Thoraco-omphalopagus conjoined twins: successful surgical separation.

Male thoraco-omphalopagus conjoined twins were evaluated systematically with respect to visceral joining. Cardiac studies included electrocardiography, echocardiography, and a dynamic radioactive heart scan. No union was found. Liver union was demonstrated by scanning and angiographic studies. No communication between the gastrointestinal tracts was found, and no other major anomalies were discovered. When the twins were approximately 3 months of age, surgical separation was performed. A common pericardium, two separate hearts, and a union of the liver were found. Prosthetic sheeting was used to close the pericardium and the thoracic-abdominal defects in both. In one, primary skin closure was possible. In the other silicone rubber sheeting was used to close a large skin defect in the chest. Both have done well over a follow-up period of 14 months. One is larger than the other, but the growth rate of both has improved in the last 6 months.

The effectiveness of Nissen fundoplication in neurologically impaired children with gastroesophageal reflux.

Fifty-seven of 101 Nissen fundoplications during the 4-year period, July 1979 to July 1983, were performed on neurologically impaired children. Mean age at the time of surgery was 5.9 years (range 1 month to 22 years). Indications for operation included: persistent vomiting, 57 patients (100%); failure to thrive, 49 patients (86%); repeated episodes of pneumonia, 49 patients (86%); esophagitis, 18 patients (32%); hiatal hernia, 14 patients (25%); episodes of apnea, 10 patients (18%); and esophageal stricture, six patients (10%). Forty-six of the 57 patients had previously failed a standard trial of nonsurgical management. Gastroesophageal reflux was documented by barium esophagograms in 51/56 patients (91%), chalasia scans in 28/32 patients (88%), esophagitis or stricture at endoscopy in 21/23 patients (91%), and acid reflux on pH monitoring in 13/16 patients (80%). Operative management included gastrostomy in 55 of the 57 patients and this was permanent in 50. Gastrostomies had previously been performed in nine patients but had failed to provide a reliable method of enteral feeding because of chronic reflux and aspiration. The surgical complication rate was 12%. Intraoperative esophageal perforation occurred in two patients, splenic tear in one, hepatic vein laceration in one, and a tight wrap in one. After surgery, bowel obstruction from adhesions developed in one patient and a midgut volvulus in another. Five of the children have died, none from causes related to the surgical procedure. Clinical and radiologic follow-up evaluations of all survivors have been done, with a mean follow-up of 3 years. In four patients the repair was felt to be inadequate. One patient had an esophageal stricture and three had recurring episodes of pneumonia. Three children showed radiologic evidence of persistent reflux, but only two were symptomatic. Two patients required a second antireflux procedure for reflux and are now free of symptoms. Nissen fundoplication appears to be a safe and beneficial procedure in neurological impaired children. Long-term follow-up evaluation of these patients showed satisfactory growth as well as a significant decrease in pulmonary disease associated with aspiration.

Selective management of injured spleen.

During the past 6 years, 68 consecutive children suffering from splenic trauma have been treated according to a selective management plan. Twenty-two patients (32%) underwent splenectomy, parenchymal repair was performed on 16 occasions (24%), and nonoperative treatment was employed in 30 children (44%). Overall results have been good in all three groups. Two children (3%) died as a result of their injuries, and 10 complications were recorded. Both deaths and 7 of the 10 complications occurred in the patients undergoing splenectomy. By utilization of a basic management plan that favors nonoperative treatment over splenic repair and repair over splenectomy, splenic salvage has been successfully accomplished in 86% of the 49 patients treated since 1977.

Dumbbell neuroblastomas in children.

A "dumbbell" neuroblastoma is a malignant neoplasm usually primary in the mediastinum or retroperitoneum with posterior extension through an intervertebral foramen to additional tumor within the spinal canal. Neurologic deficits are found in almost all cases. Nineteen patients with such tumors were reviewed with respect to diagnosis, management, and results. Treatment consisted of prompt laminectomy with total or subtotal excision of the extradural tumor. The primary tumor in the mediastinum or retroperitoneal area was removed at a second stage. All patients received postoperative radiation therapy. Eleven of the 17 patients observed more than two years are free of disease, a cure rate much higher than the overall experience with neuroblastoma. Substantial recovery of neurologic function occurred in most, but kyphoscoliotic deformities were a disturbingly frequent late complication.

Extrahepatic portal hypertension in children. Long-term evaluation.

