Surgery for ovarian masses in infants, children, and adolescents: 102 consecutive patients treated in a 15-year period.

BACKGROUND/PURPOSE: Ovarian pathology, although rare in children, must be included in the differential diagnosis of all girls who present with abdominal pain, an abdominal mass, or precocious puberty. METHODS: To improve clinical appreciation of these lesions, the authors reviewed the presentation, evaluation, and outcome of all patients with ovarian pathology surgically treated at their institution since 1985. RESULTS: One hundred two girls (aged 9.8 +/- 5.5 years; range, 2 days to 20 years) underwent 106 separate ovarian operations (43 salpingo-oophorectomies, 21 oophorectomies, 33 ovarian cystectomies, and 9 ovarian biopsies). Of those presenting with acute abdominal pain (n = 59), 25 (42%) had ovarian torsion (14 associated with a mature teratoma), and only 1 (2%) had a malignant tumor. In contrast, of those presenting with an abdominal mass (n = 23), 6 (26%) had malignancies. There was no age difference between those with benign disease (9.9 +/- 5.6 years; n = 96) and those with malignant tumors (8.6 +/- 3.9 years, n = 10). Nine children had 10 operations for presumed malignant tumors (3 dysgerminomas, 2 immature teratomas with foci of yolk sac tumor, 2 juvenile granulosa cell tumors, 1 yolk sac tumor, and 1 Sertoli-Leydig cell tumor). These patients all had unilateral salpingo-oophorectomy, 4 had chemotherapy, and all are now disease free at 8.4 +/- 4.1 years follow-up. CONCLUSION: Ovarian pathology remains a rare indication for surgery in girls less than 20 years of age. Because most of these lesions are benign, ovarian-preserving operations should be performed whenever feasible.

Right lower quadrant pain in children caused by omental infarction.

BACKGROUND: Omental infarction is a rare cause of abdominal pain, with fewer than 300 cases reported in the literature. Only 15% of reported cases occur in the pediatric population. We present our experience with 18 children diagnosed with omental infarction admitted to Texas Children's Hospital over a 15-year period. STUDY DESIGN: A retrospective review of clinical records, diagnostic images, and pathologic findings was carried out for all children diagnosed with isolated omental infarction from 1986 to 2000 in order to analyze presenting signs and symptoms, preoperative imaging, surgical management, and postoperative outcomes. RESULTS: Eighteen children were treated for isolated omental infarction. There were 12 boys and 6 girls with an average age of 7.5 years (range 2 to 13). All patients presented with acute onset of right lower quadrant pain. Only 5 of 18 (24%) had associated gastrointestinal symptoms. The average temperature at presentation was 99.4 degrees F (+/- 0.78). The average white blood cell count was 11.4 (+/- 4.4). Fourteen patients had ultrasonographys performed preoperatively: 6 of 14 incorrectly diagnosed appendicitis, 4 of 14 were nondiagnostic and 4 of 14 correctly diagnosed omental infarction. Two of 18 patients underwent computed tomography scans, which were diagnostic for omental infarction. Resection of the infarcted omentum was performed in all patients and appendectomy in 16 of 18. Thirteen patients underwent an open procedure, 5 were performed laparoscopically. There were no postoperative complications. All patients had resolution of pain postoperatively and were discharged an average of 3.0 (+/- 0.9) days after admission. CONCLUSIONS: Omental infarction is an uncommon cause of right lower quadrant pain in children and is often diagnosed as appendicitis preoperatively. Ultrasonography and computed tomography can be diagnostic. Surgical resection of the infarcted omentum results in immediate resolution of pain with no morbidity.

A longitudinal analysis of the pediatric surgeon workforce.

