Arthur J. Ross

Ovarian masses in children: A review of 91 cases of malignant and benign masses

Ovarian masses in children are uncommon. We reviewed all cases of ovarian masses presenting to this hospital from 1979 to 1990. Ninety-one patients fulfilled the criteria and had medical records available. All patients were less than 18 years old. Four were diagnosed antenatally. Thirty-four tumors presented prior to 8 years of age and 1 (2.9%) was malignant. Fifty-eight tumors presented after 8 years of age and 18 (33%) were malignant. Seventy-two patients had benign disease and 19 had malignant tumors. Of those with benign disease 22 had simple or epithelial cysts, 25 had teratomas, 13 had torsion with cyst formation, 3 had granulosa cell tumors, and 9 had other less common lesions. Analysis of symptoms could not distinguish between benign and malignant lesions; however, age was less (P < .03) and tumor size smaller (P < .001) in patients with benign lesions. Benign lesions presented at a mean age of 8.8 years. Fifty-four patients had an ultrasound, all were diagnostic: simple mass (14), complex mass (8), or cyst (32). Mean size of the masses was 9.5 x 7.7 cm. Fourteen patients had a contralateral ovarian cyst. The malignant lesions included 14 germ cell tumors (4 endodermal sinus, 4 teratoma, 2 choriocarcinoma, 2 dysgerminoma, 1 embryonal, and 1 mixed), 4 epithelial tumors (1 mucinous cystadenocarcinoma, papillary cystadenocarcinoma, papillary serous cystadenocarcinoma, and endometrioid adenocarcinoma), and one patient with leukemic infiltration (ALL). Germ cell tumors presented at a mean age of 11.8 years. Eight of these patients had an ultrasound and all showed a mass (7) or cyst (1).(ABSTRACT TRUNCATED AT 250 WORDS)

Pediatric neck masses: guidelines for evaluation

Neck masses are frequent findings in the pediatric population. Unlike the adult, there are few established guidelines for evaluation of these children. The etiology of cervical masses includes many conditions. Knowledge of these conditions and their clinical presentations is essential. To elucidate the clinical characteristics which may help in establishing a correct diagnosis, the charts of 445 patients with biopsies of neck masses performed at the Childrens Hospital of Philadelphia were analyzed. There were 244 (55%) congenital lesions, 118 (27%) inflammatory lesions, 23 (5%) non-inflammatory benign masses, 12 (3%) benign neoplasms, and 48 (11%) malignancies. The preoperative diagnosis was correct in 270 (61%) patients. Guidelines are established for the evaluation of the child presenting with a neck mass.

Morbidity and mortality of short-bowel syndrome acquired in infancy: An update

The advent of total parenteral nutrition (TPN) has made survival beyond infancy possible for large numbers of patients who have sustained massive small intestinal loss due to a variety of intraabdominal catastrophes. However, the quantity and quality of life have been limited by the development of late sequelae due both to the protracted use of TPN and the long-term complications of foreshortening of the gut. To determine to what extent the morbidity and mortality of short-bowel syndrome (SBS) may have improved over the last 10 years, we reviewed our experience since 1973 with patients losing more than 50% of total small intestinal mass in infancy. The etiologies of SBS in the 16 study patients were necrotizing enterocolitis (6), midgut volvulus (5), multiple atresias (3), gastroschisis (1), and congenital SBS (1). Overall survival was 81%; total small intestinal length (SIL) at the time of diagnosis was 44.2 +/- 7.9 cm in survivors and 30.3 +/- 7.8 cm in nonsurvivors, probability values not significant. Although no patient survived without an ileocecal valve whose total SIL was greater than 20 cm, the three deaths in this series were not related directly to the SIL, but to end-stage liver disease resulting from TPN-associated cholestasis. Among the survivors, adaptation to enteral feedings required 13.8 +/- 2.5 mo, during which time weaning from TPN occurred; weight at adaptation was 6.87 +/- 1.32 kg.(ABSTRACT TRUNCATED AT 250 WORDS)

