A Clinical Review of Esophageal Atresia : One Surgeon's Experience

From 1979 to 2006, fifty eight patients with esophageal atresia were treated by one pediatric surgeon at Hanyang University Hospital. We analyzed the clinical findings and outcome of these 58 patients. There were 30 males and 28 females. Their mean birth weight was 2,960 +/- 400 g (1,170~4,020 g). The most common type of anomaly was Gross type C (49 patients; 84.5 %). There was no type B. Fifty-two patients underwent definitive surgery. Postoperative complications were as follows: anastomotic leakage in 17 patients (32.7 %), anastomotic site stricture in 15 (28.8 %), gastroesophageal reflux in 10 (19.2 %) and recurrent TEF in 1 (1.9 %). A total of 152 associated anomalies were detected from 48 patients (82.2 %). The cardiovascular system was the most commonly affected (30 patients with 46 anomalies). The VACTERL association was present in 14 patients (24.1 %). Operative mortality was 17.3 % including self-discharge patients after operation.

Long-term Outcome after Surgery for Biliary Atresia (Study of 10 patients surviving more than 10 years)

Biliary atresia (BA) is the result of fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Kasai portoenterostomy has been the standard operative procedure in biliary atresia. Recently, there has been remarkable increase in the survival rate in cases of BA. However, long-term survivors are not clearly evaluated in Korea. To define long-term prognosis factors of patients who underwent surgery for BA, a retrospective study was undertaken of 10 (37 %) patients surviving more than 10 years among 27 patients who underwent one of Kasai procedures between 1981 and 1995. Hepatomegaly was present in 4 and splenomegaly in 7 patients. Serum bilirubin was normalized at 1 year after operation. Aspartate aminotransferase (AST, GOT), Alanine aminotransferase(ALT, GPT) were normalized at 12 years and alkaline phosphatase(ALP) was normalized at 13.5 years. Cholangitis developed mainly within 5 years after operation so close follow up is needed. Life long follow-up is needed because of progressive deterioration of liver function even after 10 years.

Clinical Study of Midgut Volvulus

Intestinal malrotation is usually asymptomatic and most often is found during abdominal exploration for other surgical diseases. However, a serious complication of intestinal malrotation, midgut volvulus is a true surgical emergency of childhood. The clinical findings, diagnostic procedures, treatment, and prognosis of midgut volvulus were reviewed by a retrospective study. Between 1980 and 2005, 29 patients with midgut volvulus ranging in age from 1 day and 15 years were treated at HanYang University Hospital. Seventy-nine percent of the patients presented before 1 month of age. Midgut volvulus occurred 2 times more frequently in male. The clinical findings were bilious vomiting (96.6 %), irritability (34.5 %), abdominal distention (13.8 %), abdominal pain (10.3 %), and palpable abdominal mass (6.9 %). The diagnosis was made by abdominal simple x-ray (17.2 %), upper gastro-intestinal contrast study (37.9 %), abdominal sonogram (20.7 %), abdominal CT (3.4 %), and abdominal exploration (20.7 %). Among the 29 patients, 5 patients developed gangrene of small intestine due to strangulation and underwent resection of bowel. Two patients died due to sepsis.

One-stage Repair of Neonatal Hirschsprung's Disease

Conventional treatment of Hirschsprung's disease consists of initial colostomy followed by pull-through operation. But, the treatment of Hirschsprung's disease has been changed along with the development of new surgical technique. Since 1995, endo-GIA has been available at our hospital and one stage Duhamel operation has been performed for neonatal Hirschsprung's disease. Between May 1995 and April 2006, 26 neonates have been treated with one stage pull-through operation by one pediatric surgeon at HanYang University Hospital. The sex ratio was 4.2:1 with male predominance. Clinical findings included abdominal distension (96.2 %), vomiting (50.0 %), delayed passage of meconium (46.2 %), constipation (23.1 %), and enterocolitis (15.4 %). Twenty two cases (84.6 %) were short-segment and 4 cases (15.4 %) were long-segment disease, of which 2 cases were total colon aganglionosis. One of the two patients with total colonic aganglionosis had double transition zones - distal ileum and hepatic flexure of the colon. The average age at operation was 14.56 +/- 8.77 days and the average weight at operation was 3.26+/-0.66 kg. Primary Duhamel operations were performed in 25 patients and Soave-Boley operations was performed in one patient. The endo-GIA 35 (Ethicon, USA) was used from 1995 until 1997, and after that endo-GIA 60 (USSC, USA) was used. The average Duhamel operation time was 88.57 +/- 22.80 minutes. Wound abscess (n = 2) and septum formation (n =1) occurred after Duhamel operation. Bowel function was normalized in 59 % within 3 months and in 95% within 1 year after operation. There was no mortality after one stage pull-through operation in neonate.

Clinical Study of Contralateral Inguinal Hernia Development in Children with Unilateral Inguinal Hernia

Contralateral exploration in children with unilateral inguinal hernia is controversial. This study was done to identify risk factors for the development of contralateral inguinal hernia in patients with unilateral inguinal hernia. The clinical experience of 4,206 inguinal hernias repaired by one pediatric surgeon on 3,358 children at HanYang University Hospital from September 1979 to December 2002 was analyzed. 1,868 (55.6%) hernias occurred on right side, 1,190 (35.4%) on left side, and 300 (8.9%) were bilateral. 2,702 children were boys and 656 were girls (M:F=4.1:1). 170 children of 3,058 children with unilateral hernias (5.6%) developed contralateral inguinal hernia at 1 day to 95 months after herniotomy. 146 children were boys and 24 were girls (M:F=6.1:1). The patients who had had herniotomy before 1 year of age developed contralateral hernia in 17.4%, compared with 5.6% overall average. The earlier the first herniotomy was performed, the more frequently contralateral hernia developed. The occurrence of contralateral inguinal hernia was more frequent in boys (146 of 2,460, 5.9%) than girls (24 of 598, 4.0%) and more frequent after left herniotomy (80 of 1,190, 6.7%) than after right herniotomy (90 of 1,868, 4.8%), but statistically not significant. 52.9% of contralateral inguinal hernia developed within 1 year after hernia repair, and 87.6% developed within 3 years. Routine exploration on contralateral side in children is not necessary.

Carcinoid Tumor of the Common Bile Duct and Gallbladder

Carcinoid tumors are derived from Kultschitzky cells, and often found in the appendix, ileum, rectum and bronchus, but those of the biliary tract are rare. Here, the case of a 59-year-old man, unexpectedly found to have a carcinoid tumor obstructing the common bile duct, is reported. A gallbladder carcinoid tumor was also found incidentally during a planned subtotal gastrectomy operation. The literature on carcinoid tumors of the extrahepatic bile duct and gall bladder is also reviewed.

The Rupture of Distal Superficial Femoral Arterial Aneurysm on Rheumatoid Arthritis Patient

Isolated true atherosclerotic aneurysm of the distal superficial femoral artery is rare disease. We experienced a case of distal superficial femoral artery aneurysm not associated with dilatation of the common femoral or popliteal artery. True arterial aneurysms are attributed to the weakening of the arterial wall due to atherosclerosis. True superficial femoral artery aneurysms were predominantly founded in elderly men and used to be diagnosed by ultrasonography, CT or angiogram. We report a case of distal superficial femoral arterial aneurysmal rupture in elderly woman who has a rheumatoid athritis and diagnosed by ultrasonography. We performed aneurysmal excision and interposition graft with PTFE.