Biomedical subjects
Publications and source records attributed to T V Santulli.
The aortic arch can be clearly demonstrated in neonatal chest radiographs by use of a high kilovoltage-filtered film with air-gap magnification. This allows identification of the presence of right aortic arch in patients with esophageal atresia and tracheoesophageal fistula; awareness of this anomaly may influence choice of surgical approach.
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Calcified caval thrombus should be considered in any infant or child where calcifications are noted in the high right retroperitoneal area on plain x-rays of the abdomen. Although typically bullet-shaped in configuration, the calcium distribution in the neonate may be atypical or incompletely developed, suggesting neuroblastoma. Definitive diagnosis can be made by inferior vena cavagram. As no deaths or complications have been attributed to the lesion in the cases thus far reported, no specific treatment is recommended.
Rarely, the mesoappendix forms the sliding component of an inguinal hernia. Appendectomy, in such instances, permits effective repair of the hernia. This was accomplished in 16 patients without wound complication or recurrence of the hernia.
Three cases of Wilms' tumor with inferior vena cava involvement are presented. All three cases were shown to have nonvisualization of the inferior vena cava on the initial intravenous pyelogram obtained by internal saphenous vein injection. The first case ended fatally, due primarily to delay in diagnosing extension of the tumor into the right atrium. There was no cardiac involvement in the other two cases, both of which were managed successfully. A right heart angiogram demonstrated intraatrial tumor in the first case and helped to identify the presence and extent of caval involvment in the other two. It is recommended that right heart angiography be performed in those cases of Wilms' tumor where the inferior vena cavagram shows non-visualization of the cava or suggests the presences of intraluminal tumor. The immediate availability of two separate surgical teams in the management of such cases is suggested.
The effects on hepatic growth of fat-free TPN and semistarvation have been studied in newborn beagle puppies. Our data indicate that liver growth during TPN is characterized by normal deposition of protein and minerals, with increased glycogen and decreased lipid content. While the lipid pattern of TPN livers is normal, the phospholipid fraction contains an abnormal fatty acid pattern, consistent with the development of essential fatty acid deficiency. The exact physiologic significance of this is unknown, but it is tempting to speculate that changes in integrity of the hepatic cell membrane as a result of this abnormal fatty acid pattern might account for some of the clinical abnormalities of hepatic function during TPN.
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Despite the fact that necrotizing enterocolitis is considered a disease of premature infants, 20% of all affected infants at Babies Hospital over the past 20 years were products of term gestations. Two distinct subgroups of such infants were noted (1) five infants with congenital heart disease and/or congestive heart failure (e.g.hypoplastic left heart syndrome), all but one of whom developed the disease in the first week of life; (2) eight infants who developed the disease at a much later age after a protracted period of diarrhea. This histopathologic features of the disease in term infants are the same as those in premature infants. Further, the pathogenesis of the disease in term infants does not appear to differ basically from that in premature infants. These facts, lead away from the concept of NEC as a disease of simple etiology.
Nineteen cases of choledochal cyst are reviewed. Two distinct groups of patients were identified. Patients under one year of age, initially diagnosed as having biliary atresia, had a higher mortality rate, a higher incidence of severe cirrhosis with portal hypertension, and associated atresia or stenosis in the biliary tree. The second group, presenting between 3 and 20 years of age with more classic symptoms, had mild cirrhosis without portal hypertension and had associated choledocholithiasis and pancreatitis. It is suggested that the younger patients had a congenital form of cystic bile duct dilatation and that the older patients had an acquired form, perhaps related to a common channel with reflux of pancreatic juice into the common bile duct. Postoperative follow-up supports the current view that choledochocyst-jejunostomy with choleystectomy has a lower rate of long-term complications than does choledochocyst-duodenostomy.
Five newborn girls presented with small intestinal obstruction and microcolon and a giant bladder (megacystis). Organic causes of obstruction were not found, and the gastrointestinal tract failed to function after appropriate diversion. Two died in the postoperative period, two lived several months on central venous hyperalimentation, and one died at 34 months of age following chronic though intermittent hyperalimentation. Pathologic studies showed an abundance of ganglion cells in both dilated and narrowed areas of intestine; the combined small bowel-colon length was one-third of normal in the absence of an evident obstructive or vascular insult. The five patients represent the most severe manifestation of defective intestinal peristalsis in a larger group of distended newborns in whom organic gastrointestinal obstruction is not found. Treatment with central venous hyperalimentation may sustain life, and some patients eventually recover gastrointestinal function. The hypoperistalsis is largely refractory to pharmacologic treatment; its cause is unknown.
It is very uncommon for acute thrombophlebitis to develop in children without an identifiable predisposing cause. During a ten year period at a children's hospital, central nervous system disease was observed in four of eight children with iliofemoral thrombophlebitis. This association suggests that a thorough neurologic evaluation should be carried out in any child with acute thrombophlebitis.
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A total of 120 children with both high, intermediate, and low types of imperforate anus were evaluated urologically. Genitourinary anomalies were associated with all types of imperforate anus but were found more often in children with high rectal deformities. Because the over-all incidence of genitourinary anomalies was high, early and complete urologic evaluation is necessary to achieve the best functional result.
Angiography has been used successfully in adults for evaluation following major trauma and to subsequently control hemorrhage associated with pelvic fracture by selective embolization. This report illustrates that the technique is applicable in the evaluation and treatment of children with similar injuries.
Necrotizing enterocolitis, a highly lethal disease in the newborn infant characterized by ischemic necrosis of the gastrointestinal tract frequently leading to perforation, is seen primarily in low birth weight infants who have undergone stress, such as hypoxia. In an animal model it was demonstrated that cold stress was as effective as hypoxia in producing the disease in formula-fed newborn rats. Breast milk was completely protective in both cold- and hypoxic-stressed animals. Presumably cold stress produces the same selective circulatory ischemia as does hypoxia. The experiment further supports the concept that any insult or stress which decreases mesenteric blood flow may initiate the changes leading to necrotizing enterocolitis. It was shown also that the incidence of the disease in formula-fed rats was related directly to the number of episodes of either cold or hypoxic stress. These results suggest that a critical amount of ischemia is necessary to initiate these changes and may help to explain the fact that not all infants exposed to hypoxia or cold stress developthe disease.