Sami Youssef

Childhood primary pulmonary neoplasms

Nine children (6 boys, 3 girls) were diagnosed with a primary endobronchial or pulmonary parenchymal neoplasm. The average age at diagnosis was 9 years. Presenting complaints included cough (7), fever (5), pulmonary infection (3), respiratory distress (3), weight loss (2), pain (2), and hemoptysis (1). Pulmonary x-rays showed persistent atelectasis, pneumonic infiltrates or mass lesions. A computed tomography scan was performed in 8. Five of six endobronchial tumors were diagnosed with bronchoscopy and biopsy. Treatment consisted of thoracotomy and pulmonary resection in 7 cases and laser resection in 2. The pathologic diagnoses were bronchial carcinoid (3), bronchial mucoepidermoid carcinoma (1), inflammatory pseudotumor (plasma cell granuloma) of the bronchus (2) and of the lung parenchyma (1), fibrosarcoma (1), and rhabdomyosarcoma (1). Postoperative chemotherapy was given only to the patient with pulmonary rhabdomyosarcoma; this child died. One child has developed a local recurrence while 7 children are alive and free of disease at an average of 2.4 years postresection. Pulmonary neoplasms are unusual in the pediatric age group and represent a wide spectrum of pathology. Including the present series, 383 tumors have been described. Seventy-six percent were malignant. Early investigation and surgical intervention are essential in children with persistent pulmonary symptoms or x-ray abnormalities. In most cases, the prognosis is excellent with complete surgical resection; however, malignancies other than bronchial adenoma are associated with significantly mortality.

Surgical indications in antenatally diagnosed ovarian cysts

The antenatal diagnosis of ovarian cysts poses a therapeutic dilemma because the natural history of these lesions is not well known. A retrospective review from 1980 to 1989 showed 29 ovarian cysts in 27 patients diagnosed by prenatal ultrasonography performed between 28 and 36 weeks of gestation. Nineteen cysts were initially observed. Eleven cysts resolved (diameter less than 2 cm), three are decreasing, three were lost to follow-up, and two underwent resection. Eight patients underwent surgical exploration immediately following birth. The diagnosis of benign ovarian cyst was confirmed histologically in all cases. A review of the literature showed an additional 230 cases of antenatally diagnosed ovarian cysts. Simple cysts of the ovary tend to resolve spontaneously and, therefore, may be treated conservatively. Serial ultrasonography allows accurate diagnosis and long-term assessment of ovarian cysts in the neonate and may prevent unnecessary oophorectomy. Patients with cysts larger than 4 cm may be candidates for percutaneous aspiration, or should undergo removal of the cyst because of a significant risk of torsion. Complex cystic masses, symptomatic ovarian cysts, and cysts that do not resolve should be removed.

Cholelithiasis in newborns and infants

Cholelithiasis in infants is rare, and has usually been associated with hemolysis, ileal disease, congenital anomalies of the biliary tree, hyperalimentation, and prolonged fasting. With the increased use of abdominal ultrasonography (US), more cases of cholelithiasis are being discovered. We report our experience with 13 infants diagnosed on abdominal US to have gallstones. There were 9 boys and 4 girls with an average age at diagnosis of 2.6 months (range, 0 to 9 months). Predisposing factors could be identified in only 6 of the 13 patients. Two patients with obstructive jaundice underwent cholecystectomy and common bile duct exploration. One patient with choledocolithiasis and common bile duct dilatation was observed. His stone passed spontaneously, with resolution of symptoms. Ten patients without cholestasis remained asymptomatic, with disappearance of lithiasis in five of them. Neonatal cholelithiasis is more common than previously suspected; it seems to affect males more often than females and is usually not associated with known predisposing factors. It appears to be a temporary, self-limiting phenomenon, and an aggressive approach is not warranted in the asymptomatic infant. Surgical or radiological intervention should be reserved for the symptomatic patients or those with underlying lithogenic disorders.

