Embryonal rhabdomyosarcoma in a Rothschild's giraffe (Giraffa camelopardalis rothschildi).

Abstract: A 3-yr-old male Rothschild's giraffe (Giraffa camelopardalis rothschildi) presented for acute swelling caudomedial to the left parietal horn. Following initial diagnostics and supportive treatment, the mass was surgically resected and intralesional chemotherapy was administered. Despite treatment, the giraffe's condition worsened and euthanasia was performed. Gross necropsy revealed neoplastic invasion and destruction of underlying parietal bone, adjacent horn base, and sinuses, and metastases in the tracheobronchial and mandibular lymph nodes and lung. Histologically, the tumor was composed of packets of anaplastic round cells. Immunohistochemical studies further characterized the tumor as an embryonal rhabdomyosarcoma. This is the first reported case of rhabdomyosarcoma in a giraffe.

Pediatric surgery workforce: supply and demand.

INTRODUCTION: Recent studies report a shortage of pediatric surgeons in the United States. We surveyed members of the American Pediatric Surgical Association (APSA) to estimate current workforce and demand and to provide data for workforce planning. METHODS: We conducted a survey of 849 APSA members to provide workforce data on their communities as follows: the number of active, retired, or inactive APSA surgeons; non-APSA fellowship graduates; surgeons without accredited fellowship training; and the estimated demand for additional pediatric surgeons. Internet search engines identified surgeons and practices offering pediatric surgical services. The US Census Metropolitan Statistical Areas (MSAs) defined service areas with populations of 100,000 or more. RESULTS: Of 137 MSAs with APSA members in practice, we obtained data from 113 (83%), with 247 (29%) of 849 surgeons responding. We estimate that the current pediatric surgical workforce consists of 1150 surgeons, with APSA members in active practice (60%) forming the single largest group, followed by general surgeons (21%). The percentage of active APSA surgeons was greater than the percentage of general surgeons in the 50 largest MSAs (76% vs 2%, respectively), whereas the opposite was observed in the smaller MSA ranked more than 51 in population (37% vs 46%, respectively). American Pediatric Surgical Association respondents estimated a national demand for 280 additional pediatric surgeons. Active APSA surgeons plan to delay retirement (8% of respondents) because it would leave their group or community shorthanded; 2% reported that retirement would leave the community without a pediatric surgeon. DISCUSSION: Workforce shortage in pediatric surgery is a problem of number and distribution. Incentives to direct trainees to underserved areas are needed. General surgeons provide pediatric services in many communities. Surgical training should include additional training in pediatric surgery.

Clinical and financial characteristics of pediatric surgery practices.

UNLABELLED: Pediatric surgical practices face many challenges. We wanted to define the clinical practice and financial support among different types of practices as follows: academic, private practice, and employed. METHODS: This study involved an Internet survey of members of the American Pediatric Surgical Association (APSA), comparisons using chi(2) and paired t test analyses. RESULTS: The response rate was 28.7% (233/811), 145 academic, 48 private, and 40 employed. More than 90% received partial to full financial hospital support. Only 7.3% received no outside support, most frequently those in private practices (16.7%; P = .016). More than 90% had resident or fellow coverage. Nearly all practices covered newborn conditions and solid tumors, with differences in pediatric trauma, patent ductus arteriosus, and urologic condition. Transfer out of community was low but increased for specific conditions during the respondents' absence, from 0.4% to 5.2% to 3.4% to 6.9% (P = .001-0.003). A minority of respondents noted that nonpediatric surgeons treated selected pediatric conditions in their communities as follows: inguinal hernia (38.4%), umbilical hernia (42.6%), abscesses (37.5%), and trauma (36.6%). Pediatric surgeons shared call within their group in 86.3%, whereas 5.6% took call alone. Many restricted call by excluding trauma (37.2%), soft tissue infections or appendectomies (21.3% for both), and older children (>12 years, 23.8%). Nearly one fifth (18.9%) expressed interest in having an APSA surgeon serve as a locum tenens in their practices. DISCUSSION: Many pediatric surgeons receive both financial and in-kind subsidies. Although they cover a wide breadth of surgical conditions, many limit the conditions that they treat to reduce call responsibilities. The workforce shortage in pediatric surgery creates call coverage problems that may affect up to 8% of US practices.

Interobserver variation in the assessment of appendiceal perforation.

