operation or at the onset of illness. The benefits of improved nutrition in terms of
reducing mortality and morbidity must be weighed against the risks of serious complications, especially sepsis. TPN should
not be employed when enteral nutrition is feasible. Every effort should be made to use the enteral route for feeding, including
the use of transpyloric feeding tubes.
Infants receiving TPN must be carefully monitored. Essential clinical measurements include weight, length, and intake and
output volumes. Blood tests must be employed judiciously and sparingly in infants and children because of their small total
blood volume. At the start of therapy and once a week thereafter, a complete blood count should be done, blood urea nitrogen
should be measured, and serum levels of electrolytes, glucose, calcium, phosphorus, magnesium, and albumin should be assessed.
Serum levels of liver enzymes, bilirubin, and creatinine should be measured at the start of therapy and every week thereafter.
On average, neonates gain 15 to 25 g/day with TPN, and older children gain 0.5% of TBW/day. Greater weight gains may signal
excessive administration of fluids or intake of calories.
The main complications of parenteral nutrition include catheter-based problems (e.g., sepsis, malfunction, and venous thrombosis),
electrolyte abnormalities, and (especially in infants receiving long-term TPN) hepatic cholestasis, which ultimately can result
in cirrhosis and hepatic failure. Strategies to minimize liver damage include early and aggressive treatment of infection,
avoidance of excessive caloric intake, cycling the TPN, limitation of protein and fat intake, and supplemental trophic enteral
nutrition.
Hemodynamic Imbalance and Shock
The signs and symptoms of shock in infants and children are poorly recognized by community physicians. This is particularly
concerning given that survival is decreased in pediatric patients when shock is not promptly recognized and treated.22 For proper assessment of shock in infants and children, it is necessary to be familiar with the normal vital signs in these
age groups [see Table 3]. The two types of shock most frequently seen are hypovolemic shock and septic shock; in infants and children, septic shock
is the most common form.23 The response of newborns and infants to hypovolemic or septic shock is significantly different from the response of older
children and adults. For example, neonates affected by profound shock tend to become bradycardic, whereas adults are more
likely to become tachycardic. Moreover, neonates, especially premature neonates, normally have a low blood pressure; consequently,
shock often does not evoke any further significant reduction of blood pressure. The hypovolemia caused by the shock results
in decreased venous return, which lowers cardiac output; the reduced cardiac output leads to poor tissue perfusion and the
development of lactic acidosis.
Gram-negative bacteria are the organisms most often responsible for septic shock. Peritonitis resulting from intestinal perforation
is a common cause of septic shock in neonates and infants; other causes are urinary tract infections, respiratory tract infections,
and contaminated intravascular catheters. The pathophysiology of septic shock differs substantially from that of hypovolemic
shock; however, in both states, stasis and pooling of blood in the capillary bed lead to reduction of the circulating blood
volume.24
Treatment
The mainstay of therapy for hypovolemic shock is administration of fluid and blood. In neonates with shock, the hematocrit
should be maintained at 45% to ensure adequate oxygen delivery. In many infants, hypovolemic shock is caused not by hemorrhage
but by dehydration (e.g., from severe gastroenteritis). This dehydration is usually hypertonic because of the significant
loss of hypotonic fluid. As a rule, neonates and infants in hypovolemic shock lose much more water than electrolytes, and
as a result, serum sodium levels may exceed 150 mEq/L. Emergency treatment involves infusion of isotonic solutions of sodium
chloride with careful monitoring of serum electrolyte concentrations.
Because septic shock, like hypovolemic shock, is characterized by reduced circulating blood volume, initial therapy involves
infusion of large volumes of crystalloid solutions. When crystalloid infusions fail to address the hemodynamic instability,
colloid solutions may then be necessary.25 In addition, broad-spectrum antibiotics should always be administered.
If fluid infusion has been maximally effective (as evidenced by a normal to elevated central venous pressure) but hypotension
persists, pharmacologic agents must be given to improve myocardial contractility. The most commonly used agents are the alpha
and beta agonists dopamine and dobutamine, which primarily have inotropic and mild vasodilatory effects at lower doses. Infants
and children in shock require continuous monitoring and close clinical observation and should therefore be managed in the
intensive care unit (ICU).
Common Surgical Problems of Newborns
Neonatal surgical problems often present as emergencies and necessitate rapid stabilization and transfer of the patient to
a pediatric surgical center. Proper initial management is crucial and may have a pronounced effect on overall outcome. The
organ systems most commonly affected are the respiratory tract and the GI tract.
Emergency Surgical Problems
In many communities, the general surgeon is frequently called on to assist in the diagnosis and initial care of a neonate
with an apparent surgical problem. After stabilization measures have been carried out and preliminary tests have been performed
to establish a diagnosis, the baby may be transported to a pediatric center that is specially equipped and staffed to manage
the specific surgical problem present.
The steps in the stabilization of a critically ill neonate before transport are similar to the ABCs of initial care in an
adult (airway, breathing, circulation). By the time the surgeon is consulted, the neonatologist or pediatrician may have accomplished
initial stabilization and begun to prepare the baby for transfer. The surgeon’s immediate responsibility may be to establish
vascular access. In some cases, a peripheral venous cannula may be appropriate; more often, a central catheter is inserted
via an umbilical vein or artery. Appropriate fluids should be infused to prevent dehydration and to correct any fluid or electrolyte
deficits. When required, a nasogastric or esophageal pouch suction tube should be placed and decompression initiated. This
maneuver is extremely important if transport by air is considered: trapped gases change in volume with alterations in barometric
pressure, and such changes may have particularly deleterious effects on infants who have intestinal gas, pneumothorax, or
pneumomediastinum.
Vitamin K should be given, irrespective of whether there is concern for bleeding, in the form of phytonadione, 1 mg (0.5 mg
for babies who weigh less than 1,500 g). Appropriate antibiotics should also be administered (e.g., ampicillin, gentamicin,
and metronidazole for most suspected enteric infections). Finally, the infant should be wrapped and placed in an incubator
or a radiant warmer to stabilize body temperature and maintain it at normal levels.
The use of sophisticated transport teams with appropriate equipment and supplies is the safest method of moving these babies
between hospitals. Early stabilization and close communication between the referring physician, the accepting physician, and
the transport team are essential for minimizing the potential risks and morbidity associated with patient transfer.
The following seven emergency surgical problems are commonly encountered in neonates. Each involves special considerations
in the preparation of the patient for interhospital transfer.
1. Congenital diaphragmatic hernia (CDH). Insert a nasogastric or orogastric tube. Ventilation by face mask is contraindicated; if ventilation is required, intubate.
2. Esophageal atresia. Insert a tube to aspirate secretions from the pouch (use a Replogle tube if available). The tube will not usually advance
past 10 cm at the lip in a full-term infant with esophageal atresia. If possible, avoid mechanical ventilation; if intubation
is required, use high-frequency, low-pressure ventilation to prevent distention and possible perforation of the stomach.
3. Congenital lobar emphysema. Support normal oxygenation. Minimize mean airway pressure.
4. Intestinal obstruction. Use nasogastric or orogastric suction. Confirm placement and function of IV lines.
5. Omphalocele or gastroschisis. Use nasogastric or orogastric suction. Cover the sac with nonadherent gauze and take care not to rupture the membrane (if
present); cover the intestine with saline-soaked gauze and a see-through bowel bag. Maintain hydration by increasing fluid
administration to replace fluid lost from the exposed bowel. Support the bowel in the midline of the abdomen to prevent venous
engorgement of the bowel. Maintain body temperature.
6. Exstrophy of the bladder. Cover the exposed bladder with a nonadherent dressing.
7. Meningomyelocele. Cover the sac with a nonadherent dressing.
A congenital pulmonary airway malformation (CPAM) can occur in any lobe of the lung but most often involves the lower lobes.