Neonatal Gastrointestinal Perforations

Neonatal gastrointestinal perforation has been associated with mortality rates of 40% to 70%. Over the past 20 years, 81 infants (46 boys and 35 girls) were treated for a gastrointestinal perforation at this institution. Perforation occurred from birth to 50 days (average, 8.2 days). Etiologies included necrotizing enterocolitis (NEC) (68%), meconium ileus (10%), and idiopathic gastric perforation (7%). Seventy-six infants underwent surgical exploration and five infants, considered too small or too sick to withstand a laparotomy, were treated with peritoneal lavage only. There were 29 deaths, an overall mortality of 36%. Ninety percent of the death occurred in patients with NEC, while all patients with gastric perforations survived. There has not been a significant improvement in survival in recent years, partly because of an increase in the proportion of NEC-related perforations. However, there is a narrowing of the mortality gap between low birth weight and normal weight infants. As the risk inherent to laparotomy in neonates is decreasing, other factors, such as the underlying etiology or the site of perforation, play a more important prognostic role.

Thymectomy for myasthenia gravis in children

Myasthenia gravis is rare in childhood. In the last 12 years, we have treated eight children under 16 years of age for this disease. There were six girls and two boys, a ratio of 3:1. Age of presentation of symptoms ranged from 10 to 16 years (mean 11 3/4). There was no significant family history and there were no significant associated diseases. Antibodies were measured in six patients and were all negative. All patients were given cholinergic drugs and five had corticosteroids as well. Three underwent tracheostomies because of severe respiratory insufficiency. There was clinical deterioration or no improvement in spite of increasing doses of medication; hence, thymectomy was performed in all after an average duration of symptoms of 12 months (range 4 to 17). All thymectomies were done through median sternotomy. There was no postoperative morbidity or mortality. No thymoma was found. Four had lymphoid hyperplasia, in three, the thymus looked normal and in one it looked very atrophic. Dramatic amelioration with eventual complete recovery was seen in six, with an average follow-up of 7.3 years (3 to 11) after thymectomy. Two patients are only one year after thymectomy and are improving progressively. We recommend early thymectomy in myasthenia gravis of childhood on the basis of very encouraging results.

What is the incidence of Barrett's and gastric metaplasia in esophageal atresia/tracheoesophageal fistula (EA/TEF) patients?

OBJECTIVE Aim of the study was to document the incidence of gastric and intestinal metaplasia (Barretts esophagus) in a closely followed group with EA/TEF, determine the efficacy of pH probe studies in diagnosing reflux and, moreover, to determine the lag period between the development of gastroesophageal reflux disease (GERD) and metaplasia. MATERIALS AND METHODS Patients who underwent EA/TEF correction were followed up over an 18-year period (1990-2009) with regular pH probe and endoscopy investigations. Biopsies were performed at 3 year intervals to evaluate for metaplastic changes in the esophageal epithelium. The interval between esophageal atresia correction and the development of erosive esophagitis, gastric metaplasia and Barretts esophagus was determined. The development of complicated GERD was statistically correlated to age at detection. RESULTS 51 patients (28 male) underwent EA/TEF correction during the study period. The ages of the children followed up ranged from 7 months to 19 years. There were 5 Type A, 44 Type C and 2 Type D fistulas. 37 patients (5 Type A, 31 Type C, 1 Type D) had documented reflux and 14 did not. 17 (46%) of the patients with reflux underwent an anti-reflux operation. 4 of the 17 underwent 2 or more anti-reflux procedures. 21 patients had pH probe studies positive for reflux, 3 had negative pH probe studies after an anti-reflux operation. 18 of the 37 patients with reflux (about 50%) needed esophageal dilatation for either anastamotic or non-anastamotic strictures. Only 4/14 (28.5%) patients without reflux underwent dilatations and only in their first year of life (p=NS). 15 patients had mucosal abnormalities in endoscopic biopsies. 8 patients had gastric metaplasia and 1 patient had Barretts esophagus. The mean age in the group with metaplasia (gastric and intestinal) was 13 years, which was significantly different from the mean age of the group (5.2 years) without mucosal changes (p<0.001). 2 patients with normal pH probe studies had metaplasia on biopsies. CONCLUSION Metaplasia arises in about 15% of patients with EA/TEF. The lag time to developing metaplasia from the time of initial surgical correction is about 10 years. Endoscopy and biopsies are the best way of detecting mucosal changes.

