Partial splenectomy in children with Gaucher's disease.

Because of hypersplenism and mechanical problems, partial splenectomy was performed in four children with Gaucher's disease. Subsequently, one of the patients underwent a total splenectomy due to bleeding from the remnant of the spleen. At the follow-up of the other three patients, an isotope scan showed that the remaining spleen was functioning well.

Craniofacial microsomia in a parasite of a heteropagus conjoined twin: a clinical and histopathologic evaluation.

This report evaluates a rare case of craniofacial microsomia in a partially developed, malformed heteropagus conjoined twin. In this instance, several major components of the craniofacial complex were involved: bones, cartilage, teeth, salivary glands, auditory apparatus, cerebrum, cranial nerves, and ocular neuroepithelium. In addition, cervical vertebrae and appendicular long bones were markedly affected. To date, the underlying disorder (whether genetic, metabolic, or environmental) responsible for the development of this congenital malformation is not fully understood. Our clinical and pathologic examinations tend to suggest that an apparent lack of an adequate arterial blood supply to the growing embryo could have contributed to the elaboration of this complex syndrome. The fact that the parasite's blood supply relied mainly upon a single, medium-sized artery (1.5 mm in diameter) could have brought about local ischemic milieus during the critical phases of embryogenesis; hence, impeding the preprogrammed migration of neural crest cells to their end targets in the upper branchial arches. In such a case, the normal sequence of cell differentiation is damaged, which in turn imposes a severe impact on the morphogenesis of the various tissues composing the craniofacial complex.

Human tails.

Two children and one infant with a "human tail" are presented. The patho-embryology of this medical curiosity is briefly discussed. Treatment is usually unnecessary but resection of part of the coccyx together with the "tail" may become indicated by coccygodynia or for aesthetic reasons.

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.

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.

Acute appendicitis located in a scrotal hernia of a premature infant.

A premature infant is presented with acute appendicitis in a scrotal hernia causing localization of the inflammatory signs to the scrotum, and relatively early operation.

Protection of the pediatric surgeon from heat stress caused by the overhead radiant heater during surgery.

To protect the pediatric surgeon from heat stress while operating on premature infants under an overhead radiant heater, we propose covering the surgeon's head with an ordinary straw hat wrapped with heavy-duty kitchen aluminum foil. The temperature under such a straw hat is approximately 37 degrees C, versus 52 degrees C under a surgical cotton cap.

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.

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.

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.

Wide gap esophageal atresia: a new method to elongate the upper pouch.

A new surgical method is presented for overbridging wide gap esophageal atresia. The method is based on the fact that the atretic upper pouch has an excellent longitudinal blood supply, and is two to three times wider than the lower pouch. Muco-muscular flap is created, which is anastomosed to the lower esophagus.

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.

Does the spleen regenerate after partial splenectomy in the dog?

Four dogs underwent two thirds partial splenectomy and the remaining spleens were followed up to 6 months by radioisotope studies and finally measured at the time of sacrifice. In this study we showed that the remaining spleens after partial splenectomy did not show any signs of regeneration.

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.

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

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.

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.

Endesophageal resection of short benign strictures of the esophagus.

A relatively simple operation for the treatment of short esophageal strictures that do not respond to dilatations has been described. This operation was successfully carried out at all levels of the esophagus. The strictures treated were caused by lye burns, peptic esophagitis and previous anastomoses.

Use of cimetidine in esophageal atresia with lower tracheoesophageal fistula.

Pneumonia, in infants suffering from esophageal atresia with lower esophageal fistula, is usually caused by gastric reflux through the fistula, In order to abolish the acidity of the gastric content, Cimetidine i.v. infusion was used during the first 12--48 hr after diagnosis, while treating the pneumonia. The amount of Cimetidine required to induce achlorhydria was established by serial aspiration of gastric content, in infants and children with gastrostomies performed for various causes.

Gastrografin treatment of intestinal obstruction due to Ascaris lumbricoides.

Gastrografin was used to relieve subacute intestinal obstruction due to Ascaris lumbricoides in children. This hyperosmolar and wetting agent proved to be highly efficient to achieve this goal. Statistical evaluation of the hospital stay and first bowel action showed a significant difference between the treated "gastrografin group' and the untreated by gastrografin "control group,' in favor of the "gastrografin group.' Our data strongly suggest the use of Gastrografin in subacute intestinal obstruction due to Ascaris lumbricoides. This treatment should be instituted provided the patient has been adequately hydrated prior to administration.