A long-term evaluation of 43 children with extrahepatic portal hypertension indicates a high success rate and excellent subsequent health when a splenorenal or mesocaval shunt can be performed. On the other hand, makeshift shunts inevitably fail. Direct operations are considerably less successful than standard shunts. Esophagogastric resections with interpositions result in long-term freedom from bleeding in only about half the cases, and portoazygous disconnection procedures have been uniformly disappointing. Sclerotherapy in a relatively recent experience has been quite successful, but long-term results are presently unavailable. The condition carries a significant mortality rate. Complications from failed operations, division of the vena cava, and multiple transfusions are numerous. The general health of long-term survivors is excellent in those with successful operations, and is surprisingly good for patients whose operations have been unsuccessful and for those who have had no operations.

Acetylthiocholinesterase staining activity of rectal mucosa. Its use in the diagnosis of Hirschsprung's disease.

Increased acetylthiocholinesterase (AchE) reactivity in the rectal lamina propria and lamina muscularis mucosae was used to diagnose Hirschsprung's disease. We processed 131 specimens with the AchE reaction; 43 were suction biopsy specimens and the rest were full-thickness specimens. Of the 68 specimens in which neurocytes were present, none demonstrated a diffuse increase in the number of nerve fibers. However, 15 showed focal increases in the numbers of fibers that were not large enough to be regarded as indicative of Hirschsprung's disease. All patients with a diffuse increase in nerve fibers, regardless of the type of biopsy, were shown to have Hirschsprung's disease. The AchE staining reaction did, however, produce a 29% rate of false-negative reactions (16 of 56 specimens) in patients with Hirschsprung's disease. These data demonstrate that an abnormal pattern of AchE reaction is diagnostic of aganglionic megacolon, whereas a normal pattern does not exclude the disease.

Iatrogenic ileal atresia secondary to clamping of an occult omphalocele.

Clamping of an occult omphalocele has led to complete division of an entrapped loop of ileum in two instances. The proximal end has been sealed in the process, producing an iatrogenic ileal atresia. The clinical picture in both instances differed from that usually found with ileal atresia. Escape of a small amount of meconium from the transected cord was noted in one, and the stump of the cord in the other appeared red and engorged. Clamping the umbilical cord routinely at least 5 cm from the abdominal wall is recommended.

Glucagon in experimental intussusception.

A standard type of ileocolic intussusception was produced in 69 puppies and the effect of glucagon on the hydrostatic reduction of the intussusceptions evaluated in a prospective, double-blind study. The reductions were attempted at intervals varying from 18 to 60 hr following the production of the intussusceptions. The overall reduction rate was 70% and there was no statistical difference in this rate between the animals receiving glucagon and those receiving placebos. None of the gangrenous intussusceptions were reducible with the hydrostatic pressure technique, irrespective of whether glucagon was used or not. A further evaluation was made of those animals in whom successful reductions were accomplished by the hydrostatic pressure method. Glucagon did result in significantly easier reductions, and an earlier return of normal vascular supply as measured by color and by arterial pulsations as compared to the control group.

Secondary thyroid neoplasms in pediatric cancer patients: increased risk with improved survival.

Between 1973 and 1983, eight children who had undergone successful multimodal management of malignant tumors developed secondary thyroid neoplasms. The primary tumors were acute lymphocytic leukemia in three, Wilms' tumor in two, and Hodgkin's disease, rhabdomyosarcoma, and ganglioneuroblastoma in one each. During this period, 174 long-term survivors with these five diagnoses were enrolled in our tumor registry, yielding a 4.6% incidence of secondary thyroid neoplasms. All eight patients received both radiation and chemotherapy. The mean radiation dose was 2,700 r with a calculated thyroid dose of 2,140 r (range, 5 to 4,200 r). Age of diagnosis of the primary tumors ranged from 1 to 8 2/12 years (mean, 5 years), and the latent period between treatment and development of the thyroid lesions averaged 6 1/2 years. Thyroid neoplasms presented at an average age of 11 4/12 years. Five patients developed solitary adenomas, one presented with multiple adenomas, and two had follicular carcinoma with regional lymph node metastases. Although thyroid neoplasms are rare in childhood, clinically apparent thyroid tumors have been observed in up to 2.5% of children following radiation exposure (mean follow-up, 24 years). The reported latent period before the development of thyroid neoplasms in irradiated patients is at least 10 years, with the peak incidence occurring 20 to 25 years after exposure. This study documents a 4.6% incidence of subsequent thyroid neoplasms in pediatric cancer patients within a relatively short follow-up period (mean, 11 years). These thyroid tumors occurred at an earlier age (mean, 11.5 years) and with a shorter latent period (mean, 6.5 years) than would be predicted from previous studies.(ABSTRACT TRUNCATED AT 250 WORDS)

Atresia of the colon.