CPAM encompasses a broad range of disease, including microcystic and macrocystic disease and the associated intralobar pulmonary
sequestrations. A CPAM of the left lower lobe can mimic diaphragmatic hernia both clinically and radiographically [see Figure 2a ].26, 27 Unlike a newborn with diaphragmatic hernia, however, a newborn with CPAM will have an abdomen with the normal degree of protuberance.
Although not common, air trapping within macrocystic lesions can occur, resulting in perinatal respiratory distress. If an
infant with CPAM experiences marked respiratory distress that necessitates intubation and mechanical ventilation, it is better
to perform an emergency lobectomy than to subject the baby to a period of mechanical ventilation, with its attendant risks
(i.e., barotrauma and oxygen toxicity). If the lesion is asymptomatic, it can be resected later in infancy to prevent infectious
complications and possible (albeit rare) transformation into a malignancy. An intralobar sequestration is a CPAM that has
a systemic arterial blood supply and a variable venous drainage. There is no tracheobronchial communication in a true sequestration.
The arterial flow can originate anywhere along the descending aorta, including subdiaphragmatic locations (e.g., celiac trunk
branches). Preoperative localization of origin of the feeding artery is important for intraoperative management, if complications
arise during ligation of the vessel [see Figure 2b ]. Extralobar sequestrations are rarely symptomatic at birth and can be safely resected within the first year of life.
Diaphragmatic Hernia
Infants with CDH may be quite ill at birth, often suffering from acute respiratory distress and hemodynamic instability. Because
the intestines are located in the chest, an infant with CDH appears to have a scaphoid abdomen [see Figure 2c ]. The entire stomach may be in the chest, and as a result, it may be difficult to pass a nasogastric tube into the stomach.
A plain x-ray usually establishes the diagnosis.
CDH is an embryopathy that results from abnormal development of the diaphragm and the lungs. The defect in the diaphragm allows
herniation of abdominal contents. On the affected side, as well as on the contralateral side, the lung shows hypoplasia, which
varies in severity. The small vessels (arterioles) are excessively muscularized and can easily constrict.28 The primary pathophysiologic consequence of CDH is not the presence of the hernia but, rather, severe pulmonary hypertension
and associated right heart failure.29 Accordingly, initial treatment is aimed at preventing or, if prevention is not possible, mitigating pulmonary hypertension
and its sequelae.
Babies with CDH require immediate resuscitation, correction of acidosis, and, in most cases, endotracheal intubation. Placement
of an orogastric tube can help decompress the GI tract. Once the baby is relatively stable, surgical intervention should be
delayed to allow time for the pulmonary hypertension to decrease or disappear. There is evidence to suggest that gentle ventilation
with permissive hypercapnia may decrease secondary barotrauma and long-term morbidity.30 If the baby cannot be stabilized with conventional ventilation, jet, high-frequency, or oscillation ventilation and inhaled
nitrous oxide31 may further stabilize the infant. In infants refractory to these measures of ventilation, extracorporeal membrane oxygenation
(ECMO) may be indicated. The hernia is usually addressed after the ECMO run is completed.32–34 During the procedure, the intestines are reduced into the abdominal cavity, and the diaphragmatic defect is repaired, either
in the OR or in the NICU.35 However, the operative procedure usually does not significantly alter the underlying pathophysiologic condition. In many
cases, the newborn’s condition improves at first after the operation but then begins to deteriorate. This response is seen
less frequently with delayed surgical repair. If conventional treatment fails to control the pulmonary hypertension, ECMO
may be instituted postoperatively. The precise impact of ECMO on overall outcome for CDH is still a matter of debate, although
most pediatric surgeons agree that this technique has a role as a lifesaving measure if all other interventions fail.36–38 Overall survival of infants born with CDH remains at approximately 67%, despite a number of advances in neonatal support
over the last 20 years.39
Lobar Emphysema
The term lobar emphysema refers to overexpansion of a segment of lung, which can compromise ventilation in a newborn if significant compression of
a healthy lung or a mediastinal shift occurs [see Figure 2d ].40 If the patient is exhibiting symptoms, urgent resection is usually required. If the patient shows no significant symptoms,
follow-up directed toward the affected areas of the lung is appropriate; occasionally, the condition resolves spontaneously.
Not uncommonly, surgeons are asked to evaluate newborns for possible obstruction of the GI tract. These patients usually present
with choking or vomiting. The list of possible causative conditions is fairly extensive, and most of these conditions must
ultimately be managed by a pediatric surgeon. Nevertheless, it may be helpful in the initial stages to employ an algorithmic
approach, which often serves to narrow the range of possible causes [see Figure 3 ].41
The color of the emesis helps establish the level of the obstruction. If the emesis is nonbilious, the obstruction is proximal
to the ampulla of Vater. If an orogastric tube cannot be placed, the diagnosis is esophageal atresia, which can be confirmed
by a chest x-ray that demonstrates a curled-up tube in the upper esophageal pouch. If the x-ray shows air below the diaphragm,
the diagnosis is a distal tracheoesophageal fistula. If there is no mechanical obstruction of the esophagus, the diagnosis
may simply be gastroesophageal reflux (GER). Other surgical causes of nonbilious emesis are preampullary duodenal atresia
or web, neonatal pyloric stenosis (uncommon), and pyloric atresia (extremely rare).
Bilious emesis in a newborn is a potentially more serious problem. Except for medical ileus, the main causes are all related
to underlying surgical problems. If the baby’s abdomen is not distended, he or she may have a proximal obstruction. If an
abdominal x-ray series shows a double-bubble sign without distal air, duodenal atresia is likely. If distal air is seen, the
diagnosis is either a duodenal web or malrotation with possible volvulus. Malrotation of the bowel is a true surgical emergency,
which must be diagnosed quickly by means of an upper GI study. In a newborn with midgut volvulus, prompt exploration is required
to prevent extensive necrosis of the midgut.
If the abdomen is distended, the obstruction is more distal. Physical examination is warranted to rule out an incarcerated
inguinal hernia. Inspection of the perineum is necessary to look for the presence or absence of an anus (anorectal malformation).
Stippled calcifications on an abdominal film are pathognomonic of meconium peritonitis (in utero bowel perforation), which
usually necessitates surgical exploration. If the anus is patent, a contrast enema study helps establish a diagnosis. A microcolon
represents an unused lower GI tract, which may be seen with jejunoileal atresia or meconium ileus. Meconium ileus, often associated
with cystic fibrosis, is usually characterized by the presence of large amounts of meconium in the terminal ileum and can
often be treated successfully by administering water-soluble contrast enemas. Other meconium-related functional obstructions
occur without microcolon, such as meconium plug syndrome and small left colon syndrome, both of which usually respond to treatment
with enemas. If the lower GI study shows a normal or slightly dilated colon with a narrowed or spastic-appearing rectosigmoid,
the likely diagnosis is Hirschsprung disease, which can be confirmed by suction biopsy of the rectum.
The overall incidence of neonatal abdominal wall defects is approximately one in every 2,000 births; however, the incidence
of gastroschisis has increased 10- to 20-fold in the last few decades.42–45 Omphalocele represents an arrest in development, which may explain the increased frequency of chromosomal abnormalities and
other structural birth defects in children with this condition.46 The exact cause of gastroschisis is unclear, but it does not represent a normal stage of development; it appears to be more
common with younger mothers, and environmental factors seem to play a role.42–45 The extra-abdominal intestine is prone to vascular compromise, which explains the higher incidence of bowel atresia in these
patients [see Figure 4a ].47–49
Although omphalocele is associated with a higher overall mortality than gastroschisis is, the latter is more challenging to
manage in the immediate postnatal period. The exposure of bowel results in greater insensible loss of fluid and heat. Kinking
of the intestinal blood supply may lead to venous congestion and ischemia. It is crucial to place children with gastroschisis
in a warm environment and to protect the bowel (which is easily accomplished with the help of a plastic bowel bag). IV access
should be established immediately, and resuscitation should be initiated, guided by the infant’s heart rate. Transfer to a
pediatric surgical center is mandatory. The surgical options are (1) primary repair of the defect, if the abdominal cavity
accommodates the exposed organs easily, and (2) gradual reduction of the intestines by means of a silo technique [see Figure 4b ].50
Emergency surgical intervention is rarely required for management of omphalocele. Such defects may be either closed primarily
or repaired with any of several staged approaches, depending on their size. Giant omphaloceles are best treated observantly,
with the overlying membrane used as a temporary silo and definitive repair delayed until a reasonable intra-abdominal domain
has developed.