OBJECTIVE: To describe the trends in the pediatric surgeon workforce during the last 25 years and to provide objective data useful for planning graduate medical education requirements. SUMMARY BACKGROUND DATA: In 1975, the Study on U.S. Surgical Services (SOSSUS) was published, including a model to survey staffing. A pediatric surgeon workforce study was initiated in conjunction with SOSSUS as a population, supply, and need-based study. The study has been updated every 5 years using the same study model, with the goals of determining the number and distribution of pediatric surgeons in the United States, the number needed and where, and the number of training programs and trainee output required to fill estimated staffing needs. This is the only such longitudinal workforce analysis of a surgical specialty. METHODS: Questionnaires were sent to 100 pediatric surgeons representing the 62 standard metropolitan statistical areas (SMSAs) in the United States with a population of 200,000 or more to verify the names and locations of all active pediatric surgeons and to gain information about the 5-year need for new pediatric surgeons by region. A program was developed to predict the number of pediatric surgeons relative to the total population and the 0-to-17-year-old population in the subsequent 30 years using updated data on the present number and ages of pediatric surgeons, age-specific death and retirement rates, projections of U.S. population by age group, and varying numbers of trainees graduated per year. As each 5-year update was done, previous projections were compared with actual numbers of pediatric surgeons found. The trends during the last 25 years were analyzed and compared and additional information regarding the demographics of practice, trends in reimbursement, and volume and scope of surgery was obtained. RESULTS: The birth rate has been stable since 1994. The 0-to-17-year-old population has been increasing at 0.65% per year; a 0.64% annual rate is projected to 2040. At present, 661 pediatric surgeons are distributed in every SMSA of 200,000 or more population, with an average age of 45 and an average age of retirement 65. The actual number of pediatric surgeons in each 5-year survey has consistently validated previous projections. Trainee output has increased markedly in the past 10 years. The rate of growth of the pediatric surgeon workforce at present is 50% greater than the forecasted rate of increase in the pediatric age group, and during the past 25 years the rate of growth of the pediatric surgeon workforce has been double that of the pediatric population growth. Nationally, significant changes in reimbursement, volume of surgery, and demographics of practice have occurred.

Hemangioma of a thoracic sympathetic ganglion mimicking a neuroblastoma.

An infant presented with an asymptomatic paraspinal thoracic mass. Chest radiograph and CT scan supported the diagnosis of neuroblastoma, as did the intraoperative gross appearance and location of the mass, which involved the sympathetic chain. However, histologic diagnosis confirmed an isolated hemangioma of the sympathetic ganglion, which has not been reported previously. Hemangioma should be considered in the differential diagnosis of all mediastinal masses.

Intestinal button implantation for obstipation and fecal impaction in children.

Chronic obstipation and fecal impaction in children can be difficult management problems for pediatricians. We describe a novel approach to the management of obstipation and fecal impaction: the implantation of the button gastrostomy device in the appendiceal stump or the terminal ileum. We report the procedure in two children. One child had pseudoobstruction syndrome complicated by recurrent obstipation; the other had cystic fibrosis complicated by recurrent obstruction from meconium ileus equivalent. Both children received a polyethylene glycol/electrolyte solution, with or without pancreatic enzymes, which was administered through the button. The children have remained essentially asymptomatic for at least 10 months. We believe that this is the first report of the use of a button gastrostomy device to successfully manage chronic obstipation and recurrent fecal impaction in children.

Multiple endocrine syndrome type IIb in early childhood.

A 3-week-old girl who was born with club feet had signs of failure to thrive. On physical examination the child appeared normal; she had no abnormalities in the mucous membranes of the mouth, the eyelids, or in the neck, and her other systems, including heart, chest, abdomen, and neurologic systems, were clinically normal. Radiologically, the gastrointestinal tract was normal, but rectal biopsy showed neuromas. Her serum calcitonin level was measured both at basal and after pentagastrin stimulation at 5 weeks of age and found to be high, but whether it was consistent with the normal level at this early age or was caused by medullary thyroid carcinoma was not clear. At 3 months, the corneal nerves of both eyes were examined and showed considerable thickening, and multiple endocrine syndrome type IIb was suspected. The serum calcitonin level at 8 and 14 months was increased. A total thyroidectomy was done, and C-cell nodular hyperplasia and adenomatosis was found in the isthmus. The postoperative serum calcitonin level decreased to low normal and did not increase after pentagastrin stimulation. To the authors' knowledge, this case represents the youngest patient diagnosed with multiple endocrine syndrome type IIb in the absence of family history of the disease.

Colonic interposition after esophagogastrectomy in a child with Zollinger-Ellison syndrome.

Zollinger-Ellison syndrome is a rare childhood entity that usually presents with peptic ulcer disease and is resistant to medical management. In contrast to the treatment of the disease in adults, total gastrectomy is the procedure of choice for pediatric patients. We report on a child with Zollinger-Ellison syndrome complicated by esophageal stricture in whom we performed a new operative procedure using a colonic interposition. The use of the procedure has obviated the side effects commonly associated with gastrectomy.

