Biomedical subjects
Publications and source records attributed to G Stringel.
We report 16 cases of lower limb hypertrophy seen in the past 5 years; of these, four had a typical picture of Klippel-Trenaunay syndrome. We discuss diagnostic modalities, complications, treatment, and outcome. Special emphasis is placed on the treatment with intermittent pneumatic compression and the importance of an organized approach to the condition.
The ideal surgical closure for large omphaloceles remains controversial. Most techniques described advocate removal of the amniotic sac prior to repair of the abdominal wall defect. Herein, the authors describe a surgical technique preserving the amniotic sac, which has distinct advantages in achieving safe and rapid closure of the omphalocele by primary or secondary means in selected patients.
Extensive aganglionosis including the ileum occurs in less than 5% of children with Hirschsprung's disease; in these cases nutrition, fluid, and electrolyte balance are a problem. The author reports an infant with extensive aganglionosis including the ileum in whom an endorectal pull-through of the Soave type as modified by Boley was performed with a longitudinal side-to-side anastomosis between the aganglionic and normal intestine, preserving terminal ileum and ileocecal valve. At two and one-half years of age the infant is well. He is toilet trained and has 1 to 4 soft bowel movements per day as he is in the 50th percentile for weight. When extensive aganglionosis involves the small intestine, saving the important function of the terminal ileum and the ileocecal valve is possible and highly desirable.
Bloody nipple discharge in infancy has been rarely reported in the medical literature. Its cause is unknown. We report a three-year-old male infant and a five-month-old female infant with bloody nipple discharge. Because of persistent bloody discharge, a subcutaneous mastectomy was performed in the boy; the problem resolved in the girl after a period of observation. The specimen showed histologic changes identical to those seen in adult mammary duct ectasia. All the endocrinologic work-up was normal. We suspect that bloody nipple discharge in infancy is underreported. This is a benign condition with histologic changes similar to adult mammary duct ectasia and if persistent, should be properly investigated; biopsy or excision are not indicated.
About 10% to 15% of all duplication cysts in the alimentary tract are esophageal. Esophageal duplication cysts are intimately attached to the alimentary tract, are lined by mucous membrane and have smooth muscle. This paper describes a 2-year-old child who presented with symptoms of progressive respiratory distress. A diagnosis of esophageal duplication cyst was made. At surgery a low cervical incision was made and the sternal manubrium split, thereby providing adequate exposure. The cyst was then removed. The most useful investigations were chest roentgenography and barium esophagography. Computerized tomography showed a small, round foreign body in the middle of the cyst that was subsequently found to be a bingo chip. Communication between the cyst and the esophagus was not obvious at the time of surgery and had not been demonstrated by barium esophagography. When complete excision of the cyst is not possible because of inflammatory reaction all the mucosa must be removed to prevent recurrence. Careful postoperative respiratory support and broad-spectrum antibiotic therapy are recommended.
Total colectomy with endorectal mucosectomy and ileal pull-through to preserve fecal continence is well known for ulcerative colitis. A female patient developed severe ulcerative colitis at 5 years of age and had an emergency subtotal colectomy and ileostomy at 7 years of age. The rectum was preserved. At 9 years of age rectal mucosectomy and a pull-through using isolated jejunal loop were performed. Postoperatively she was treated with ano-rectal physiotherapy and balloon dilatations of the new rectum. Six months later the ileostomy was closed. Two and a half years after her operation she has 2-4 soft bowel movements daily, perfect diurnal control with occasional night soiling. A pull-through using an isolated loop of jejunum leaves intact a good functioning ileostomy. The blood supply to the jejunum is better than the terminal ileum. Bacterial contamination with jejunum is less than with ileum. There is no need for a reservoir; if the pull-through fails valuable terminal ileum is not lost.