Common Surgical Problems in Infants and Children
Pyloric Stenosis
Hypertrophic pyloric stenosis affects between two and five of every 1,000 children; it is more common in white males.51 Patients typically present with progressive, projectile, nonbilious emesis 2 to 6 weeks after birth. They may show signs
of severe dehydration with a hypochloremic, hypokalemic metabolic alkalosis (although the literature suggests that the incidence
of this finding may be decreasing).52 Medical approaches to managing this problem have been described,53, 54 but surgical correction after resuscitation is the treatment of choice. The classic open pyloromyotomy developed by Fredet-Ramstedt
is still performed, although advances in minimally invasive techniques have made it possible to perform laparoscopic pyloromyotomy
with safety and efficacy.55–58
Gastroesophageal Reflux
For appropriate treatment of GER in infants, it is important to distinguish between uncomplicated physiologic GER and symptomatic
GER.59 Symptoms that necessitate treatment include poor weight gain, esophagitis, apnea, apparent life-threatening events, and pulmonary
complications. Upper GI contrast studies and 24-hour continuous pH monitoring are the gold standards for assessment. Endoscopy
and esophageal biopsy may be useful for diagnosis and the evaluation of treatment. If GER does not resolve with simple feeding
and positioning adjustments, acid suppressants, prokinetic therapy, or surface agents may prove effective. If GER is associated
with anatomic abnormalities (e.g., hiatal hernia), apparent life-threatening events, or symptoms that persist despite optimal
medical therapy, surgical intervention is usually indicated. In infants and younger children who require a feeding gastrostomy,
a protective fundoplication may be necessary if severe reflux is demonstrated on the upper GI study.
In the United States, the procedure most commonly performed to treat GER is a 360° Nissen fundoplication,60 although partial posterior (Toupet) fundoplication appears to yield comparable results.61, 62 Both procedures are usually performed laparoscopically. Initial success rates with surgical treatment are high, but long-term
complications (especially recurrent reflux) are frequent, resulting in redo rates ranging from 7 to 26%.63
Neck And Soft Tissue Masses
A neck mass is a common reason for a child to be seen by a surgeon. Even though the vast majority of these masses are benign,
they often cause significant worries for parents. A detailed history and a thorough physical examination are crucial for narrowing
down the otherwise extensive differential diagnosis [see Table 4].
The masses that are most commonly encountered in infants and children are enlarged lymph nodes caused by reactive postinfectious
hyperplasia. Such masses feel soft, are often slightly tender on palpation, are mobile, and tend to shrink over time. A higher
degree of concern is appropriate with neck masses that are located supraclavicularly, grow rapidly, develop at multiple sites,
are fixed to surrounding tissues, or feel hard. Swift diagnosis of the underlying process is crucial in this situation. Fine-needle
aspiration biopsy may render an initial diagnosis,64, 65 but most treatment protocols require more tissue, which can be obtained through open surgical biopsy. Before the procedure,
a chest x-ray should be obtained to determine whether the mass involves the mediastinum.
A mass in the anterior midline may be a remnant of the thyroglossal duct. Such masses should be excised at diagnosis to prevent
any subsequent infectious complications. To minimize the risk of recurrence, the procedure includes the removal of the midportion
of the hyoid bone.66 A lateral neck mass, with or without a sinus tract to the skin, may be a remnant of the fetal branchial apparatus. Such masses
should also be completely excised to reduce the risk of complications.
Abdominal Wall Hernia
Inguinal and umbilical hernia repairs are among the most commonly performed procedures in pediatric surgery. Umbilical hernias
are commonly diagnosed at birth but tend to close spontaneously. The complication rate is very low; thus, most pediatric surgeons
postpone surgical repair until the patient is 5 years old. Spontaneous closure of umbilical hernias has been described as
late at 14 years of age.67 Inguinal hernias, on the other hand, pose a risk of incarceration, especially during infancy, and should therefore be repaired
soon after they are diagnosed.68–70 High ligation of the sac is the treatment of choice. This operation is usually straightforward, but it can be challenging
at times, particularly in premature infants with large hernias. Complications are rare; they tend to occur more frequently
if the infant is premature or if the procedure was done on an emergency basis.70 To minimize the risk of injury to the spermatic cord, a “no touch” technique should be employed. Whether the contralateral
side should be explored remains controversial; some surgeons now use laparoscopy to assess the processus vaginalis of the
opposite side.71
Undescended Testicles
Approximately 4% of male neonates have an undescended testis at birth, but only about 1% still have this condition at the
age of 1 year.72 Accordingly, most pediatric surgeons recommend that neonates with an undescended testis be observed during the first year
of life. If the testicle has not descended by the end of this period, inguinal orchiopexy may be performed; the success rate
with this option is higher than 80%.73 It is not unusual to find a structurally abnormal testicle during exploration. Potential complications include testicular
atrophy and recurrent ascent of the gonad. Hormonal treatment (with human chorionic gonadotropin or luteinizing hormone–releasing
hormone) is widely employed in Europe but has a success rate of only about 50%. Laparoscopy is a useful and cost-effective
option for the evaluation of nonpalpable testes.73–75 Adults with a history of undescended testes have lower fertility rates and a higher risk of subsequent testicular cancer,
necessitating continued surveillance of the testes after successful orchiopexy.76–79
Circumcision
Circumcision remains one of the most commonly performed procedures in male newborns. Approximately 1.2 million circumcisions
are performed in the United States each year, at a total cost of at least $150 million.80 There continues to be a great deal of controversy regarding the medical benefits and risks of this procedure. The most recent
American Academy of Pediatrics policy statement states that “…preventive health benefits of elective circumcision in male
newborns outweigh the risks of the procedure.”80 Some authors argue that urinary tract infections, sexually transmitted diseases, and genital cancer occur less frequently
in circumcised males; however, others dispute these findings.81–83 Circumcision can be performed either in an outpatient setting or in the nursery. The use of local anesthesia is recommended.80 Complications are rare and usually minor, but mutilating injuries to the penis have been reported.84, 85
Abdominal Pain
Abdominal pain is a common finding in children and may arise from any of a wide variety of causes. Among surgical diseases,
acute appendicitis is the most common cause of abdominal pain. In addition, however, the differential diagnosis must include
perforated duodenal ulcer, Meckel diverticulum, ulcerative colitis, Crohn disease, ruptured ovarian cyst, pelvic inflammatory
disease, and, in younger children, intussusception. It is important to remember that medical problems (e.g., constipation,
gastroenteritis, pancreatitis, and pneumonia) can also give rise to substantial abdominal discomfort.
Appendicitis
The diagnosis of appendicitis is based on the presence of localizing physical findings in the right lower quadrant of the
abdomen. Even if the history and laboratory data are not typical, the presence of such findings should allow the surgeon to
make the diagnosis and proceed with appropriate management.