Mesenteric ischemia in hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS) has been widely viewed as a uniformly fatal form of congenital heart disease. Between January 1984 and December 1990, 387 patients with the diagnosis of HLHS were treated at this institution. Mesenteric ischemia was clinically diagnosed in 31 patients (8% incidence) and confirmed by pathology or surgery in 25 of those patients. The mean age at the time of onset was 17.5 +/- 5.4 weeks and only 13% were premature newborns. In 80% of the patients a low perfusion state and significant hypotension were documented within 48 hours prior to the diagnosis of bowel ischemia. Nine patients (29%) required operative intervention (bowel resection 4, diffuse ischemia 3, and simple drainage 2). Overall, at operation or at autopsy, nine patients (29%) had diffuse gastrointestinal ischemia. Of 31 patients with mesenteric ischemia, 26 children (84%) died shortly after onset of the gastrointestinal symptoms regardless of means of management. Five patients (10%) initially improved with aggressive medical and/or surgical management; however, 4 subsequently died secondary to complications of their primary cardiac disease. Therefore, the overall mortality of patients with mesenteric ischemia was 97%. Previous reports have estimated that up to 7% of full-term newborns with symptomatic congenital heart disease may develop necrotizing enterocolitis (NEC). Our unique group of patients with HLHS is comprised mostly of full-term infants who developed onset of mesenteric ischemia at a mean age of 4 months associated with an underlying low perfusion state. This mesenteric ischemia has been erroneously identified as NEC.(ABSTRACT TRUNCATED AT 250 WORDS)

Total intestinal aganglionosis: A new technique for prolonged survival

Total small bowel aganglionosis is uniformly fatal; and prolonged nutritional treatment for the resulting severe short bowel syndrome in the absence of a therapy designed to achieve a functional bowel length has not been warranted. We report an operative technique, long segment small bowel myectomymyotomy, which has produced a functioning length of intestine capable of supporting ever increasing amounts of enteral nutrition. A term female was noted to have neonatal intestinal obstruction, and two laparotomies proved total colonic and near-total small bowel aganglionosis. At 2 months of age reoperation was done and the aganglionosis was proved to extend to 7 cm below the ligament of Treitz. From this transition zone to 10 cm distally, a myectomy was done removing a 1 cm wide length of seromuscular tissue to the level of the submucosa. From the distal end of the myectomy, another 40 cm of bowel received an antimesenteric border myotomy cutting to the submucosal level followed by spreading of the cut surface to a width of 1 cm. This left the patient with 55 to 60 cm of small bowel from the ligament of Treitz to the end of the myotomy at which point an end ostomy was created. The remainder of the small bowel was excised and the colon exteriorized as a mucous fistula. The patient was continued on total parenteral nutrition alone for ten days at which time small volume enteral feeds were introduced. By 5 months of age, 25% of calories were enteral; by 6 months, 33% of calories were enteral; and by 8 months, 45% of intake was enteral.(ABSTRACT TRUNCATED AT 250 WORDS)

The effects of ischemia and reperfusion on intestinal motility

The interdigestive migrating motor complex (MMC) has been demonstrated to be a reliable indicator of intestinal motility and function. The effects of low perfusion on the MMC have never been studied. Fourteen newborn Yorkshire piglets (5 to 18 days old, weighing 2.9 +/- 0.4 kg) underwent celiotomy under general anesthesia with placement of four jejunal electrodes (50 cm apart) as well as a superior mesenteric artery (SMA) Doppler flow probe and a pericardial catheter. Group 1 (n = 5) had operation alone. Group 2 (n = 9) had nonocclusive mesenteric ischemia induced by reversible cardiac tamponade for 5 hours between postoperative days 6 to 12. All subjects had MMC phase III electrical activity, cycling time, and propagation velocity recorded daily. In group 2 MMCs were recorded prior to and during ischemia, and during reperfusion. Group 2 animals had 75% +/- 4% decrease in SMA flow during the tamponade period. During the ischemic period, the MMC cycling time (CT) increased from 67 +/- 10 (mean +/- SEM) to 98 +/- 12 minutes (P < .05) and MMC propagation velocity (PV) decreased to 4.2 +/- 2.2 from a baseline value of 10.5 +/- 1.5 cm/min (P < .05). During reperfusion CT and PV values were not significantly different from baseline. The validity of this model is confirmed by the comparable baseline recordings in groups 1 and 2, and by the return of MMC to baseline values within 4 to 7 hours of reperfusion, as seen in group 2.(ABSTRACT TRUNCATED AT 250 WORDS)

Resection with primary anastomosis for necrotizing enterocolitis: A contrasting view

