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Background: The purpose of this study was to review our experience and early follow-up with 15 one-stage pull-through procedures performed at the time of diagnosis in neonates and infants with Hirschsprung's disease., Methods: Historic chart review of all patients with the new diagnosis of Hirschsprung's disease seen at the C.S. Mott Children's Hospital at the University of Michigan, Ann Arbor, between June 1989 and June 1992 was performed. Progress notes, operative and anesthetic records, pathology reports, and outpatient follow-up notes were used for data collection., Results: Operative technique included a modified endorectal pull-through after determining the presence of ganglion cells by frozen section. No colostomies were performed before or after operation. Three patients had affected family members. All infants were born at term. Operation was performed within 24 hours of diagnosis and as early as 48 hours of age. Twelve patients had standard rectosigmoid disease, two had total colonic disease, and another had long-segment disease. Bowel function returned within 4 days of operation, and feeding was instituted by postoperative day 6 in all patients. All of the patients with rectosigmoid disease are doing well. Patients with long-segment or total colonic disease had more problems but are currently at home and doing well. Morbidity included two postoperative bowel obstructions and three patients with postoperative enterocolitis., Conclusions: A single definitive operation may be used to treat Hirschsprung's disease diagnosed in the newborn. Long-term follow-up in these patients is required.

Systemic oxygen delivery (DO2) is normally four to five times higher than oxygen consumption (VO2), and VO2 is independent of DO2. If DO2 is decreased to less than twice VO2, a state of anaerobic metabolism and supply dependency occurs. Some authors have reported that this biphasic relationship is altered in the adult respiratory distress syndrome or sepsis to a condition of continuous supply dependency. If that were true, it would affect both our understanding and management of metabolism during sepsis. Therefore we measured VO2 and DO2 in a dog peritonitis model. DO2 was regulated with controlled pericardial tamponade. During sepsis VO2 increased 28% from a mean baseline of 5.6 to 7.3 cc O2/kg/min (p less than 0.005). As progressive cardiac tamponade was applied during sepsis, the DO2/VO2 ratio fell. When the DO2/VO2 ratio was greater than 2.4, VO2 remained independent of DO2. At DO2/VO2 ratios less than 2.4, VO2 was dependent on the level of DO2, and it diminished rapidly as DO2 decreased. Oxygen saturation in mixed venous blood (SvO2) consistently reflected the DO2/VO2 ratio in a fashion similar to that in normal dogs. A ratio of DO2/VO2 of 2.4 corresponded with an SvO2 of 42% +/- 12%, which was identified as a statistically significant critical SvO2 that marked onset of VO2 supply dependence. In this dog septic model, VO2 is independent of DO2 when DO2 is adequate. A state of continuous supply dependency does not exist. SvO2 reflects the status of the DO2/VO2 relationship in the septic state.

Abdominal aneurysms are rare in children and are usually found in association with congenital cardiac or aortic malformations, connective tissue disorders, trauma, or previous arterial catheter placement. A 4-year-old girl who had a common iliac artery aneurysm, who had no history of arterial catheter placement or trauma, and who had no evidence of Marfan's or Ehlers-Danlos syndrome, arteritis, coarctation of the aorta, or other diseases associated with childhood aneurysms is presented. Resection of the aneurysm and arterial reconstruction were performed without the use of prosthetic material or vein graft. Pathologic examination showed no evidence of inflammation or medial degeneration in any of the layers of the arterial wall. This is the fourth report found in the literature of documented idiopathic abdominal aneurysm in a child. The conditions associated with abdominal aneurysms in childhood are discussed, and the literature is reviewed.