BACKGROUND: Following an appendectomy, surgeons define appendicitis, for treatment and billing purposes, into one of four categories: normal appendix, acute appendicitis, gangrenous appendicitis, and perforated appendicitis. Treatment of appendicitis is predicated upon classification at the time of visual inspection. Further, this classification often plays a role in the assessment of hospital outcomes. The currently accepted classification system is based solely upon intraoperative surgeon opinion and not objective data. Inconsistent surgeon grading of the severity of appendicitis may have implications in both management and outcomes. OBJECTIVE: The aim of this study was to assess the interobserver variation among surgeons in grading of the inflammatory severity of acute appendicitis as recognized on visual findings at operation. METHODS: A cross-sectional study design. 110 surgeons, and surgical residents were randomly selected. Surgeons were shown images of intraoperative appendicitis and were asked to evaluate the severity of the appendicitis (i.e., normal, inflamed, gangrenous, and perforated). Demographic information regarding the type of practice, hospital setting, and the number of encounters with patients with acute appendicitis were assessed. An Intraclass Correlation Coefficient score, represented by R, was calculated to assess interobserver reliability in grading the inflammatory severity of acute appendicitis. The two-way analysis of variance procedure for multivariate analysis was used for this calculation. RESULTS: The study group consisted of 100 surgeons, 62 practicing surgeons, and 48 surgical trainees. Overall, 79% of the surgeons treated predominantly adults with appendicitis, 18% treated primarily children, and 3% treated both children and adults. Hospital practices included university hospitals (47%), community hospitals (33%), children's hospitals (14%), and others (6%). Overall, there was poor agreement among surgeons in assessing the severity of appendicitis. Among all attending surgeons, the agreement of defining an image as to whether it was perforated or not was 27% (R4 = 0.27). Completion of a general surgery residency did improve the interobserver agreement, when compared with trainees. CONCLUSION: There is poor agreement among surgeons in describing the severity of appendicitis. Treatment protocols based on more accurate assessment and categorization could potentially lead to more favorable, cost-effective outcomes. Further, studies determining efficacy in the diagnosis and treatment of appendicitis should consider observer variability. Future work must attempt to define critical objective assessment points, such as visible discontinuity of the appendix or fecal soilage, to assure a better correlation of findings with prognosis.

Pediatric surgery workforce: population and economic issues.

BACKGROUND: Whether a shortage of pediatric surgeons exists in the United States, such as those observed in the total physician and general surgical workforces, is an important issue that will affect decisions regarding training, credentialing, and reimbursement. Our goal was to update information regarding the demand and supply of pediatric surgeons. METHODS: Online American Pediatric Surgical Association (APSA) membership directory gave numbers of pediatric surgeons and their residence by metropolitan statistical areas (MSA), defined by the US census. Population and economic data were obtained from appropriate US government agencies. RESULTS: There were 835 APSA members and 375 MSA. Eliminated were 86 MSA (with 12 APSA members) with incomplete data, 14 MSA (0 members) with populations less than 100,000, and 25 members with listed locations outside an MSA. The remaining 798 members and 275 MSA comprised the study. The number of APSA members in an MSA correlated closely with MSA population (R(2) = 0.836) and 2006 births (R(2) = 0.767). Metropolitan statistical areas without an APSA member had a smaller population and birth rate than those with one or more members (P = .0001). An MSA with 1 APSA member had a higher population (P = .0003) and births per APSA member ratios (P = .0014) than MSA with 2 and 3 or more members. The presence of a medical school or a pediatric training program had no effect on population or births-to-APSA member ratios. There was no correlation between numbers of APSA members and state GDP or state GDP per capita. We used a low, medium, and high threshold to predict the need for pediatric surgeons based upon population per APSA member +/- 1 SD (272,466 +/- 163,386) to predict a need of 82 to 1344 pediatric surgeons, an increase in the APSA membership by 10% to 168%. CONCLUSION: Based on population estimates and APSA membership, a current shortage of pediatric surgeons exists. Measures should be taken to address this workforce issue.

Incidence of postoperative hyponatremia and complications in critically-ill children treated with hypotonic and normotonic solutions.

