Accessory scrotum and anorectal malformation associated with "pseudo" prune belly in a neonate.

We report a patient with high anorectal anomaly and accessory scrotum. To the best of our knowledge only four other similar cases have been described. The patient also had renal dysplasia, partial prune-belly syndrome, and dismal outcome because of urinary septicemia. To our knowledge, accessory scrotum, high anorectal anomaly, and prune-belly syndrome in the same patient has never been reported.

Neonatal intussusception as a presenting sign of Hirschsprung's disease.

A case of ileocecal intussusception as a presenting sign of Hirschsprung's disease in the neonatal period is presented with a review of the literature.

Preserved viability of the isolated bowel segment, created by omentoenteropexy: a histological observation.

Creation of the isolated bowel segment (IBS) by omentoenteropexy in rats and dogs consists of a two-step procedure: initial omentoenteropexy to the antimesenteric border of a jejunal segment, and division of its mesentery 6 weeks later. Viability of this IBS is maintained by the angiogenic process, which occurs at the level of the myoenteropexy. Histological studies were performed by light microscopy on the bowel wall structures of the IBS before and after the mesenteric division, as well as after a lengthening procedure performed on the IBS in one dog. The authors conclude that (1) in rats and dogs the intestinal wall structures of all IBS variants created by omentoenteropexy appear close to normal, and (2) neovascularization can be clearly detected at the level of the omentoenteropexy.

Barrett's epithelium with complete stricture of the esophagus: hypothesis of its etiology.

A 4-year-old very debilitated boy, who suffered from recurrent vomiting (regurgitation) since birth, is described. At the age of 18 months, partial obstruction of the esophagus was apparent, and only semisolid food could be swallowed. The signs of obstruction progressed, and the child was admitted urgently, at the age of 4 years, to the authors' department because of severe dehydration. After resuscitation, a barium swallow and esophagoscopy showed a complete obstruction of the esophagus between its middle and lower third. Through a left thoracotomy incision, 4 cm of the diseased esophagus were resected and continually established by end-to-end esophagoesophageal anastomosis. Pathological examination showed complete obstruction of the esophagus with Barrett's epithelium above and below the stricture. Severe periesophagitis was also present because of sealed perforation of the esophagus. One week after the operation the child was on a normal diet. On follow-up, 6 months later, he has no signs of obstruction and is gaining weight. It is postulated that the presence of Barrett's epithelium in the esophagus in congenital, but the complications, such as stricture formation, are usually caused by chronic irritation, such as gastroesophageal reflux. Barrett's epithelium alone infrequently will cause

Wilms' tumor: the Northern Israel Oncology Institute experience.

Between 1971 and 1992, 42 children (median age = 2 years) with histologically proven Wilms' tumor were evaluated at the Northern Israel Oncology Institute. Most patients were staged according to National Wilms' Tumor Study (NWTS) criteria by which 18 were clinical stage I-II and 24 were III-IV. Combined therapy (surgery, chemotherapy, and radiotherapy) was given with a change to lower dose radiotherapy (< 2,000 cG) after 1981. The pre-1981 group (13 patients) suffered six relapses, most of which were pulmonary, whereas the 1981-1992 group (29 patients) had three relapses. The actuarial 5-year survival rate using Kaplan-Meier tables was 79% for the entire group but 100% for the 1981-1992 group and 38% for the 1971-1980 group. One case of immediate hepatic toxicity and one sudden death, unknown cause, of a patient in remission occurred in the group. Late deleterious effects were seen in five other patients; four had scoliosis and one developed osteoid osteoma of a rib. Despite these encouraging results, the necessity of ongoing monitoring for further long-term toxicities is apparent.

Outcome of partial splenectomy for type I Gaucher disease.

Partial splenectomy was introduced to achieve the benefits of splenectomy and to avoid the risk of overwhelming infection in patients with symptomatic Gaucher disease. We observed regrowth of the splenic remnant, reemergence of preoperative symptoms, and new bone involvement among most of our patients who had undergone partial splenectomy. Enzyme replacement therapy has markedly limited indications for splenectomy, partial or total, for Gaucher disease.

Jejunal mucosal function of the isolated bowel segment created by omentoenteropexy in dogs: a study by in situ luminal perfusion.