Although there is some debate regarding the utility of imaging studies in patients with the classic findings of acute appendicitis,
it appears that ultrasonography or computed tomography (CT) can be helpful in patients with delayed presentations or atypical
symptoms.86–89 If perforated appendicitis with an abscess is identified, a safe and cost-effective treatment approach is to give antibiotics
(first IV, then oral), drain the collection percutaneously, and perform an interval appendectomy 6 weeks later.90–94
Crohn Disease
Occasionally, a teenager who exhibits the classic signs and symptoms of appendicitis turns out to have Crohn disease of the
terminal ileum. This condition will be obvious at operation, when the mesenteric fat is seen to be creeping up along the sides
of the inflamed ileum. If the base of the appendix is free of disease, an appendectomy should be performed at the time of
exploration.95 Once the child has fully recovered from the operation, a definitive workup, including a small bowel series and a colonoscopy
with biopsy, should be performed to ascertain the extent of the Crohn disease. If an enterocutaneous fistula develops, TPN
will be required for a prolonged period. If an intestinal stricture develops (typically, in the terminal ileum), resection
may be required. Associated perianal Crohn disease can be very challenging to treat. Medical therapy and surgical therapy
should be carried out conjointly. Conservative surgical options (e.g., local drainage, fistulotomy, and setons) are preferred;
if sepsis does not resolve or if severe incontinence persists, a stoma may be required.96
Idiopathic intussusception usually develops between the ages of 6 months and 2 years. The invagination of small bowel into
the cecum results in severe, crampy, intermittent abdominal pain, often associated with obstructive symptoms and bloody stools.
Marked lethargy is also a frequent symptom. Diagnosis is usually confirmed with a sonogram showing a “target sign” [see Figure 5a ]. Air enemas and ultrasound-guided hydrostatic reduction have both been employed to treat this condition, with great success
[see Figure 5b ].97–99 Surgical exploration should be reserved for patients in whom reduction is unsuccessful and patients in whom there is reason
to suspect an anatomic lead point that is causing the intussusception.
Meckel Diverticulum and Lower GI Hemorrhage
Meckel diverticulum represents a remnant of the omphalomesenteric duct. It occurs in approximately 2% of the pediatric population.
If symptomatic, it can be associated with acute and substantial lower GI bleeding, obstruction, intussusception, omphalitis,
or diverticulitis.100, 101 The differential diagnosis of lower GI bleeding in children also includes GI infections, colonic polyps, duplication cysts,
and inflammatory bowel disease. Meckel diverticulitis presents with symptoms very similar to those of acute appendicitis and
should always be ruled out if the appendix appears normal on exploration. Management of an asymptomatic diverticulum discovered
during an abdominal operation remains controversial, even though resection has been shown to carry a low risk of complications.102, 103
Venous Access
To obtain venous access in an infant, a venous cutdown is performed, and a pediatric silicone (Broviac) catheter is passed
through the common facial, external jugular, or internal jugular vein to the superior vena cava. This procedure is carried
out in the OR or, if necessary, in the NICU. Adequate exposure must be obtained, proper instruments and fluoroscopy devices
should be available, and strict aseptic conditions must be maintained. The venous catheter is tunneled from the point of entry
into the vein to a skin exit site 5 to 10 cm away, with the aim of minimizing the likelihood of bloodstream contamination
from dressing changes at the skin exit site. The catheter is brought out on the chest wall, where it is easily accessible
and unlikely to be disrupted by an active patient. When no vein sites are available in the neck, the catheter may be advanced
into the central venous circulation via a cutdown on the great saphenous vein and tunneled to an exit site on the abdominal
wall or the leg.104 In older children and adults, percutaneous puncture of the subclavian vein is often performed in place of venous cutdown;
this technique can also be used in infants.105, 106 Regardless of the technique employed, manipulation of the catheters must be done with close attention to asepsis and antisepsis
to minimize the risk of bacterial and fungal infection.
Pediatric Trauma
Whereas accidents are the third most common cause of death in the US population as a whole, they are the single most common
cause of death in children between the ages of 1 and 15 years.107 Every year, about 20 million injuries occur in children, resulting in approximately 15,000 deaths and 100,000 cases of permanent
disability. The first approach to be considered in managing pediatric trauma is clearly prevention. In fact, preventive efforts
have been gaining momentum, thanks in part to support from federal funds and from dedicated individual advocates. These efforts
have included greater emphasis on the use of pediatric restraint devices in automobiles, improvements in the design of motor
vehicles, the wearing of helmets by bicycle and motorcycle riders, improvements in the design of space heaters, the use of
fire-retardant material for children’s clothes (as dictated by the Flammable Fabrics Act), proper packing and labeling of
poisons and medications, and the installation of appropriate fencing around swimming pools.
General Principles
Although the general principles of trauma care are essentially the same for children as for adults, several significant differences
must be taken into account in the care of pediatric accident victims.108 For example, children do not react to trauma in the same way as adults do. They often have difficulty in expressing pain
and in articulating their complaints. They are often extremely frightened after an accident, and this fear may cause them
to give misleading signals (e.g., by exhibiting signs of an acute abdomen even though no intra-abdominal injury has occurred).
Children who experience stress often undergo developmental regression, typically accompanied by severe depression. All of
these psychological factors must be considered in the treatment of a pediatric accident victim.
Another key difference between children and adults is that children are still growing. Postoperative metabolic management
after any form of stress, whether from a surgical procedure, from trauma, or from some other event, must take this difference
into account. Children can compensate for hypovolemia effectively and keep their vital signs in the normal range, even in
the presence of shock. However, a small blood loss that would be insignificant in an adult can result in marked hemodynamic
changes in a small child. Moreover, water and heat loss can be far more extensive in small children than in older children
and adults because smaller children have a greater surface area in relation to their weight and have a relative lack of insulating
subcutaneous fat. Hypothermia aggravates acidosis and makes hemodynamic resuscitation much more difficult. Gastric dilatation
from marked aerophagia during crying, which can result in vomiting and pulmonary aspiration, is very common in young children
after all forms of trauma. Finally, the nutritional requirements of injured children are greater than those of injured adults
because children, as growing organisms, naturally have a high metabolic rate. Consequently, supplemental nutrition often must
be started earlier in the treatment of a child than it would be in the treatment of an adult in a comparable condition.
Not only are there significant physiologic and psychological differences between children and adults after trauma, but there
also are differences in accident patterns. Most childhood injuries result from blunt trauma. Head trauma is far more common
in children than in adults; in fact, it accounts for most of the morbidity and mortality in the pediatric population. After
motor vehicle accidents, which are the major cause of trauma in both children and adults, the next most frequent causes of
trauma in children are events that are less important causes in adults: falls, bicycle accidents, drowning, poisoning, and
burns from fires. Nonaccidental trauma (NAT) [see Nonaccidental Trauma, below] is a unique and important cause of trauma in children.
Pediatric accident victims must be treated in centers that have experience in the care of traumatized children.109, 110 Such centers must have an emergency department with a section that is specifically set aside for the care of children and
is staffed by nurses and physicians who are familiar with the management of pediatric trauma. They must have a hospital with
a pediatric ICU that is also staffed by experienced medical and paramedical personnel. Finally, they must have a transportation
system that is capable of rapidly transporting critically ill pediatric trauma victims both by air and by land or water. The
Pediatric Trauma Score [see Table 5] has been successfully used not only as a tool for grading severity of injury but also as a means of comparing trauma care
across institutions.
Airway Management, Pain Relief, and Sedation
Establishing and maintaining a secure airway are of the utmost importance in treating any injured child. Most pediatric trauma
programs have mechanisms in place that delegate this responsibility to the attending emergency physician or anesthesiologist
so that the surgical team can continue assessing the patient. The airway team can also provide conscious sedation or elective
intubation if a painful procedure (e.g., reduction of fractures or suturing of lacerations) proves necessary.
Management of Head Injury
Closed head injuries (CHIs) are the most common cause of death in children and account for close to 7,000 fatalities in the
United States each year.111 Any child who had a witnessed loss of consciousness, is mentally altered, or has an abnormal Glasgow Coma Scale (GCS) score
should undergo urgent CT scanning of the head. If the GCS score is lower than 8, intubation is necessary to protect the airway.
The main focus of therapy for severe CHI is on minimizing secondary insult to the brain by optimizing oxygen delivery. Management
involves close monitoring of cerebral perfusion pressure and judicious use of sedatives, anticonvulsants, pressors, intraventricular
drainage, or even surgical decompression to optimize cerebral perfusion. Evidence-based practice guidelines for the management
of pediatric CHI patients are now available.112
Because children with even minor structural injuries to the brain are at risk for hyponatremia, close monitoring of sodium
levels is required.113, 114 Patients who have postconcussive symptoms (e.g., vomiting, seizures, or headache) should be admitted, even if the head CT
reveals no structural injury.
