Mortality of chronic hemodialysis and renal transplantation in pediatric end-stage renal disease.

The long-term mortality of chronic hemodialysis and renal transplantation was analyzed in all children treated for end-stage renal disease at Children's Hospital Medical Center over the pase 8 1/2 years. A total of 216 transplantation or dialysis courses in 120 patients were studied. No patients were excluded from treatment or analysis. Overall actuarial survival was 92% at six months, 90% at 12 months, and 89% at five years. When actuarial survival for each form of treatment was examined, patient survival was 100% at six months and 95% at five years for chronic hemodialysis; 92% at six months and five years for living related transplantation; and 88% at six months and 85% at five years for cadaveric transplantation. We conclude that most children with end-stage renal disease can be kept alive with current treatment programs, and that the mortality of chronic hemodialysis in children is comparable to that of renal transplantation.

Adenocarcinoma at the site of ureterosigmoidostomies for exstrophy of the bladder.

Adenocarcinoma of the colon is a rare complications of ureterosigmoidostomy done for exstrophy of the bladder. Only 13 cases have been previously reported, occurring from 10 to 46 yr after the procedure. In a series of 150 patients who have had ureterosigmoidostomies carried out at the Children's Hospital Medical Center, Boston, over the past 40 yr, 4 patients have been treated at our institution for this complication. The tumor was noted 20 to 32 yr after the initial procedure. Two patients are alive and well 1 and 4 yr after resection. The increasing incidence of this complication with advancing age will require careful observation and will undoubtedly influence the indications for the procedure. An additional question of great importance is how to evaluate and follow the thousands of patients who have had this procedure done at one time in their lives. It is suggested that an annual intravenous pyelogram is indicated in those patients with functioning ureterostomies and that a colonoscopy to the level of the anastomoses be carried out at 6-mo intervals in all patients. In those patients in whom the ureterosigmoidostomies have been taken down, a sleeve resection of that area of the colon should be strongly considered.

Pancreatitis and intravenous fat: an association in patients with inflammatory bowel disease.

Since recently adding Intralipid (IL) to our standard total parenteral nutrition (TPN) regimen for children with inflammatory bowel (IBD) disease, two children undergoing major intestinal resections have developed acute pancreatitis in the early postoperative period. Herein, we present the clinical summaries of these two patients, discuss the multifactoral etiologies of acute pancreatitis, and review the role of emulsified fat solutions in TPN in children with IBD.

Perforation of Meckel's diverticulum in infancy.

Nine infants underwent operations for perforation of a Meckel's diverticulum over a 21-yr period. All of the infants were acutely ill when initially seen by the surgical service. Lethargy, irritability, anorexia, fever, abdominal tenderness, and passage of blood in the stools were common clinical manifestations. The preoperative diagnosis of perforated Meckel's diverticulum was not made in any of the cases. Skilled preoperative and postoperative management is important for the survival of these infants. There was no mortality and minimal morbidity. In retrospect, the peptic etiology of perforation in this age group is a key to early recognition. The past history of most of the patients includes previous episodes of blood in the stools and episodic abdominal complaints.

Juxtapancreatic intestinal duplications with pancreatic ductal communication: a cause of pancreatitis and recurrent abdominal pain in childhood.

Pancreatic duplications with ductal communications should be included in the differential diagnosis of any child presenting with recurrent abdominal pain of unknown etiology and should be considered as a possible cause of pancreatitis in childhood. Such duplications most likely arise from nonregressing diverticula of the pancreatic bud during embryologic development. Their clinical presentation is unique from other duplications because of their anatomic association with the pancreatic duct. Pain and weight loss are the major presenting complaints, although many patients have nausea and vomiting. Serum chemistries, in particular the serum amylase, are usually normal and are of little help in the differential diagnosis. Radiographic evaluation has not been particularly helpful in the past. ERCP, ultrasonic examination, and CT scan show great promise, however. Operative intervention should be tailored for the individual patient. The operation performed will depend upon operative findings. Intraoperative pancreatograms or cystograms are very helpful in differentiating these cysts from others at the time of operation. Pathologically, most of the duplications have a thickened muscular coat that usually has some evidence of inflammation. They are usually lined with gastric mucosa. Except in the most severe cases, the pancreas is histologically normal, suggesting that most of the pain experienced by these patients is secondary to inflammation within the duplication. The inflammatory response may completely destroy the mucosal lining and cause fibrosis within the muscular coat of the duplication. In those instances, these lesions cannot be differentiated from pancreatic pseudocyst. This may account for some of the "idiopathic" pseudocysts reported in the literature.

Congenital lymphedema of the penis: a method of reconstruction.

Congenital lymphedema of the genitalia has profound physical and psychological consequences for the growing child. Extensive resection of this tissue and reconstruction by skin grafting offers a less than satisfactory cosmetic result. Over the past year we have employed a method of total excision of the lymphedematous tissue of the penile shaft with cosmetic reconstruction without skin grafting. A circumferential incision was made 5-10 mm from the coronal sulcus and deepened to the level of Buck's fascia. The skin and subcutaneous tissue were then completely dissected away from the penis. The skin was everted and all of the abnormal lymphedematous tissue excised up to the dermal skin margin. The skin was then tailored to the size of the penile shaft and reapproximated. This method has been employed in two patients with the advantages of (1) shorter hospitalization, (2) lack of morbidity associated with the skin donor site, and (3) satisfactory cosmetic results.

Circular esophagomyotomy of upper pouch in primary repair of long-segment esophageal atresia.

A method of performing circular esophagomyotomy of the upper pouch is described as an effective technique for use in repair of esophageal atresia where the ends cannot be brought together.

Fate of the distal esophageal remnant following esophageal replacement.

When a colon segment is used for esophageal replacement in patients with esophageal atresia, the distal esophageal remnant is often left in place. We recently treated two patients who developed esophagitis in their esophageal remnants and did not respond to cimetidine and antacids. They were treated by esophagectomy at 22 and 37 years of age with relief of symptoms. One had an ulcer and stricture near the gastroesophageal junction and the second had gastric mucosa (Barrett's esophagus) replacing most of the squamous epithelium. Six additional patients were then reviewed who had resection of their distal esophageal segments between 1978 and 1987. Esophagitis was present in all. Also identified were two specimens with Barrett's esophagus and four with mural bronchial glands as well as surface respiratory and metaplastic squamous epithelium in two, and cartilagenous remnants in two. The findings of chronic inflammation in the esophageal remnant and three cases of Barrett's esophagus raise concern about the possible long-term complication of malignancy. Therefore, we recommend that esophagectomy be performed at the time of esophageal replacement if feasible, or later if symptoms occur or barium studies show esophagitis or ulceration.