Hemoperitoneum from perforated Meckel's diverticulum.

Approximately 4% of patients with Meckel's diverticulum will experience complication, the most common of which are intestinal obstruction, gastrointestinal bleeding, acute inflammation, and perforation. We report an extremely rare complication of perforated Meckel's diverticulum which presented as hemoperitoneum.

Massive upper gastrointestinal hemorrhage in an infant following cardiac surgery.

This is a case of massive upper gastrointestinal hemorrhage and hematoma formation in a 32-day-old infant following uneventful repair of congenital heart disease. Stress ulcers and gastrointestinal hemorrhage are rare complications of cardiac surgery in the young. Conservative management often fails and early surgery can be lifesaving. The etiology of this problem remains unknown and more work is needed so that effective preventive measures can be developed.

Bronchogenic cysts above and below the diaphragm: report of eight cases.

Bronchogenic cysts are developmental foregut anomalies usually located within the mediastinum. Although typically asymptomatic, infection or compression of adjacent structures may become prominent. Surgical excision is recommended to establish diagnosis, alleviate symptoms if present, and prevent future complications. We report our recent experience with bronchogenic cysts including 7 located within the mediastinum and a rare instance of a cyst below the diaphragm.

Emergency gastrotomy: treatment of choice for iron bezoar.

The authors believe that gastrotomy and surgical removal of tablets is the treatment of choice in massive iron ingestion with development of an "iron bezoar" unresponsive to gastric lavage. Prompt surgical treatment in such cases may be lifesaving.

Experience with peritoneo-venous shunting for congenital chylous ascites in infants and children.

The management of chylous ascites presenting in association with primary lymphedema of the limbs and possibly lymphatic malformation in the lungs is difficult when the increasing abdominal distension causes respiratory distress. Laparotomy may be useful in traumatic chylous ascites or in intestinal lymphangiectasia localized to a segment of the bowel. It would seem that when conservative management such as diuretics, diet, and repeated abdominal paracentesis do not improve the respiratory distress, a peritoneo-venous shunt is logical. This report of two patients with severe chylous ascites and generalized lymphatic malformations causing or accentuating respiratory distress, stresses the possibility that peritoneo-venous shunts may not have the same long term function as seen in cirrhotic ascites. Long-term follow-up on the use of these shunts specifically for chylous ascites is not available.

Congenital posterolateral diaphragmatic hernia: a retrospective study.

Congenital posterolateral diaphragmatic hernia continues to be associated with a high mortality from ventilatory failure. To evaluate the malformation and factors associated with survival, 43 children with congenital diaphragmatic hernia seen in the neonatal period were studied; 20 survived. The male:female ratio was 2.1. No consistent antenatal factors could be implicated, and, except for intestinal malrotation and lung hypoplasia, associated anomalies were uncommon. The most common cause of death was ventilatory failure and marked bilateral lung hypoplasia was a common autopsy finding. Most of those died were admitted within 8 hours of birth. Tolazoline has been used in 10 patients since 1975 and, of these, 4 survived. Postoperatively a transient period of satisfactory lung function correlated well with response to vasodilators, which may be of value in future therapy.

Congenital valvular aortic stenosis: surgical management and long-term results.

Case histories of 50 consecutive infants and children ith congenital valvular aortic stenosis treated at The Montreal Children's Hospital during the past 16 years were reviewed in order to determine the efficacy of the initial treatment as well as the long-term results. The operative technique employed was to incise fused commissures out almost to the anulus without causing aortic insufficiency, but often the accomplishment was limited by valve anatomy or cusp dysplasia. Six of the seven operative deaths occurred in infants. Eight to 16 year follow-up on the first 25 survivors revealed one late death, four "good" results, five aortic valve replacements, and three second valvotomies. Ten patients have recurrent aortic stenosis and two have moderate aortic insufficiency. Aortic valvotomy is a palliative operation, and about a third of the children operated upon will require a second operation within 10 years.

Congenital diaphragmatic hernia: pathophysiology and pharmacologic support.