Cervical Spine Clearance
Cervical spine injuries in children are very rare but can have catastrophic consequences if missed. Children, more so than
adults, are at risk for spinal cord injury without radiographic abnormality (SCIORA). The ligamentous laxity of the pediatric
cervical spine allows for injury in the absence of obvious vertebral column disruption.115 As with adult trauma patients, it is crucial to follow established protocols for systematically clearing the cervical spine.
Early immobilization, clinical examination, plain x-rays, and advanced imaging studies can all be useful. Because of the anatomic
differences between children and adults, certain modifications to adult protocols are necessary.116, 117
Blunt Solid-Organ Injury
Abdominal trauma in children is usually blunt. Penetrating injuries occur in only about 20% of children who sustain trauma
and are managed in essentially the same way as they are in adults.
Children who sustain major trauma often have intra-abdominal injuries. Because gastric dilatation and reflex ileus are far
more common in children than in adults after a major injury, the initial clinical evaluation of the child’s abdomen may be
highly misleading. Early insertion of a nasogastric tube decompresses the stomach and allows more accurate physical examination
of the abdomen; in addition, it reduces the risk of aspiration pneumonitis. Once the child is stable with respect to hemodynamic
and respiratory status, the abdomen should be carefully examined for external evidence of trauma (e.g., ecchymoses, abrasions,
and tire tracks). The abdomen should then be carefully and gently palpated, with the awareness that a frightened child often
tightens his or her rectus muscles in a way that gives a false impression of intra-abdominal injury. Serial abdominal examinations
are essential.
CT has become the gold standard for evaluation of blunt abdominal injury in stable trauma patients.118 This modality is extremely accurate in evaluating solid-organ injuries and determining the amount of blood in the peritoneal
cavity. It is also useful for detecting pneumoperitoneum resulting from intestinal perforation.119 To minimize exposure to radiation, the scan should be performed at the lowest radiation level feasible, and repeat scans
should be avoided if possible.120 The usefulness of the focused assessment for the sonographic examination of the trauma patient (FAST) in children remains
controversial at present.121–123
Because hollow viscus injury is uncommon in children who sustain blunt abdominal trauma, nonoperative treatment is the accepted
method of management for most hepatic and splenic injuries. Associated fractures of the lower ribs are rare in children with
injuries to the liver or the spleen, although they are fairly common in adults with such injuries.124
A CT scan can accurately confirm the diagnosis of a splenic injury. A major reason why nonoperative treatment is recommended
for pediatric splenic injuries is that the risk of overwhelming postsplenectomy infection (OPSI) is far higher in children
than in adults; the younger the child, the higher the risk of OPSI. OPSI develops in 3.3% of pediatric patients who have undergone
splenectomy and carries a mortality of 50%.125
Once a hepatic or splenic injury has been diagnosed, management should follow the established guidelines for treatment of
blunt solid-organ injuries in children.126–128
Ongoing hemodynamic instability despite aggressive resuscitation is the main indication for operative intervention; fortunately,
it is a rare occurrence. The likelihood of successful nonoperative management of hepatic and splenic injuries continues to
be higher in designated pediatric trauma centers.129
The finding of intraperitoneal fluid on a CT scan without evidence of solid-organ injury remains a challenging clinical problem.
Management of patients with this finding should be individualized; treatment options include observation with serial examinations,
laparoscopy, and surgical exploration.119, 130
Among the less common injuries to intra-abdominal organs that occur in children are perforation of the stomach when an accident
occurs shortly after eating (while the stomach is distended), perforation of the small intestine and large intestine at a
point of fixation (e.g., the ligament of Treitz or the cecum), rupture of the left diaphragm, and damage to the duodenum or
pancreas. The likelihood of visceral injury increases dramatically if bruising from a lap belt or a chance fracture is present.131–133 Retroperitoneal perforation is suggested by the presence of air around the right kidney on a plain abdominal x-ray. Traumatic
pancreatic injury is suggested by an elevation in serum amylase and lipase levels and by the presence of pancreatic edema
on ultrasonography or CT. Obviously, perforations of the stomach, the intestine, or the duodenum call for exploratory laparotomy;
in most cases, simple suture repair of the laceration is sufficient. Injury to the pancreas, on the other hand, can usually
be managed nonoperatively with nasogastric decompression and IV fluids. Fracture of the pancreatic duct is quite rare in children
and is usually secondary to compression of the pancreas against the vertebral column. In the past, this injury was usually
treated with exploratory laparotomy and distal pancreatectomy, but current experience indicates that it can often be successfully
treated by nonoperative means, although there is a risk that pseudocysts will subsequently develop.134–136 Intramural duodenal hematoma is relatively uncommon in children and is usually well managed by providing nasogastric decompression
for about 10 days and instituting TPN.137
In any child who has sustained abdominal trauma, the diagnosis of a pelvic fracture should be seriously considered. Pelvic
fractures can result in significant bleeding into the retroperitoneum, as well as injuries to the bladder and the urethra.
The diagnosis can be confirmed by x-ray studies of the pelvis. In most cases, the fracture can be treated with bed rest, immobilization,
and the replacement of lost blood.138
Children with blunt multisystem trauma seldom die if they are alive when brought to an emergency department, unless they have
sustained head injuries. Care of these patients requires aggressive, coordinated efforts on the part of a multispecialty team
that is under the direction of a pediatric surgeon. The establishment of specifically designated pediatric trauma centers
around the country was one of the most important developments in the care of children to occur in the 1980s; since then, multiple
studies have demonstrated that this measure has substantially improved outcomes for injured children.139
Nonaccidental Trauma
One of the unique varieties of pediatric trauma is NAT. It is a sad fact that intentional trauma is the most common cause
of fatal injury in children younger than 1 year. The exact incidence of NAT is not known, but it is believed that in the United
States, about 160,000 children are seriously injured by deliberate abuse each year.140 Risk factors include a low socioeconomic background, a single-parent family, low birth weight, and multiple siblings.141, 142 The victims tend to be younger than 2 years, except when sexual abuse is involved, in which case, the average age is about
10 years. The abuse can take many different forms, such as physical or mental injury, nutritional or hygienic neglect, delayed
or inadequate treatment of disease, sexual abuse, and verbal abuse. Salient clues to the diagnosis include an unreasonable
delay in seeking medical help for the child, inconsistencies between the trauma observed and the injury mechanism described,
poor hygiene, and marked depression or lack of emotion in the child. The injuries most commonly seen are soft tissue injuries,
burns, fractures, and head trauma. X-ray evidence of healing fractures of different ages (a finding described as long ago
as 1946) and of rib fractures is highly correlated with abuse.124, 143 Visceral injuries (e.g., hepatic fractures, splenic fractures, duodenal hematomas, and pancreatic fractures) are also associated
with abuse, although less frequently.
Once a physician suspects NAT, he or she is both legally and ethically obligated to report the situation to the appropriate
hospital team and to the social services agency of the local jurisdiction. The typical hospital team usually includes a physician,
a social worker, and a nurse. Prompt and full reporting often improves the chances that the parents will receive positive
counseling, which may well reduce their subsequent abuse of the child.
Financial Disclosures: None Reported
References
1. Coran AG. Perioperative care of the pediatric patient. Surg Annu 1991;23(Pt 1):31.
2. Friis-Hansen B. Changes in body water compartments during growth. Helv Paediatr Acta 1955;10:12.
3. Friis-Hansen BJ, Holiday M, Stapleton T, et al. Total body water in children. Pediatrics 1951;7:321.
4. Friis-Hansen B. Body water compartments in children: changes during growth and related changes in body composition. Pediatrics
1961;28:169.
5. Friis-Hansen B. Body composition during growth: in vivo measurements and biochemical data correlated to differential anatomical
growth. Pediatrics 1971;47 Suppl 2:264.
