Early recognition and surgical treatment of Hirschsprung's disease prevents serious mortality and morbidity from enterocolitis and obstruction. Usually this disease is characterized by a single aganglionic segment of the colon extending distally to the anal margin. In surgical treatment, the surgeon performs a frozen section biopsy to confirm whether there are ganglion cells. If there are intervening ganglionic sites in aganglionic bowel, there may be confusion in diagnosis and treatment. The authors experienced one case of total colonic aganglionosis with skip area. A transverse loop colostomy was performed on a 7 day-old male baby with colon perforation due to Hirschsprung's disease. But intestinal obstruction persisted and required two more operations to find the true nature of the disease. There were aganglionic segments from the anal margin to the terminal ileum 3.7cm proximal to the ileocecal valve. The entire transverse colon and appendix were normally ganglionated.
Infantile hypertrophic pyloric stenosis(IHPS) is common in full-term babies, and relatively rare in prematures. The diagnosis of IHPS in premature infants may be obscured because of the lack of classic symptoms and signs and the absence of the standard criteria for ultrasonic diagnosis. The purpose of this study is to discover the clinical differences between premature and full-term infants with pyloric stenosis, and determine the appropriate diagnostic methods for early diagnosis in premature infants. The clinical records of 52 IHPS patients who had been operated upon from October, 1994 to April, 1997 were reviewed. The incidence of IHPS in premature infants was 25 %. The onset of symptom was 4.7 weeks of age in premature, and 2.9 weeks in full-term babies. Diagnosis was established by typical symptoms. signs. and diagnostic imaging studies. In two premature infants, diagnosis was confirmed by upper gastrointestinal(GI) series, because ultrasonography did not meet the diagnostic criteria. Two premature infants initially diagnosed as gastroesophageal reflux by esophagography. were found to have IHPS by upper GI series. For the diagnosis of IHPS, a new set of criteria for premature babies has to be developed.
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The omphalomesenteric duct normally obliterates by the sixth week of intrauterine life. Incomplete obliteration results in various abnormalities which may be apparent in the newborn infant. These include fistula resembling ileum, a prolapsed loop of intestine through the umblicus and a fistula draining intstinal contents. The tract may contain ectopic tissue of stomach, colon or pancreas. Although this malformation should be recognizable at birth, 40 % of patients are not treated until after the first month of life. In the past 28 months since .the Samsung Medical Center opened its doors to public, the authors have treated 4 cases of patent omphalomesenteric duct remnant includng one case of T-shaped total prolapse of the duct and adjacent ileum. These cases will be discussed and the literature reviewed.
Pulmonary sequestration is a complex anomaly involving the pulmonary parenchymal tissue and its vasculature. It presents as a cystic mass of nonfunctional lung tissue without communication with the tracheobronchial system. Usually, it receives blood supply from anomalous systemic vessels. Therefore, preoperative diagnosis of the pulmonary sequestration is difficult, especially when it is located in the abdomen and combined with congenital cystic adenomatoid malformation (CCAM). We encountered such a mass(CCAM type 2) detected prenatally by ultrasonography. It was a kidney bean shaped, pinkish mass straddling the thorax and abdomen on the right side. Because of the sonographic appearance, neuroblastoma was diagnosed preoperatively. The mass was completely extirpated without difficulty.
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