OBJECTIVE: To determine the incidence and clinical consequences of postoperative hyponatremia in children. STUDY DESIGN: We performed a retrospective analysis of postoperative admissions to the pediatric intensive care unit (excluding cardiac, neurosurgical, and renal). The incidence of severe (serum sodium < 125 mmol/L or symptoms) and moderate (serum sodium < 130 mmol/L) hyponatremia in children receiving hypotonic (HT) and normotonic (NT) fluids was calculated. RESULTS: Out of a total of 145 children (568 sodium measurements; 116 HT and 29 NT), we identified 16 with hyponatremia (11%). The incidences of moderate (10.3% vs 3.4%, P = .258) and severe (2.6% vs 0%; P = .881) hyponatremia were not significantly different in the HT and NT groups. There were no neurologic sequelae or deaths related to hyponatremia. CONCLUSIONS: In our study group, hyponatremia was common, but morbidity and death were not observed. Careful monitoring of serum sodium level may be responsible for this lack of adverse outcomes. Larger, prospective studies are needed to determine whether the incidence of hyponatremia differs between the HT and NT groups.

Current management of appendicitis.

Appendicitis is the most common surgical disease of the abdomen in children. Pediatric appendicitis varies considerably in its clinical presentation, contributing to delay in diagnosis and increased morbidity. The methods of diagnosis and treatment of appendicitis also vary significantly among clinicians and medical centers according to the patient's clinical status, the medical center's capabilities, and the physician's experience and technical expertise. Recent trends include the increased use of radiologic imaging, minimally invasive and nonoperative treatments, shorter hospital stays, and home antibiotic therapy. Little consensus exists regarding many aspects of the care of the child with complicated appendicitis. This article examines the most debated aspects of the diagnosis and management of the diseased pediatric appendix.

Seminars: controversies in the management of pediatric thyroid malignancy.

Thyroid cancer in children is a rare malignancy with unusual biological behavior. It often presents at advanced stages, yet behaves in a benign manner, when compared to its adult counterpart. Surgeons have debated the risks and benefits of aggressive surgical procedures for over a decade. A consensus treatment plan for childhood thyroid cancer has not been achieved, however radical surgical procedures have, in general, fallen out of favor. The best operative and adjuvant strategy for these children can only be determined when a better understanding of the tumor cell biology and genetics of this cancer is known. This review discusses the current controversies in the management of differentiated childhood thyroid malignancy.

Analysis of head injury admission trends in an urban American pediatric trauma center.

BACKGROUND: Head injury is the leading cause of death in children. Child safety legislation and risk-specific intervention programs have flourished to mitigate the incidence of injury to children. This analysis documents the trend in head injury to children in a specific institution. METHODS: Analysis of 5,003 head injury admissions to a pediatric trauma center over thirteen years was performed. Admission rates were calculated using the appropriate population denominator from census data. Poisson regression analysis was applied to estimate the relative risk of head injury admission by year in different age, sex, mechanism of injury and severity group. RESULTS: There has been a 70% decline in the head injury admission rate since 1989, consistent with regional and national data. The decline was present in all mechanisms of injury and age groups except for less than 1 year of age. The decline in total trauma admissions over the same time period was 50% and the decline in total hospital admission was 10%. CONCLUSION: Pediatric head injury has significantly declined in the last 13 years at a Level I pediatric trauma center.

Hospital- and patient-level characteristics and the risk of appendiceal rupture and negative appendectomy in children.

CONTEXT: The rates of appendiceal rupture and negative appendectomy in children remain high despite efforts to reduce them. Both outcomes are used as measures of hospital quality. Little is known about the factors that influence these rates. OBJECTIVE: To investigate the association between hospital- and patient-level characteristics and the rates of appendiceal rupture and negative appendectomy in children. DESIGN, SETTING, AND PATIENTS: Retrospective review using the Pediatric Health Information System database containing information on 24,411 appendectomies performed on children aged 5 to 17 years at 36 pediatric hospitals in the United States between 1997 and 2002. MAIN OUTCOME MEASURES: Rates of negative appendectomy and appendiceal rupture; the odds ratio (OR) of negative appendectomy and appendiceal rupture by hospital, patient age, race, and health insurance status, and hospital fiscal year and appendectomy volume. Negative appendectomy rate was defined as the number of patients with appendectomy but without appendicitis divided by the total number of appendectomies. RESULTS: The median negative appendectomy rate was 3.06% (range, 1%-12%) and the median appendiceal rupture rate was 35.08% (range, 22%-62%). The adjusted OR for appendiceal rupture was higher in Asian children (1.66; 95% confidence interval [CI], 1.24-2.23) and black children (1.13; 95% CI, 1.01-1.30) compared with white children. Children without health insurance and children with public insurance had increased odds of appendiceal rupture compared with children who had private health insurance (adjusted OR, 1.36; 95% CI, 1.22-1.53 for self-insured; adjusted OR, 1.48; 95% CI, 1.34-1.64 for public insurance). No correlation existed between negative appendectomy rate and race, health insurance status, or hospital appendiceal rupture rate. The negative appendectomy rate improved as the hospital appendectomy volume increased. CONCLUSION: The rate of appendiceal rupture in school-aged children was associated with race and health insurance status and not with negative appendectomy rate and therefore is more likely to be associated with prehospitalization factors such as access to care, quality of care, and patient or physician education.