6. Friis-Hansen B. Water distribution in the foetus and newborn infant. Acta Paediatr Scand Suppl 1983;305:7–11.
7. Friis-Hansen B. The extracellular fluid volume in infants and children. Acta Paediatr 1954;43:444.
8. Aperia A, Broberger O, Thodenius K, et al. Renal control of sodium and fluid balance in newborn infants during intravenous
maintenance therapy. Acta Paediatr Scand 1975;64:725.
9. Aperia A, Broberger O, Herin P, et al. Postnatal control of water and electrolyte homeostasis in pre-term and full-term
infants. Acta Paediatr Scand Suppl 1983;305:61.
10. Doyle LW, Sinclair JC. Insensible water loss in newborn infants. Clin Perinatol 1982;9:453.
11. Dechert R, Wesley J, Schafer L, et al. Comparison of oxygen consumption, carbon dioxide production, and resting energy
expenditure in premature and full-term infants. J Pediatr Surg 1985;20:792.
12. Dechert RE, Wesley JR, Schafer LE, et al. A water-sealed indirect calorimeter for measurement of oxygen consumption (VO2), carbon dioxide production (VCO2), and energy expenditure in infants. JPEN J Parenter Enteral Nutr 1988;12:256.
13. Jaksic T, Shew SB, Keshen TH, et al. Do critically ill surgical neonates have increased energy expenditure? J Pediatr
Surg 2001;36:63.
14. Garza JJ, Shew SB, Keshen TH, et al. Energy expenditure in ill premature neonates. J Pediatr Surg 2002;37:289.
15. Agus MS, Jaksic T. Nutritional support of the critically ill child. Curr Opin Pediatr 2002;14:470.
16. Heymsfield SB, Bethel RA, Ansley JD, et al. Enteral hyperalimentation: an alternative to central venous hyperalimentation.
Ann Intern Med 1979;90:63.
17. Johnson LR, Copeland EM, Dudrick SJ, et al. Structural and hormonal alterations in the gastrointestinal tract of parenterally
fed rats. Gastroenterology 1975;68:1177.
18. Dudrick SJ, Wilmore DW, Vars HM, et al. Long-term total parenteral nutrition with growth, development, and positive nitrogen
balance. Surgery 1968;64:134.
19. Dudrick SJ, Wilmore DW, Vars HM, et al. Can intravenous feeding as the sole means of nutrition support growth in the
child and restore weight loss in an adult? An affirmative answer. Ann Surg 1969;169:974.
20. Heird WC, Winters RW. Total parenteral nutrition: the state of the art. J Pediatr 1975;86:2.
21. Filler RM, Eraklis AJ, Rubin VG, et al. Long-term total parenteral nutrition in infants. N Engl J Med 1969;281:589.
22. Carcillo JA, Kuch BA, Han YY, et al. Mortality and functional morbidity after use of PALS/APLS by community physicans.
Pediatrics 2009;124:500.
23. Wesley J, Coran A. Infants and children. In: Barrett J, Nyhus LM, editors. Treatment of shock: principles and practice.
Philadelphia: Lea & Febiger; 1986. p. 211.
24. Holcroft JW, Trunkey DD, Carpenter MA. Extravasation of albumin in tissues of normal and septic baboons and sheep. J
Surg Res 1979;26:341.
25. Carcillo JA. Choice of fluids for resuscitation in children with severe infection and shock. BMJ 2010;341:515.
26. Wesley JR, Heidelberger KP, DiPietro MA, et al. Diagnosis and management of congenital cystic disease of the lung in
children. J Pediatr Surg 1986;21:202.
27. Coran AG, Drongowski R. Congenital cystic disease of the tracheobronchial tree in infants and children: experience with
44 consecutive cases. Arch Surg 1994;129:521.
28. Stolar CJH, Dillon PW. Congenital diaphragmatic hernia and eventration. In: O’Neill JA, Rowe MI, Grosfeld JL, et al,
editors. Pediatric surgery. St. Louis: CV Mosby; 1998. p. 819.
29. Nobuhara KK, Wilson JM. Pathophysiology of congenital diaphragmatic hernia. Semin Pediatr Surg 1996;5:234.
30. Bagolan P, Casaccia G, Crescenzi F, et al. Impact of a current treatment protocol on outcome of high-risk congenital
diaphragmatic hernia. J Pediatr Surg 2004;39:313.
31. Okuyama H, Kubota A, Oue T, et al. Inhaled nitric oxide with early surgery improves the outcome of antenatally diagnosed
congenital diaphragmatic hernia. J Pediatr Surg 2002;37:1188.
32. Bartlett RH, Andrews AF, Toomasian JM, et al. Extracorporeal membrane oxygenation for newborn respiratory failure: forty-five
cases. Surgery 1982;92:425.
33. Reickert CA, Hirschl RB, Schumacher R, et al. Effect of very delayed repair of congenital diaphragmatic hernia on survival
and extracorporeal life support use. Surgery 1996;120:766.
34. Steimle CN, Meric F, Hirschl RB, et al. Effect of extracorporeal life support on survival when applied to all patients
with congenital diaphragmatic hernia. J Pediatr Surg 1994;29:997.
35. Lago P, Meneghini L, Chiandetti L, et al. Congenital diaphragmatic hernia: intensive care unit or operating room? Am
J Perinatol 2005;22:189.
36. Wilson JM, Lund DP, Lillehei CW, et al. Congenital diaphragmatic hernia—a tale of two cities: the Boston experience.
J Pediatr Surg 1997;32:401.
37. Dillon PW, Cilley RE, Mauger D, et al. The relationship of pulmonary artery pressure and survival in congenital diaphragmatic
hernia. J Pediatr Surg 2004;39:307.
38. Stege G, Fenton A, Jaffray B. Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia. Pediatrics
2003;112:532.
39. Van den Hout L, Schaible T, Cohen-Overbeek TE, et al. Actual outcome in infants with congenital diaphragmatic hernia:
the role of a standardized postnatal treatment protocol. Fetal Diagn Ther 2011;29:55.
40. de Lorimier AA. Respiratory problems related to the airway and lung. In: O’Neill JA, Rowe MI, Grosfeld JL, et al, editors.
Pediatric surgery. St. Louis: CV Mosby; 1998. p. 873.
41. Ricketts RR. Workup of neonatal intestinal obstruction. Am Surg 1984;50:517.
42. Rasmussen SA, Frias JL. Non-genetic risk factors for gastroschisis. Am J Med Genet 2008;148C:199.
43. Castilla EE, Mastroiacovo P, Orioli IM. Gastroschisis: international epidemiology and public health perspective. Am J
Med Genet 2008;148C:162.
44. Loane M, Dolk H, Bradbury I. Increasing prevalence of gastroschisis in Europe 1980–2002: a phenomenon restricted to young
mothers? Pediatr Perinat Epidemiol 2007;21:363.
45. Alvarez SM, Burd RS. Increasing prevalence of gastroschisis repairs in the United States: 1996–2003. J Pediatr Surg 2007;42:943.
46. Stoll C, Alembik Y, Dott B, et al. Risk factors in congenital abdominal wall defects (omphalocele and gastroschisis):
a study in a series of 265,858 consecutive births. Ann Genet 2001;44:201.
47. Kronfil R, Bradnock TH, Sabharwal A. Intestinal atresia in association with gastroschisis: a 26 year review. J Pediatr
Surg 2010;26:891.
48. Arnold MA, Chang DC, Nabaweesi R, et al. Risk stratification of 4344 patients with gastroschisis into simple and complex
categories. J Pediatr Surg 2007;42:1520.
49. Snyder CL, Miller KA, Sharp RJ, et al. Management of intestinal atresia in patients with gastroschisis. J Pediatr Surg
2001;36:1542.
50. Komuro H, Imaizumi S, Hirata A, et al. Staged silo repair of gastroschisis with preservation of the umbilical cord. J
Pediatr Surg 1998;33:485.