Hypoglycemia secondary to organic hyperinsulinism in children can be caused by diffuse or localized pancreatic lesions. Differentiation between these two types of lesions is of utmost importance since the surgical approach will be different. Some tumors escape detection by all preoperative investigations including ultrasound, scintiscan, arteriography, and computerized tomography. We are reporting on a 12-year-old boy with organic hyperinsulinism in whom we were unable to localize a tumor preoperatively. Peroperative determination of insulin levels from a number of sites on the pancreas enabled us to localize an insulin-producing pancreatic adenoma. This technique can be done easily by catheterizing the splenic and portal vein through a branch of the splenic vein and by serial sampling at 2 cm intervals along the portal and splenic veins. Insulin levels at these sites were determined by quick double-antibody radioimmunoassay, which allows the determination of the insulin levels within 50 minutes after sampling. There was a perfect biochemic and anatomic correlation allowing us to perform a precise distal pancreatectomy. The technique can be used to localize pancreatic adenomas and to decide how much pancreas to remove in diffuse lesions avoiding "blind" pancreatectomies.
Condyloma Acuminata (venereal warts) are sexually transmitted involving the human papilloma virus. It has become commoner in adults in the last decade. While infection is most often by sexual intercourse, it can be transmitted at birth and with close contact with infected individuals. It is common in marked sexual promiscuity. Condyloma acuminata in children should alert the physician to the possibility of sexual abuse or early sexual activity. We report 14 cases of condyloma acuminata in children; from 6 months to 17 years. Sex incidence was equal. All had social and family problems in common, except for one who developed perianal condyloma after repeated rectal dilatations because of a pull through procedure for Hirschsprung's Disease. Two cases of sexual abuse were documented. Treatment methods included podophyllin, liquid nitrogen, 5 fluorouracil cream, fulguration and laser therapy. An adequate social history was available only in six cases. Investigations should include VDRL and cultures for Gonococcus, careful medical and social history for neglect or abuse.
Abscess of the spleen is a rare entity. It may develop after generalized infection, hematologic disorders and trauma. The authors report the case of a 7-year-old boy who presented with a 2-month history of spiking fever, anorexia, fatigue and weight loss. He had left subcostal tenderness and a palpable spleen. He had a history of trauma to the left flank 5 months before admission but a splenic scan obtained at that time appeared normal. A splenic abscess was diagnosed by gallium scanning, computerized tomography and ultrasonography. Although splenectomy has been advocated as the treatment of choice for splenic abscess, this patient was treated successfully with appropriate antibiotics and simple drainage, preserving the spleen. Cultures grew Staphylococcus aureus. Of all the diagnostic methods available, ultrasonography is the least invasive study that will make the diagnosis and is less expensive than some methods.
Intestinal perforation after blunt abdominal trauma in children is rare and thus the diagnosis may be delayed. For this reason the authors reviewed their experience with 12 children to recommend a protocol for investigation that would reduce the delay in diagnosis. Of the 12 perforations, 2 were gastric, 2 duodenal, 7 jejunal and 1 colonic. The diagnosis of jejunal perforation, in particular, was usually delayed because free air was not seen radiologically in the first few hours after injury. This may be because of delayed rupture or spasm of the injured intestine. Serial films were valuable in aiding the diagnosis and are recommended, together with assessment of solid organ injury by radionuclide scanning. In this series peritoneal lavage was not used. No child died.
Catheterization of the umbilical artery is a common procedure in neonatal intensive care units. The authors studied the records of 100 consecutive newborns who underwent this procedure to review the indications for and complications of umbilical artery catheterization and to discuss preventive measures and alternative techniques. Only polyvinylchloride barium-impregnated catheters were used (nos. 3.5 and 5.0 French). The commonest indications were respiratory distress syndrome, asphyxia and congenital heart disease. Of the 100 infants, 75 weighed less than 2500 g. Ampicillin and calcium were the commonest medications infused (70 and 65 babies respectively). There were three major complications, two of which were gangrene of the lower extremity. Amputation of the foot was necessary in one and amputation of the toes in the other. The third complication was the development of gluteal necrosis. In all three cases, catheter placement was low and the infusion was ampicillin. In two of the babies, calcium was also administered. Minor complications were seen in 32 cases, with vascular spasm in the lower limb being the most common. All catheter tips were cultured; there was bacterial colonization in 13%, Staphylococcus epidermidis being the commonest organism. Proven necrotizing enterocolitis was seen in eight infants and was suspected in eight others. Blanching is a serious sign and was seen in the three infants with major complications. The infusion should be stopped immediately. When necrotizing enterocolitis is suspected, the catheter should be removed.