The incidence of death from congenital diaphragmatic hernia appears to be unchanged in recent years despite advances in resuscitation, transport, and ventilatory support. Bilateral lung hypoplasia and abnormal pulmonary vascular reactivity as developmental consequences of the defect appear to play a major role in the continued high mortality rate. Recent advances in pharmacologic support have further elucidated the mechanisms of ventilatory failure in these patients and may represent means of improving survival in the future.

Vasodilator response and prediction of survival in congenital diaphragmatic hernia.

In an effort to find an accurate prediction of survival in congenital diaphragmatic hernia (CDH) patients with severe respiratory distress and persistent pulmonary hypertension, we reviewed the response to vasodilators and te clinical course of 13 patients with this condition. All patients had been operated upon within the first 24 hr of life and had postoperative descending aortic PaO2 values less than or equal to 50 torr on maximum ventilatory support. The only significant change in standard blood gas values after an intravenous test dose of 2 mg/kg of tolazoline was in the PaO2 (p less than 0.05). Patients with good response (PaO2 increase greater than 100 torr) survived. Patients with fair response (PaO2 increase between 20 and 100 torr) had equivocal survival. Patients with poor response (PaO2 increase less than 20 torr) died. There was a postoperative period up to several weeks of marked fluctuations in arterial oxygen tension in survivors before stability occurred. Vasodilator responsiveness appears to be an accurate, therapeutic indicator of the chances for survival in CDH patients with persistent pulmonary hypertension and right to left shunting.

Pancreaticojejunostomy for fulminating pancreatitis and pancreatic ascites in a Jehovah's Witness.

Pancreatitis is generally restricted to adults, most often caused or aggravated by alcoholism or biliary tract disorders, and rarely develops in children. When the diagnosis is made, the patient usually can be treated medically; however, the disease often has nonspecific early symptoms and is not discovered until potentially fatal complications have ensued. We present an unusual case of severe posttraumatic pancreatic ascites in an anemic 3-yr-old child whose parents were of the Jehovah's Witness faith. She underwent successful surgical treatment involving distal pancreaticojejunostomy with preservation of the spleen.

Tolazoline therapy for persistent pulmonary hypertension after congenital diaphragmatic hernia repair.

To determine whether vasodilators are useful in persistent pulmonary hypertension associated with congenital diaphragmatic hernia, we reviewed the clinical course, laboratory data, and outcome of 37 patients with respiratory distress and diaphragmatic hernia requiring an operation before 24 hours of life. These patients were divided into two groups, Group I (n = 17) included patients treated prior to the use of tolazoline; Group II (n = 20) included those treated after tolazoline became available. Postoperative severe respiratory distress was observed in ten patients in Group I, and all died. In Group II, 16 patients had severe postoperative respiratory distress and four survived; 12 of these 16 patients received tolazoline, including all four survivors. Treated survivors had significantly higher increase in Pao2 after a test dose of tolazoline than did nonsurvivors. A transient "honeymoon period" of adequate oxygenation correlated with good response to tolazoline, and the presence of both was predictive of survival. No patient survived with the combination of no "honeymoon period" and no response to tolazoline, whereas response to tolazoline without a honeymoon period was sometimes followed by survival. All nonsurvivors had severe lung hypoplasia at autopsy.

Carcinoma of the stomach in the young adult.

Of 1,383 patients with carcinoma of the stomach seen at this institution during a 30 year interval, 37 were 35 years of age or younger. Fourteen of the patients were less than 30 years of age and the females predominated. While all young adults underwent roentgenologic examination of the upper gastrointestinal tract, only 12 had findings considered diagnostic for a malignant lesion of the stomach at the time of the initial study. Endoscopy with biopsy was done in 16 patients and confirmed the diagnosis of carcinoma in 13 patients. Carcinoma of the stomach was first diagnosed at laparotomy in 13 patients. Only 30 of 37 patients were considered operative candidates. Of the gastric resections done, 19 were curative and two, palliative. Seventy-six per cent of the lesions were either linitis plastica or poorly differentiated adenocarcinomas, and lymph node metastases were present in 31 patients. Prognosis was dismal. Thirty-one patients were dead within two years of diagnosis and only two patients have survived five years. In this series, carcinoma of the stomach in young people was particularly lethal. A high index of suggestive symptoms should prompt an early, aggressive roentgenologic and endoscopic evaluation of the upper gastrointestinal tract if any chance for an increase in cure rates is to be realized.