Eleven infants with colon atresia have been managed by staged procedure with survival and good health in ten. Complications have been relatively few. At the initial operation the proximal atretic segment is exteriorized as an end colostomy. Such a procedure is simple and safe, results in rapid relief of the obstruction, and permits normal feeding by mouth within a few days. At a second procedure several weeks or months later, intestinal continuity is established by an end-to-end or end-to-side anastomosis. Gastroschisis has been an associated anomaly in four of these cases. This association gives additional support to the concept that intrauterine interference to the blood supply to a segment of intestines is the etiologic factor responsible for such atresias.

The use of glucagon in the diagnosis and management of illeocolic intussusception.

Twenty-one of 25 episodes of ileocolic intussusception were successfully reduced by the barium enema hydrostatic technique supplemented by glucagon. No apparent serious complications occurred from the use of this drug. The comfort of the children and the ease of filling the colon and small bowel seemed enhanced by the glucagon.

Congenital vas deferens--ureteral connection.

Congenital drainage of the vas deferens into a ureter permits retrograde passage of urine through the vas to the epididymis. If this anomaly is accompanied by urinary tract pathology with vesicoureteral reflux, hydroureter, stasis, and infection, the passage of urine into the vas can very well lead to epididymitis of a chronic or recurring nature and secondary scrotal abscesses. Two cases of this rare anomalous connection are presented in which such scrotal infections occurred. The diagnosis should be suspected on clinical grounds and can be confirmed by cystograms in which the dye refluxes up the ureter and passes into the vas. The association of rectal anomalies is of embryologic significance and may be additionally important clinically by further suggesting the possibility of the ureteral-vas anomaly.

Gastroschisis update.

Sixty-four infants with gastroschisis have been managed in the 9-yr period, 1970-1979, with four postoperative deaths. The silo technique has been the standard method of management in this series, permitting initial expansion of the abdominal cavity without increased abdominal pressure and respiratory embarassment. Removal of the silo and complete closure of the abdominal wall deficit were possible 5-12 days later. A high proportion of the infants were below 2500 g in weight at birth (61%); and although 3 of the 4 postoperative deaths occurred in the low birth weight group, this did not appear to be a factor in mortality. Associated anomalies occurred in 25 of the 64, but only the coincident intestinal atresias (6) were of major significance. These additional anomalies were not responsible for deaths. Two deaths occurred from problems dating from birth, one from aspiration and the second from sepsis. The other two resulted from postoperative complications resulting in infarction of the midgut. Both were caused in part by failure to adequately enlarge the abdominal wall defect at the time of the initial procedure. Other postoperative complications were relatively few. Although all required intravenous nutritional support, the long term results in terms of growth and development and of intestinal function were quite satisfactory.

Blue Rubber Bleb Nevi as a cause of intussusception.

The Blue Rubber Bleb Nevus syndrome is a rare disease characterized by a distinctive type of hemangioma which involves the skin and the gastrointestinal tract. In the latter location, these lesions are often responsible for chronic blood loss and secondary anemia, and in rare situations may act as a leading point for an intussusception. The diagnosis of intussusception in children older than 3 or 4 yr is frequently difficult and delayed. In a child with typical skin lesions of the Blue Rubber Bleb Nevus syndrome, an acute illness with manifestations of intestinal obstruction should indicate the possibility of an associated intussusception.

Esophageal sclerotherapy: an effective modality in children.

During the past five years, sclerotherapy has been used at our institution in 13 children for the management of recurrent major variceal bleeding. The varices were secondary to extrahepatic portal hypertension in seven patients and to intrahepatic portal hypertension in the remaining six. Sclerotherapy was performed under direct vision using either rigid or flexible endoscopic equipment, and the sclerosing agents were injected directly into the varices. The average age at initiation of sclerotherapy was 9 years (range: 1 to 19 years). The follow-up has ranged from 2 to 4 1/2 years with a mean of 3 1/2 years. Complete obliteration of all varices was obtained in eight of these patients. Two children have minimal residual varices, in one of whom 17 sclerotherapy procedures have been performed to date. One additional patient had a severe episode of bleeding during esophagoscopy, and transesophageal ligation of varices was required for control. Two patients have died following initiation of sclerotherapy. In neither case was the death the result of bleeding esophageal varices or a complication of endosclerosis. Bleeding from varices was the major clinical problem in all of these children, and this problem has been largely corrected by the sclerotherapy program. With one exception, there have been no episodes of variceal bleeding requiring transfusion in these patients following initiation of this therapy. One child developed an esophageal ulcer postinjection, but none have developed esophageal strictures. One patient developed an allergic reaction to the sclerosant that was treated during subsequent injections with prior administration of an antihistamine (diaphenhydramine chloride) and steroids.(ABSTRACT TRUNCATED AT 250 WORDS)

An ultrastructural study of the intimal injury induced by an indwelling umbilical artery catheter.