An isolated bowel segment (IBS) was created in dogs by omentoenteropexy, using staged procedures. (1) Omentoenteropexy was performed at the antimesenteric border of a 15-cm jejunal segment, which was exteriorized at both ends (IBSB). (2) After 6 weeks, once dual vascularization to the IBS had been established, its mesentery was divided (IBSA) or longitudinally split, thus achieving its elongation (IBSE). A control dog underwent a Thiry-Vella (T-V) loop procedure of an identical jejunal segment. Viability of the IBSB and IBSA was previously proven by the authors, through angiographic studies. In the present study, the absorption capability of IBS variants was assessed using in situ luminal perfusion, with a bicarbonate buffer containing glucose and labeled glycine. Jejunal transport rates of these solutes were calculated from the differences in their concentrations in the perfusion solution and in the effluent. Comparisons were made among the IBS variants and between them and the T-V loop. No significant difference in the absorption capability of glucose and glycine was noted between the various IBS variants. There was a marked reduction of glucose absorption and a moderate reduction of glycine absorption in all IBS variants. when compared with the fresh T-V loop, most probably because of disuse atrophy of the mucosa. In conclusion, absorption of glucose and glycine is preserved in the IBS, created by omentoenteropexy, both after its mesenteric division and following the IBS elongating procedure.

Traumatic rupture of the choledochus treated temporarily by Roux-en-Y entero-hepato-duodenal ligamentostomy.

The authors report on a 3-year-old boy who had traumatic rupture of the choledochus. Temporary internal drainage was achieved by Roux-en-Y end-to-side entero-hepato-duodenal ligamentostomy. Two months later, a standard Roux-en-Y end-to-side choledocho-jejunostomy was performed. Five years after the injury, the child is well and has no jaundice.

Creation of the isolated bowel segment in animals by omentoenteropexy.

An isolated bowel segment (IBS) was created in rats and dogs by a two-stage procedure. Initial coaptation of the bowel segment to the omentum ("omentoenteropexy") is followed by division of its mesentery several weeks later. The viability of the segment is maintained by angiogenesis at the coaptation site. Neoformed blood vessels were shown by angiographic studies, and in a dog the IBS was used for esophageal interposition.

A simple technique for anchoring tubes to the skin.

The author describes a simple technique for anchoring various tubes used in the treatment of trauma patients (gastrostomy tubes, jejunostomy tubes, intercostal drains, etc.) to the skin with sutures. In this technique the tube is stretched while the suture is tied around it, thus producing a tight fit.

Mesenteric fibromatosis.

The authors present the case of a 13-year-old boy in whom, after irradiation for Hodgkin's disease and exploratory laparotomy, mesenteric fibromatosis developed. The "tumor" was resected, and 1 year postoperatively the patient is well.

Type II diabetes mellitus, congestive heart failure, and zinc metabolism.

Zinc status was assessed in patients with type II diabetes mellitus and congestive heart failure (CHF). Three groups of patients were enrolled into the study: Group 1: 15 patients with type II diabetes mellitus and CHF; Group 2: 20 patients with isolated type II diabetes mellitus; and Group 3: nine patients with isolated CHF. Twenty-four-hour urine was measured for creatinine, protein, and zinc, and blood was drawn for creatinine, proteins, liver enzymes, hemoglobin A1c, and zinc. Insulin treatment and hemoglobin A1c were comparable in the diabetic patients of groups 1 and 2, but group 1 was also treated with captopril and diuretics like the CHF patients of group 3. Plasma zinc levels were statistically similar in all three groups, but urinary zinc excretion (mumol/24 h) and urinary zinc: creatinine (mumol/mmol) ratio were significantly higher in the type II diabetics and CHF group (27.2 +/- 1.5; 1.69 +/- 0.6, respectively) compared to the diabetic patients alone (19.4 +/- 0.76; 0.97 +/- 0.3, respectively) and the CHF patients (9.7 +/- 0.3; 0.62 +/- 0.3, respectively). and the CHF patients (9.7 +/- 0.3; 0.62 +/- 0.3, respectively). Patients with type II diabetes mellitus and CHF were treated with higher doses of captopril than the CHF patients (56.25 +/- 24 mg vs 18.8 +/- 11 mg P < 0.05). Thus, patients with type II diabetes mellitus and CHF excrete larger amounts of zinc, which may eventually lead to zinc deficiency.

Partial splenectomy in Gaucher's disease: follow-up report.