Resection with primary anastomosis is currently being advocated for treatment of infants with necrotizing enterocolitis. To determine whether our own data would support such an approach, we reviewed retrospectively our experience with this disease since 1974. Since that time, 173 infants have been admitted for treatment of advanced (surgical) disease in its acute phase, of whom 143 underwent resection for cure; the remainder either underwent laparotomy with decompression (3), laparotomy with drainage (3), laparotomy alone (14), died at operation (1), or could not be resuscitated sufficiently to withstand operation (9). Excluded were patients who underwent operative repair of late stricture (6), all of whom survived with no morbidity. Among those resected for cure, 27 infants were carefully selected by the operating surgeon for treatment by means of resection with primary anastomosis, based on the limited and apparently discrete nature of their disease; in three the procedure was combined with a decompressing enterostomy. In the majority of cases (14), the disease was found to involve multiple areas of intestine, but was limited to a particular anatomic region, usually distal ileum and/or ascending colon; in the remainder, it was due to discrete ileal or jejunal perforation or ulcer. Overall survival among those resected for cure was 65% (96/143). It was 48% (13/27) among those treated by means of resection with primary anastomosis but 72% (83/116) among those who underwent resection with enterostomy. However, if the early years of the series (1974 to 1976) are excluded, a time when resection with enterostomy had not yet become established as standard therapy, overall survival was 77% (77/100), 64% (9/14) among those anastomosed primarily.

Cologastric Fistula as a Complication of Percutaneous Endoscopic Gastrostomy

Percutaneous endoscopic gastrostomy (PEG) is frequently used for long-term enteral nutrition or gastrointestinal decompression in both adults and children. The rare complication of a cologastric fistula following PEG has been seen recently in two pediatric patients. One fistula did not close after removal of the gastrostomy tube. A mechanism for the occurrence of this complication in these two children is proposed and technical points are emphasized to prevent this complication.

Primary hyperparathyroidism in infancy

Primary hyperparathyroidism in the neonate is a rare and often fatal disorder. These infants typically display severe hypercalcemia, respiratory distress, muscular hypotonia, and skeletal demineralization. They are usually diagnosed within the first three months of life and have hyperplasia of the four parathyroid glands. Twenty-nine infants with primary hyperparathyroidism are reported in the literature. Mortality is 87.5% in medically managed patients and 24% in surgically managed patients. Surgical management has not been satisfactory, in that recurrent hypercalcemia has been encountered in most patients undergoing subtotal parathyroidectomy, and total parathyroidectomy has resulted in the need for lifelong calcium and vitamin D supplementation. We have recently cared for a term newborn female in whom the diagnosis of primary hyperparathyroidism was made clinically on the second day of life, and later was confirmed biochemically. The baby underwent neck exploration on the 11th day of life and was successfully treated with total parathyroidectomy and parathyroid autotransplantation. Although initially rendered eucalcemic, the infant subsequently developed recurrent hypercalcemia requiring the removal of some of the autograft. Currently, the child is more than 2 years following surgery, growing well, and off all medication. The world literature is reviewed in this report of one of the first and the youngest infants, to our knowledge, to undergo parathyroid autotransplantation. In view of its success in avoiding the complication of repeated neck exploration for recurrent hyperparathyroidism or the creation of permanent hypoparathyroidism, we recommend this surgical approach for the rare neonate with primary hyperparathyroidism.

The usefulness of open-lung biopsy in the pediatric bone marrow transplant population

From October 1976 to October 1986, 126 children had bone marrow transplants at the Childrens Hospital of Philadelphia. The indications were acute lymphocytic leukemia (ALL) (30), nonlymphocytic leukemia (24), aplastic anemia (15), solid tumors (47), and miscellaneous conditions (10). Of these, 21 (17%) underwent 22 open-lung biopsies. Fourteen of these patients showed no causative microorganism. When a cause was found it was viral (usually cytomegalovirus [CMV]) in three, fungal in one, Pneumocystis carinii alone in two, both viral and pneumocystis in one, and a combination of viral, bacterial, and pneumocystis in one. Thirteen patients died due to continued deterioration after the biopsy. In only two patients was there a significant change in antimicrobial therapy as a result of the biopsy. Both had Pneumocystis (one in combination with virus and bacteria). One patient with chronic infiltrates showed a lymphocytic interstitial pneumonia, which responded well to steroids. Open-lung biopsy is currently of limited value in this patient population. Survival is dismal unless the patient has Pneumocystis. We believe that prospective studies should be set up to compare open-lung biopsy with empiric antimicrobial therapy. A major emphasis must be on prevention.