Most hemangiomas do not cause major problems but they may be functionally or emotionally unacceptable. In uncomplicated cases spontaneous involution is frequent and treatment consists of expectant care. We report three newborn babies and two infants with giant hemangiomas in whom acute life-threatening complications required immediate treatment. Complications included cardiac failure, hemorrhage, platelet trapping, and disseminated intravascular coagulation (DIC). Modes of therapy employed were surgical excision, steroids, embolization, radiotherapy, intermittent pneumatic compression (Jobst), and continuous compression treatment (Jobst). Our five cases show that several modalities of treatment may be necessary to control the life-threatening complications of giant hemangiomas.
Chylothorax occurring postoperatively in children is found most commonly after cardiovascular operations, but may occur after almost any thoracic procedure. Seven cases of postoperative chylothorax seen at the Children's Hospital of Eastern Ontario in Ottawa from 1976 to 1983 are reported. Two resolved with nonoperative management and five required surgical intervention. The authors discuss the clinical presentation, treatment and outcome. They describe a simple, safe and effective method of ligation of the thoracic duct just above the diaphragm.
Eosinophilic gastroenteritis is a rare condition, characterized by diffuse infiltration of the gastrointestinal tract by eosinophils. This involves mainly the stomach, duodenum and small bowel and rarely the esophagus, colon, pancreas, gallbladder, prostate and urinary bladder. Most reported cases have occurred in adults in the third decade of life. The authors report on a 15-day-old infant who initially presented with rectal bleeding and a scan that indicated a Meckel's diverticulum was present. At laparotomy, however, a Meckel's diverticulum was not found. The appendix was slightly distended and there was a mild inflammatory reaction. Appendectomy was performed. Microscopically the appendix showed diffuse eosinophilic infiltration. The blood eosinophil count was elevated. The patient recovered well, but had several minor episodes of rectal bleeding following ingestion of milk. He remained asymptomatic on a milk-free diet. The etiology of eosinophilic gastroenteritis is unknown and there is no specific treatment. In uncomplicated cases, diet, corticosteroids and adrenocorticotropic hormone are used. Operation is reserved for cases of obstruction and hemorrhage.
Idiopathic perforation of the bile duct is rare in children. Sixty-seven cases were reported in the English literature to 1980. It is, nevertheless, the second commonest surgical cause of jaundice in the neonate. The etiology is unknown though distal obstruction and weakness in the bile duct wall have been postulated. Limited surgical treatment with external drainage is the preferred therapy. In isolated cases internal drainage procedures or repeated aspiration have been successful. The diagnosis should be suspected in the presence of jaundice and ascites with or without abdominal pain and signs of peritoneal irritation. We describe a 3-month-old girl presenting with anemia, vomiting, jaundice, and ascites. This was initially diagnosed as hepatitis but bilious fluid was found on paracentesis. Computerized tomography with cholangiography and 99 MTC Diisopropyl IDA cholescintigraphy confirmed the diagnosis. The latter seems to be more accurate than I-131 Rose Bengal. The perforation was at the junction of the hepatic and cystic ducts. It was treated successfully by external drainage and a cholecystostomy. Direct attempts to close the perforation, or more complicated surgical procedures, are unnecessary while nonoperative treatment carries a high mortality. At follow-up after 1 year the IV cholangiogram and liver-function tests are normal. Cholecystostomy provided good drainage of the biliary ducts as well as easy access for follow-up cholangiography.
Campylobacter jejuni Skirrow biotype 1, Lior serotype 8 was isolated from the appendix of an 11-year-old boy who had a 6-h history of acute abdominal pain. Histological diagnosis on the appendix section was early acute appendicitis. Dilute carbol fuchsin stain and indirect fluorescent antibody test performed on the appendix section also revealed the presence of Campylobacter sp. The patient developed a significant bactericidal antibody titer of 1,024, providing substantial clinical evidence of the pathogenicity of the isolate. This case indicated that not only may abdominal pain caused by Campylobacter enteritis mimic appendicitis, but the organism may actually be recovered from the infected appendix.
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