This is a follow-up report of three children who had partial splenectomy and are suffering from Gaucher's disease. A few years following partial splenectomy in every patient, the remaining spleen enlarged and hypersplenism reappeared; partial splenectomy did not prevent bone destruction if it already existed before partial splenectomy. Treatment with Ceredase parallel with partial splenectomy may have theoretical and practical advantages.

Hyperoxaluria is not a cause of nephrocalcinosis in phosphate-treated patients with hereditary hypophosphatemic rickets.

The treatment of X-linked hypophosphatemia (XLH) consists of phosphate and vitamin D3 derivatives. Transient hypercalciuria and hypercalcemia are well-known signs of vitamin D intoxication. Despite urinary calcium excretion control, the danger of nephrocalcinosis in treated patients has been emphasized. It has recently been suggested that hyperoxaluria might be a causative factor of nephrocalcinosis other than calcium in phosphate-treated XLH patients. We measured urinary oxalate and phosphate excretion in 12 patients with the syndrome of hereditary hypophosphatemic rickets with hypercalciuria (HHRH) receiving only oral phosphates and in 5 XLH patients receiving both oral phosphates and vitamin D. No correlation was found between the dosage of phosphate supplements or urinary phosphate excretion and urinary oxalate excretion, in either group of patients. Nephrocalcinosis, presenting as hyperechogenicity of the medullary pyramids, was found in 2 of the 5 XLH patients and only in 2 HHRH patients who had been treated with excessive doses of vitamin D2 and calcium, prior to the true diagnosis being established. We conclude: (1) hyperoxaluria is not a cause of nephrocalcinosis in phosphate-treated patients with hereditary hypophosphatemic rickets; (2) prolonged phosphate treatment alone does not induce nephrocalcinosis in HHRH patients, and (3) we believe that in XLH patients, nephrocalcinosis is essentially due to vitamin D overdosage at some stage, or noncompliance in phosphate intake, leading to repeated undetected hypercalciuric periods.

Pneumatic perforation of the esophagus in children.

The excessive pressure in a carbonated beverage-containing plastic bottle, released by the teeth of an 11-year old boy, caused perforation of his lower pharynx. The possible mechanism is discussed.

Late presentation of Bochdalek hernia: our experience and review of the literature.

During the last 18 years, 81 patients were diagnosed in the Department of Pediatric Surgery, Rambam Medical Center, as having congenital diaphragmatic hernia, 11 of whom (13.5%) presented after the first 24 h of life. In this retrospective study we describe our experience with late presentation (beyond 8 weeks after birth) of Bochdalek hernia in 5 of the 11 patients, and we review the literature.

Insertion of a soft silastic nasogastric tube at operation for esophageal atresia: a new technical method.

A new and simple technical method for insertion of a soft silastic nasogastric tube into the stomach in neonates born with esophageal atresia is described. The technique is simple, easy to perform, and enables feeding the baby through this tube a day after the operation. Our method is based on the principle used in insertion of Broviac-Hickman catheters, namely the use of a "peel-away sheath" through which the silastic feeding tube is threaded. We have used this technique in five patients with esophageal atresia without any complication.

The use of endotracheal tubes to assist in manipulation of esophageal strictures, in elongation of the esophagus in esophageal atresia, and in gastrojejunal feeding tube placement.

Because of the special qualities of a PVC endotracheal tube (smooth, soft, pliable but still with a patent lumen), it serves very well for the intubation of the esophagus or the duodenum through an established gastrostomy. In this article the use of endotracheal tubes in different manipulations of the esophagus and gastroduodenum is described.

[Recurrent esophago-respiratory tract fistula after repair of esophageal atresia with tracheo-esophageal fistula].

Esophageal atresia is a congenital malformation present in 1 in 4500 live births. 85% of such newborns also have a fistula connecting the distal esophagus with the trachea. Operative management includes closing the fistula by disconnecting the distal esophagus from the trachea and performing end-to-end esophago-esophageal anastomosis. A late complication is a recurrence of the fistula between esophagus and respiratory tract. The recurrent fistula is usually between the esophagus and the trachea, but it may involve the bronchi or lung tissue. This complication causes significant respiratory morbidity, mainly recurrent pneumonia, and sometimes death. The diagnosis of recurrent fistula is usually by barium swallow and/or esphagobronchoscopy. Repair is a surgical challenge. We report 5 infants with recurrent esophago-respiratory tract fistulas out of a total of 68 cases of esophageal atresia with tracheo-bronchial fistula.