In an experimental study, catheterization of the abdominal aorta with small umbilical artery catheters consistently produced significant intimal injury. The duration of catheterization varied from 1 hr to 7 days, and recovery periods following catheter removal varied from 1-150 days. Endothelial disruption was observed in all animals sacrificed within 24 hr of catheter removal. Healing progressed rapidly when the duration of catheterization was short, but was protracted with longer periods of catheterization. Thrombi were frequently found adhering to the exposed subendothelial surface. Routine light, scanning, and transmission electron microscopy demonstrated consistent cellular changes, which depended upon the duration of catheterization and the length of recovery periods. A consistent relationship between the degree of intimal injury and the duration of catheterization was observed.

Pancreatic pseudocyst complicating treatment of acute lymphoblastic leukemia.

In the management of children with acute lymphoblastic leukemia, L-asparaginase has become established as an effective drug in the usual multi-agent therapy; and the significance of pancreatitis as a complication of this drug is well recognized. Less well appreciated, however, is the progression of such pancreatitis in some patients to pseudocyst formation and the possible necessity for surgical management. Two adolescent girls who developed pancreatic pseudocysts while being treated with L-asparaginase are described in this report. Both were being treated for acute lymphoblastic leukemia for periods of 18 and 4 months, respectively, prior to the onset of pancreatitis. Both were in remission of their leukemic disease when typical clinical and laboratory manifestations of acute pancreatitis developed. In one girl, a pancreatic pseudocyst became apparent 2 weeks following the diagnosis of acute pancreatitis and in the other girl, this complication developed over a period of 8 weeks. The usual nonsurgical management of pancreatitis over protracted periods of time was ineffective in the treatment of the pseudocysts. Surgical drainage (internal in one and external in the other) was successful in both in eradicating the pseudocyst, and in neither did further evidence of pancreatic disease subsequently occur. In both resumption of chemotherapy, omitting L-asparaginase, was well tolerated. One has been in remission of leukemia and in good health for a 3-year period of follow-up observation, while the other subsequently had a relapse of leukemia and died 18 months following the onset of pancreatitis.

Aeromedical transportation for infants and children.

Transfer of a critically ill child to a pediatric center is indicated when patient needs have exceeded local expertise or facilities. It is illogical to impose on the referring hospital the added burden of transporting such a patient. The receiving hospital is better equipped to extend its specialized services by assuming responsibility for interim management and transfer of the patient. An aeromedical transport system capable of quick response was built around National Guard helicopters. In 21 cases, doctors, nurses, and equipment were flown to children in community hospitals, and urgent treatment was begun in half the time formerly possible. Only after the patients were stabilized were they returned safely under continuing appropriate management. The helicopter is a versatile and speedy tool to deliver expertise and equipment when and where needed. Timely arrival of specialty services can be expected to improve patient care and salvage.

Partial splenectomy in staging laparotomy for Hodgkin's disease: an alternative approach.

Partial splenectomy, with resection of the lower one-fourth to one-third spleen, was performed in 10 children with Hodgkin's disease as part of an otherwise standard staging laparotomy. The technique proved to be safe and practical, with no postoperative complications. No evidence to date indicates that splenic involvement was missed, and all the children have thus far continued to do well, with no deaths from either underlying disease or postsplenectomy sepsis. The procedure is undergoing a prospective clinical trial in an effort to determine whether or not it is comparable from a diagnostic point of view to total splenectomy. Available data indicate that the risk of missing intraabdominal disease in a staging laparotomy with a partial splenectomy is quite low. Such a procedure should eliminate the substantial risk of postsplenectomy sepsis in such children.

The value of radionuclide scanning in early diagnosis of intestinal infarction.

Small focal areas of intestinal infarction were produced experimentally in mice by two techniques: (1) intussusception and (2) suture strangulation of a loop of jejunum. The radionuclide technetium 99m diphosphonate (99mTc-diphosphonate) was subsequently administered intravenously, and abdominal scans were obtained. The scans demonstrated focal infarcted lesions accurately, and every positive scan was associated with an area of infarction, as demonstrated by pathologic examination. However, negative scans did not definitely exclude an area of infarction: in the intussusception study there were five false negative scans in 25 animals, and in the loop ligation study there were two false negative scans in 28 animals. The technique may have clinical application in such intestinal diseases as necrotizing enterocolitis in infancy in which early diagnosis of small areas of necrosis is currently difficult by clinical and radiologic methods.