51. Hernanz-Schulman M. Infantile hypertrophic pyloric stenosis. Radiology 2003;227:319.
52. Papadakis K, Chen EA, Luks FI, et al. The changing presentation of pyloric stenosis. Am J Emerg Med 1999;17:67.
53. Huang YC, Su BH. Medical treatment with atropine sulfate for hypertrophic pyloric stenosis. Acta Paediatr Taiwan 2004;45:136.
54. Yamataka A, Tsukada K, Yokoyama-Laws Y, et al. Pyloromyotomy versus atropine sulfate for infantile hypertrophic pyloric
stenosis. J Pediatr Surg 2000;35:338.
55. van der Bilt JD, Kramer WL, van der Zee DC, et al. Laparoscopic pyloromyotomy for hypertrophic pyloric stenosis: impact
of experience on the results in 182 cases. Surg Endosc 2004;18:907.
56. Oomen MWN, Hoekstra LT, Bakx R, et al. Open versus laparoscopic pyloromyotomy for hypertrophic pyloric stenosis: a systematic
review and meta-analysis focusing on major complications. Surg Endosc 2012;26:2104.
57. Perger L, Fuch JR, Komidar L, et al. Impact of surgical approach on outcome in 622 consecutive pyloromyotomies at a pediatric
teaching institution. J Pediatr Surg 2009;44:2119.
58. Yagmurlu A, Barnhart DC, Vernon A, et al. Comparison of the incidence of complications in open and laparoscopic pyloromyotomy:
a concurrent single institution series. J Pediatr Surg 2004;39:292.
59. Rudolph CD, Mazur LJ, Liptak GS, et al. Guidelines for evaluation and treatment of gastroesophageal reflux in infants
and children: recommendations of the North American Society for Pediatric Gastroenterology and Nutrition. J Pediatr Gastroenterol
Nutr 2001;32 Suppl 2:S1.
60. Fonkalsrud EW, Ashcraft KW, Coran AG, et al. Surgical treatment of gastroesophageal reflux in children: a combined hospital
study of 7467 patients. Pediatrics 1998;101:419.
61. Bensoussan AL, Yazbeck S, Carceller-Blanchard A. Results and complications of Toupet partial posterior wrap: 10 years’
experience. J Pediatr Surg 1994;29:1215.
62. Weber TR. Toupet fundoplication for gastroesophageal reflux in childhood. Arch Surg 1999;134:717.
63. Pacilli M, Chowdhury MM, Pierro A. The surgical treatment of gastro-esophageal reflux in neonates and infants. Semin
Pediatr Surg 2005;14:34.
64. Tunkel DE, Baroody FM, Sherman ME. Fine-needle aspiration biopsy of cervicofacial masses in children. Arch Otolaryngol
Head Neck Surg 1995;121:533.
65. Liu ES, Bernstein JM, Sculerati N, et al. Fine needle aspiration biopsy of pediatric head and neck masses. Int J Pediatr
Otorhinolaryngol 2001;60:135.
66. Sistrunk W. The surgical treatment of cysts of the thyroglossal tract. Ann Surg 1920;71:121.
67. Meier DE, OlaOlaorun DA, Omodele RA, et al. Incidence of umbilical hernia in African children: redefinition of “normal”
and reevaluation of indications for repair. World J Surg 2001;25:645.
68. Zamakhshary M, To T, Guan J, et al. Risk of incarceration of inguinal hernia among infants and young children awaiting
elective surgery. CMAJ 2008;179:1001.
69. Vaos G, Gardikis S, Kambouri K, et al. Optimal timing or repair of an inguinal hernia in premature infants. Pediatr Surg
Int 2010;26:379.
70. LautzTB, Raval MV, Reynolds M. Does timing matter? A national perspective on the risk of incarceration in premature neonates
with inguinal hernia. J Pediatr 2001;158:573.
71. Bhatia AM, Gow KW, Heiss KF, et al. Is the use of laparoscopy to determine presence of contralateral patent processus
vaginalis justified in children greater than 2 years of age? J Pediatr Surg 2004;39:778.
72. Scorer CG. The descent of the testis. Arch Dis Child 1964;39:605.
73. Docimo SG. The results of surgical therapy for cryptorchidism: a literature review and analysis. J Urol 1995;154:1148.
74. Lorenzo AJ, Samuelson ML, Docimo SG, et al. Cost analysis of laparoscopic versus open orchiopexy in the management of
unilateral nonpalpable testicles. J Urol 2004;172:712.
75. Leung AK, Robson WL. Current status of cryptorchidism.Adv Pediatr 2004;51:351.
76. Miller K, Coughlin M, Lee P. Fertility after unilateral cryptorchidism. Paternity, time to conception, pretreatment testicular
location and size, hormone and sperm parameters. Horm Res 2001;55:249.
77. Lee PA, O’Leary LA, Songer NJ, et al. Paternity after bilateral cryptorchidism. A controlled study. Arch Pediatr Adolesc
Med 1997;151:260.
78. United Kingdom Testicular Cancer Study Group. Aetiology of testicular cancer: association with congenital abnormalities,
age at puberty, infertility and exercise. BMJ 2008;308:1393.
79. Petterson A, Richiardi L, Nordenskjold A, et al. Age at surgery for undescended testis and risk of testicular cancer.
N Engl J Med 2007;356:1835.
80. American Academy of Pediatrics: Task Force on Circumcision. Circumcision policy statement. Pediatrics 2012;130:585.
81. Agot KE, Ndinya-Achola JO, Kreiss JK, et al. Risk of HIV-1 in rural Kenya: a comparison of circumcised and uncircumcised
men. Epidemiology 2004;15:157.
82. Schoen EJ. Benefits of newborn circumcision: is Europe ignoring medical evidence? Arch Dis Child 1997;77:258.
83. Hellsten SK. Rationalising circumcision: from tradition to fashion, from public health to individual freedom—critical
notes on cultural persistence of the practice of genital mutilation. J Med Ethics 2004;30:248.
84. Williams N, Kapila L. Complications of circumcision. Br J Surg 1993;80:1231.
85. Sylla C, Diao B, Diallo AB, et al. [Complications of circumcision: report of 63 cases]. Prog Urol 2003;13:266.
86. Meier DR, Guzzetta PC, Barber RG, et al. Perforated appendicitis in children: is there a best treatment? J Pediatr Surg
2003;38:1520.
87. Wilcox RT, Traverso LW. Have the evaluation and treatment of acute appendicitis changed with new technology? Surg Clin
North Am 1997;77:1355.
88. Garcia Pena BM, Cook EF, Mandl KD. Selective imaging strategies for the diagnosis of appendicitis in children. Pediatrics
2004;113:24.
89. Pena BM, Taylor GA, Fishman SJ, et al. Costs and effectiveness of ultrasonography and limited computed tomography for
diagnosing appendicitis in children. Pediatrics 2000;106:672.
90. Bufo AJ, Shah RS, Li MH, et al. Interval appendectomy for perforated appendicitis in children. J Laparoendosc Adv Surg
Tech A 1998;8:209.
91. Ho CM, Chen Y, Lai HS, et al. Comparison of critical conservative treatment versus emergency operation in children with
ruptured appendicitis with tumor formation. J Formos Med Assoc 2004;103:359.
92. Weber TR, Keller MA, Bower RJ, et al. Is delayed operative treatment worth the trouble with perforated appendicitis in
children? Am J Surg 2003;186:685.
94. Jamieson DH, Chait PG, Filler R. Interventional drainage of appendiceal abscesses in children. AJR Am J Roentgenol 1997;169:1619.
95. Dagradi V, Delaini GG, Carolo F, et al. [Appendectomy and Crohn disease]. Chir Ital 1984;36:986.
96. Whiteford MH, Kilkenny J 3rd, Hyman N, et al. Practice parameters for the treatment of perianal abscess and fistula-in-ano
(revised). Dis Colon Rectum 2005;48:1337.
97. Crystal P, Hertzanu Y, Farber B, et al. Sonographically guided hydrostatic reduction of intussusception in children.
J Clin Ultrasound 2002;30:343.