Spectrum of intrapartum management strategies for giant fetal cervical teratoma.

BACKGROUND/PURPOSE: The management of the fetus with a large neck mass that obstructs the airway remains a clinical challenge. The authors review their experience with giant fetal cervical teratoma and discuss options for management. METHODS: A retrospective review of all patients referred since 1994 for prenatal management of a fetal neck mass was performed. Variables examined included gestational age at diagnosis and delivery, size and location of the neck mass, presence of fetal hydrops, associated anomalies, management methods, operating time, and outcome. RESULTS: Seven patients were identified with a prenatal diagnosis of giant cervical teratoma. Four patients had fetal hydrops; of these, 2 died in utero of hydrops, and a third fetus underwent elective termination. The remaining hydropic and previable fetus underwent fetal surgery for resection of the mass. The 3 nonhydropic patients underwent ex utero intrapartum treatment (EXIT) procedures for airway control. Endotracheal intubation was possible in one patient, and one received a tracheostomy. In the third fetus, neither intubation nor tracheostomy were possible, and resection of the neck mass was performed on placental support. There were no deaths in the surgical group. CONCLUSIONS: The management of fetal giant cervical teratoma includes a spectrum of options. For the rare previable fetus with hydrops, fetal resection may be indicated. In patients with airway obstruction, EXIT procedure provides the luxury of time to obtain airway control either by intubation, tracheostomy, or, if necessary, tumor resection on placental support.

Laparoscopy is useful in the diagnosis and management of foramen of Morgagni hernia in children.

Foramen of Morgagni hernia is a rare form of congenital diaphragmatic hernia. We report the use of laparoscopy to diagnose and manage suspected foramen of Morgagni hernias in children. Cardiophrenic masses were seen on chest radiography in two asymptomatic children aged 1 and 3 years. Further radiologic workup was nondiagnostic. Laparoscopy was performed in both children, and the foramen of Morgagni hernias were immediately identified. Laparoscopic repair was performed on one of the children. Both children had uneventful postoperative courses, were discharged home early, and had no evidence of recurrence at the 6-month follow-up examination. Laparoscopy is a safe and effective technique to diagnose and potentially repair foramen of Morgagni hernias. We recommend the use of this modality as the primary diagnostic evaluation in infants and children with suspected foramen of Morgagni hernias.

The diagnosis and management of endocrine tumors causing hypertension in children.

In contrast to that in adults, hypertension in children is frequently amenable to surgical therapy. With advancing techniques in imaging, surgery, and anesthesia, the outcomes of surgery are excellent for children with endocrine tumors causing hypertension.

Classical positioning decreases subclavian vein cross-sectional area in children.

BACKGROUND: Recommendations for subclavian vein catheter placement in children are extrapolated from adult experience. The purpose of this study was to determine the ideal body position to optimize the size of the subclavian vein in children for percutaneous catheter placement. METHODS: Children underwent ultrasound imaging of the subclavian vein in four supine body positions: head in a neutral position with the chin midline (NL) and no shoulder roll (SR); head turned 90 degrees away (TA) and no SR; head NL with an SR; and head TA with an SR. The cross-sectional area (CSA) of the subclavian vein was calculated and statistical significance was determined using the Student's t test and the Wilcoxon signed rank test. RESULTS: Nine children participated in the study, with a mean age of 5.3 years. The CSA of the subclavian vein was 0.39 +/- 0.24 cm2 with the head NL and no SR, compared with 0.31 +/- 0.20 cm2 with the head TA or 0.32 +/- 0.23 cm2 with the head TA and SR. This represented a significant reduction in the CSA of the subclavian vein by 22% and 18%, respectively (p < 0.05). CONCLUSION: In children, the recommended maneuvers of turning the head or turning the head and placing a posterior shoulder roll significantly reduce the cross-sectional area of the subclavian vein. Maintaining the head in a normal position with the chin midline without a shoulder roll optimizes subclavian vein size. Positioning children in this manner may serve to reduce the morbidity associated with percutaneous subclavian vein cannulation.