Gastrointestinal manifestations of Sipple syndrome in children.

The diagnosis and management of patients with multiple endocrine neoplasia (MEN) type IIA and type IIB are of special challenge to pediatric surgeons. Patients characteristically present early in life with significant intestinal symptoms at a time when the characteristic phenotypic features of MEN IIB are frequently absent. We are reporting 12 patients with MEN type II (9 with type IIA and 3 type IIB or Sipples syndrome), all of whom presented with gastrointestinal manifestations. All 12 patients had signs and symptoms of bowel obstruction during the neonatal period. An unusual association of Hirschsprungs disease and MEN IIA was noted in our nine patients found among a kindred of 92 individuals. All three patients with Sipples syndrome (MEN IIB) had severe gastrointestinal symptoms since birth, including recurrent pseudoobstruction. The possibility of MEN type II should be considered in all cases of bowel obstruction in the newborn period. Screening for medullary carcinoma of the thyroid must be carried out from infancy. A detailed family history is very important to avoid unnecessary surgery for bowel obstruction in Sipples syndrome.

Minimally conjoined omphalopagi : a consistent spectrum of anomalies

Omphalapagus twins constitute less than one third of all siamese twins. Most omphalopagi are attached by a skin bridge that often contains hepatic tissue. Only four cases of omphalopagi attached by an intestinal bridge have been reported. The authors present two additional cases of conjoined twins minimally attached by a small bowel and bladder bridge. In both instances, the spectrum of anomalies included a ruptured omphalocele and imperforate anus with cloacal anomalies. The attachment consisted of an open urachal remnant joining the two bladders, and the short small bowel of twin A attached to the terminal ileum of twin B. Separation was uneventful. For one set of twins, the case part of the proximal colon of twin B was used to lengthen the bowel of twin A. Three of the twins survived and underwent additional procedures for repair of the cloacal anomalies. All four previously reported cases of minimally conjoined omphalopagi presented with a remarkably similar spectrum of anomalies. All had ruptured omphaloceles, imperforate anus with cloacal anomalies, and urachal anomalies. Intestinal connection was consistently at a point corresponding to the Meckel diverticulum site of twin B, with blood supply to the small bowel of twin A, probably via the vitelline artery. The consistent spectrum of anomalies encountered with minimally conjoined omphalopagi allows planning of separation. Caution is required to avoid overlooking the patent urachus, and intestinal lengthening procedures based on the vitelline artery become an important consideration.

The anterior perineal approach for pull-through operations in high imperforate anus

Recent widespread interest in a new approach to imperforate anus is ample testimony to the difficulty of the classical operations and to the unsatisfactory results. In the early seventies, Mollard began to use an anterior perineal approach to high imperforate anus as an alternative to the sacrococcygeal pull-through. It entails a semicircular retroscrotal incision allowing direct access and visualization of the urethra and puborectalis sling. Since 1976 we have switched to the anterior perineal approach. We now have experience with 30 patients, 27 males and 3 females. Two of the females had complicated cloacal anomalies, and three teenage patients were reoperated for incontinence following previous unsuccessful pull-throughs. Of the 27 primary operations, there were 17 with an uneventful early postoperative course. There were two major and eight moderate or minor complications. There was no mortality. A 3-year follow-up is available in 14 patients. The results are good in 8, fair in 4 and poor in 2. The advantages of this technique are threefold: it allows clear visualization and precise identification of the puborectalis sling; the dissection proceeds parallel to and with constant vision of the urethra, avoiding injury to it; and it obviates the need to change the position of the patient during the procedure. This operation is easier and safer than the sacral approach, it avoids cutting through the puborectalis sling as recently described, and, finally, it respects the criteria established by Stephens.

Intrahepatic biliary-enteric bypass for complete extrahepatic biliary obstruction in children

Intrahepatic biliary-enteric anastomosis is rarely practiced in pediatric surgery. The authors report on two children who have been successfully treated using this method. First described by Longmire and Sandford in 1948, intrahepatic biliary-enteric anastomosis is possible in children. This procedure is only indicated as a last recourse for circumstances in which the extrahepatic biliary tree is completely obstructed.