98. Navarro OM, Daneman A, Chae A. Intussusception: the use of delayed, repeated reduction attempts and the management of
intussusceptions due to pathologic lead points in pediatric patients. AJR Am J Roentgenol 2004;182:1169.
99. Rubi I, Vera R, Rubi SC, et al. Air reduction of intussusception. Eur J Pediatr Surg 2002;12:387.
100. Ruscher KA, Fisher JN, Hughes CD, et al. National trends in the surgical management of Meckel’s diverticulum. J Pediatr
Surg 2011;46:893.
101. St-Vil D, Brandt ML, Panic S, et al. Meckel’s diverticulum in children: a 20-year review. J Pediatr Surg 1991;26:1289.
102. Fa-Si-Oen PR, Roumen RM, Croiset van Uchelen FA. Complications and management of Meckel’s diverticulum—a review. Eur
J Surg 1999;165:674.
103. Grimaldi L, Zingaro N, Trecca A. [Surgical management of incidental Meckel’s diverticulum: the necessity to obtain the
informed consent]. Minerva Chir 2005;60:71.
104. Fonkalsrud EW, Berquist W, Burke M, et al. Long-term hyperalimentation in children through saphenous central venous catheterization.
Am J Surg 1982;143:209.
106. Bonventre EV, Lally KP, Chwals WJ, et al. Percutaneous insertion of subclavian venous catheters in infants and children.
Surg Gynecol Obstet 1989;169:203.
107. Dowd MD, Keenan HT, Bratton SL. Epidemiology and prevention of childhood injuries. Crit Care Med 2002;30(11 Suppl):S385.
108. Kapklein MJ, Mahadeo R. Pediatric trauma. Mt Sinai J Med 1997;64:302.
109. Osler TM, Vane DW, Tepas JJ, et al. Do pediatric trauma centers have better survival rates than adult trauma centers?
An examination of the National Pediatric Trauma Registry. J Trauma 2001;50:96.
110. Sanchez JL, Lucas J, Feustel PJ. Outcome of adolescent trauma admitted to an adult surgical intensive care unit versus
a pediatric intensive care unit. J Trauma 2001;51:478.
111. Khoshyomn S, Tranmer BI. Diagnosis and management of pediatric closed head injury. Semin Pediatr Surg 2004;13:80.
112. Adelson PD, Bratton SL, Carney NA, et al. Guidelines for the acute medical management of severe traumatic brain injury
in infants, children, and adolescents. Chapter 17. Critical pathway for the treatment of established intracranial hypertension
in pediatric traumatic brain injury. Pediatr Crit Care Med 2003;4(3 Suppl):S65.
113. Donati-Genet PC, Dubuis JM, Girardin E, et al. Acute symptomatic hyponatremia and cerebral salt wasting after head injury:
an important clinical entity. J Pediatr Surg 2001;36:1094.
114. Berkenbosch JW, Lentz CW, Jimenez DF, et al. Cerebral salt wasting syndrome following brain injury in three pediatric
patients: suggestions for rapid diagnosis and therapy. Pediatr Neurosurg 2002;36:75.
115. Pang D. Spinal cord injury without radiographic abnormality in children, 2 decades later. Neurosurgery 2004;55:1325.
116. Buhs C, Cullen M, Klein M, et al. The pediatric trauma C-spine: is the ‘odontoid’ view necessary? J Pediatr Surg 2000;35:994.
117. Browne GJ, Lam LT, Barker RA. The usefulness of a modified adult protocol for the clearance of paediatric cervical spine
injury in the emergency department. Emerg Med (Fremantle) 2003;15:133.
118. Lindner T, Bail HJ, Manegold S, et al. [Initial diagnosis after blunt abdominal trauma: a review of the literature].
Unfallchirurg 2004;107:892.
119. Albanese CT, Meza MP, Gardner MJ, et al. Is computed tomography a useful adjunct to the clinical examination for the
diagnosis of pediatric gastrointestinal perforation from blunt abdominal trauma in children? J Trauma 1996;40:417.
120. Fenton SJ, Hansen KW, Meyers RL, et al. CT scan and the pediatric trauma patient—are we overdoing it? J Pediatr Surg
2004;39:1877.
121. Coley BD, Mutabagani KH, Martin LC, et al. Focused abdominal sonography for trauma (FAST) in children with blunt abdominal
trauma. J Trauma 2000;48:902.
122. Miller MT, Pasquale MD, Bromberg WJ, et al. Not so FAST. J Trauma 2003;54:52.
123. Suthers SE, Albrecht R, Foley D, et al. Surgeon-directed ultrasound for trauma is a predictor of intra-abdominal injury
in children. Am Surg 2004;70:164.
124. Garcia VF, Gotschall CS, Eichelberger MR, et al. Rib fractures in children: a marker of severe trauma. J Trauma 1990;30:695.
125. Bisharat N, Omari H, Lavi I, et al. Risk of infection and death among post-splenectomy patients. J Infect 2001;43:182.
126. Stylianos S. Evidence-based guidelines for resource utilization in children with isolated spleen or liver injury. The
APSA Trauma Committee. J Pediatr Surg 2000;35:164.
127. Leinwand MJ, Atkinson CC, Mooney DP. Application of the APSA evidence-based guidelines for isolated liver or spleen injuries:
a single institution experience. J Pediatr Surg 2004;39:487.
128. Stylianos S. Compliance with evidence-based guidelines in children with isolated spleen or liver injury: a prospective
study. J Pediatr Surg 2002;37:453.
129. Cochran A, Mann NC, Dean JM, et al. Resource utilization and its management in splenic trauma. Am J Surg 2004;187:713.
130. Beierle EA, Chen MK, Whalen TV, et al. Free fluid on abdominal computed tomography scan after blunt trauma does not mandate
exploratory laparotomy in children. J Pediatr Surg 2000;35:990.
131. Griffet J, Bastiani-Griffet F, El-Hayek T, et al. Management of seat-belt syndrome in children: gravity of 2-point seat-belt.
Eur J Pediatr Surg 2002;12:63.
132. Beaunoyer M, St-Vil D, Lallier M, et al. Abdominal injuries associated with thoraco-lumbar fractures after motor vehicle
collision. J Pediatr Surg 2001;36:760.
133. Rogers LF. The roentgenographic appearance of transverse or chance fractures of the spine: the seat belt fracture. Am
J Roentgenol Radium Ther Nucl Med 1971;111:844.
134. Canty TG Sr, Weinman D. Management of major pancreatic duct injuries in children. J Trauma 2001;50:1001.
136. Keller MS, Stafford PW, Vane DW. Conservative management of pancreatic trauma in children. J Trauma 1997;42:1097.
137. Desai KM, Dorward IG, Minkes RK, et al. Blunt duodenal injuries in children. J Trauma 2003;54:640.
138. Chia JP, Holland AJ, Little D, et al. Pelvic fractures and associated injuries in children. J Trauma 2004;56:83.
139. Hall JR, Reyes HM, Meller JL, et al. The outcome for children with blunt trauma is best at a pediatric trauma center.
J Pediatr Surg 1996;31:72.
140. Harris BH, Stylianos S. Special considerations in trauma: child abuse and birth injuries. In: O’Neill JA, Rowe MI, Grosfeld
JL, et al, editors. Pediatric surgery. St. Louis: CV Mosby; 1998. p. 359.
141. Wu SS, Ma CX, Carter RL, et al. Risk factors for infant maltreatment: a population-based study. Child Abuse Negl 2004;28:1253.
142. Palazzi S, de Girolamo G, Liverani T. Observational study of suspected maltreatment in Italian paediatric emergency departments.
Arch Dis Child 2005;90:406.
143. Caffey J. Multiple fractures in the long bones of infants suffering from chronic subdural hematoma. Am J Radiol 1946;56:163.
144. American College of Surgeons Committee on Trauma. ATLS student course manual. Chicago: American College of Surgeons;
2004.