Routine MRI evaluation of low imperforate anus reveals unexpected high incidence of tethered spinal cord.

BACKGROUND/PURPOSE: Previous clinical practice has included evaluation for the presence of tethered cord in those children who have imperforate anus with a high lesion. To define the incidence in children with low lesions, the authors reviewed their experience with a protocol employing routine magnetic resonance imaging (MRI), regardless of the level of the lesion, to determine the presence of a tethered cord in all children with imperforate anus. METHODS: A retrospective review of children with imperforate anus was conducted over the last 13 years at our institution. Lesions were categorized as high versus low based on the supralevator or infralevator position of the fistula. RESULTS: Sixty-three patients completed evaluation for a tethered cord. Twenty-two (34.9%) of these 63 patients had a tethered cord: 11 of 41 (26.8%) patients with high lesions and 11 of 22 (50.0%) of those with low lesions. Of those children with a low lesion, 83% of the boys had a tethered cord, whereas 38% of the girls had a tethered cord. Forty-five percent of the patients with low lesions and a tethered cord did not have any other lumbosacral anomalies. All 22 children with a tethered cord underwent surgical release. CONCLUSIONS: The incidence of tethered cord in children with low lesions of imperforate anus is not lower than those with high lesions. The authors advocate early evaluation of all children with imperforate anus for a tethered cord.

Principles for the surgical management of patients with Proteus syndrome and patients with overgrowth not meeting Proteus criteria.

BACKGROUND: Proteus syndrome is a rare, sporadic disorder consisting of disproportionate overgrowth of multiple tissues, vascular malformations, and connective tissue or epidermal nevi. Patients with Proteus syndrome present with diverse and variable phenotypes because of the syndrome's mosaic pattern of distribution. METHODS: Eighty patients with Proteus syndrome, satisfying published diagnostic criteria, and 51 patients with overgrowth not meeting Proteus criteria were identified from the literature. Three additional patients, one patient with Proteus syndrome and 2 patients with overgrowth, were treated at the author's institutions and are discussed in detail. All nonorthopedic and noncutaneous surgical interventions were reviewed. RESULTS: Fourteen genitourinary, 9 gastrointestinal, and 5 otolaryngologic operations were performed on patients with Proteus syndrome. Six genitourinary, 5 gastrointestinal, and 2 otolaryngologic operations were performed on patients with overgrowth not meeting Proteus criteria. Eight patients with Proteus syndrome and 4 patients with overgrowth experienced thoracic manifestations, generally diffuse cystic pulmonary lesions, but only 1 of 12 underwent surgical treatment. CONCLUSIONS: Patients with visceral manifestations of either Proteus syndrome or overgrowth not meeting Proteus criteria should be treated in a similar manner. Lesions involving the ovaries and testes, because of the high incidence of neoplasm, should be managed aggressively. Gastrointestinal and renal lesions may be managed conservatively with frequent follow-up to minimize abdominal explorations. All patients undergoing surgery should have a thorough preoperative assessment of their airway and pulmonary reserve because of the relatively high frequency of tonsillar hypertrophy and pulmonary cystic involvement.

Guiding practice improvements in pediatric surgery using multidisciplinary clinical pathways.

Clinical pathways guide the evaluation and care of patient populations and can advance the strategic initiatives of the organization as well. To meet external and internal demands, this organization developed pathways to reduce variability in practice, integrate available evidence into care delivery, improve care coordination and documentation, increase efficiencies, and provide outcomes data to consumers and payers. The authors highlight processes and outcomes of some of the organization's surgical pathways, including guidelines for children with second degree burns grafted with TransCyte, those children hospitalized for tonsillectomy or adenoidectomy, appendectomies, atrial and ventricular septal defects, and percutaneous endoscopic gastrostomy or surgical gastrostomy procedures.