Sumio Kohno

Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults

The aim of this study was to review the cases 200 children and 40 adults who had cyst excision combined with hepaticoenterostomy (CEHE) for choledochal cyst, with particular emphasis on post-CEHE complications and their surgical management. Patients who had CEHE at the age of 15 years or less were defined as children, and those aged 16 years or older were defined as adults. The mean age when patients became initially symptomatic was 3 years in children and 26 years in adults. Eleven adults became symptomatic as children (< or = 15 years of age). The mean age of CEHE in children and adults was 4.2 years and 35 years, respectively. The time interval between the onset of initial symptoms and CEHE was significantly less in children than in adults (P < .0001). Of the 200 children, 176 had primary CEHE, and 24 had secondary CEHE converted from cystoenterostomy or other biliary surgery. Seventy children had intraoperative cyst endoscopy, which enabled us to examine the proximal intrahepatic bile ducts for stenosis and debris, and to wash out debris, protein plugs, and stones from the intrapancreatic ducts. Of the 40 adults, 22 had primary CEHE, 18 had secondary CEHE. The mean follow-up period was 10.9 years in children and 10.7 years in adults. The number of patients with post-CEHE complications in children and adults was 18 (9.0%) and 17 (42.5%), respectively. The post-CEHE complication rate in children was significantly lower than in adults (P < .0001). The 18 children had 25 post-CEHE complications such as cholangitis, intrahepatic bile duct stones, pancreatitis, stone formation in the intrapancreatic terminal choledochus or pancreatic duct, and bowel obstruction. Twenty-seven post-CEHE complications developed in the 17 adults including 2 cases of cholangiocarcinoma. There were no post-CEHE complications in the 70 children who had intraoperative cyst endoscopy. No stone formation was seen in the 145 children who had CEHE at the age of 5 years or less. Eight stone formations were seen in seven (12.7%) of the remaining 55 children aged over 5 years. Stones developed in seven (17.5%) adults. The incidence of post-CEHE stone formation in children aged 5 years or less was significantly lower than in other children and adults (P < .0001). Reoperation was required in 15 children: revision of hepaticoenterostomy in 4, percutaneous transhepatic cholangioscopic lithotomy (PTCSL) in 1, excision of intrapancreatic terminal choledochus in 2, endoscopic sphincterotomy of the papilla of Vater in 1, pancreaticojejunostomy in 1, and laparotomy for bowel obstruction in 6. Ten adults required reoperations: revision of hepaticoenterostomy in 2, PTCSL in 2, left hepatic lobectomy in 1, endoscopic sphincterotomy in 2, exploratory laparotomy in 2, and adhesiolysis in 1. The authors conclude that early diagnosis followed by CEHE is the treatment of choice for choledochal cyst, and intraoperative cyst endoscopy is recommended as a valuable adjunct to CEHE.

Choledochal cysts: Special emphasis on the usefulness of intraoperative endoscopy

Long-term complications after excisional surgery for choledochal cyst include stone formation within the intrahepatic bile duct (IHBD) and within the intrapancreatic portion of the bile duct (IPBD). The authors reviewed 180 patients with choledochal cysts (137 women) seen during a 30-year period and report their recent experience with intraoperative cyst endoscopy. Primary cyst excision with hepaticoenterostomy was performed in 150 cases, and a secondary cyst excision, after various lesser drainage procedures, in 24. Six children were treated early in the series with a cyst enterostomy. The mean age and follow-up period were 4.3 and 9.4 years, respectively. At surgery, dilatation of the IHBD was identified in 96 patients, and protein plugs or stones were observed in 31 common pancreaticobiliary channels. Fifty-five common channels were noted to be dilated. Hepaticojejunostomy was performed in 123 patients without intraoperative endoscopy. Late stone formation occurred within the IHBD in 4 of these cases and within residual segments of intrapancreatic cyst in 2 (mean age and follow-up period were 4.9 and 13.0 years, respectively). Intraoperative endoscopy was performed in 51 patients (mean age and follow-up period 3.6 and 4.3 years), and stone formation has not been observed in any patients in this group. Intraoperative cyst endoscopy is recommended as a valuable adjunct to choledochal cyst excision.

Small bowel pseudo-obstruction in Kawasaki disease

Between June 1977 and May 1986, 310 patients with Kawasaki disease were admitted to Shizuoka Childrens Hospital. Among these patients, seven showed the symptoms of intestinal paralysis during the acute stage. We present three of these cases who showed typical intestinal pseudoobstruction radiologically, and discuss the treatment of this type of intestinal involvement.

Usefulness of echocardiographic measurement of bilateral pulmonary artery dimensions in congenital diaphragmatic hernia

In nine patients with left-sided congenital diaphragmatic hernia (CDH) developing within 24 hours of birth, the authors measured the dimensions of the bilateral main pulmonary arteries by echocardiography, and investigated whether the left:right main pulmonary artery dimension ratio (PAD ratio) was a useful index for predicting pulmonary hypoplasia and persistent fetal circulation (PFC). Echocardiography was performed shortly after admission, the PAD ratio was calculated, and the clinical course of each patient was determined. When the PAD ratio was approximately 1.0, patients did not suffer from PFC and had little evidence of pulmonary hypoplasia. When the PAD ratio was low, the patients suffered from PFC and had pulmonary hypoplasia on the side of the hernia. Thus, the PAD ratio measured by echocardiography appears to be a useful index for predicting pulmonary hypoplasia and the risk of PFC in patients with CDH.

Congenital microgastria in an infant with the VACTERL association

A patient with microgastria and the VACTERL association is presented. The association of widespread anomalies with microgastria in our patient strongly suggests that such anomalies may all result from mesodermal deficiencies caused by defective migration at the primitive streak stage during the 3rd to 4th week of embryogenesis.

Pseudomembranous Enterocolitis and Hemorrhagic Necrotizing Enterocolitis in Hirschsprung's Disease

From 1977 to 1991, we encountered 67 patients with Hirschsprungs disease and 14 of them developed enterocolitis, with 3 cases being fatal. Enterocolitis occurred preoperatively in 12 infants, as well as after ileostomy in one and after a pull-through procedure in another. Seven infants had severe enterocolitis, including three with pseudomembranous enterocolitis and four with hemorrhagic necrotizing enterocolitis. Enterocolitis in Hirschsprungs disease mainly occurs due to intestinal obstruction and ischemia; however, in some cases, Clostridium difficile overgrowth and its toxin also appears to be related to severe pseudomembranous enterocolitis. In severe enterocolitis, antibiotics and enterostomy often prove to be ineffective, and thus an early resection of the affected bowel appears to be necessary. Moreover, when the aganglionic segment extends to the small bowel, severe enterocolitis tends to occur in the aganglionic intestine even after performing an enterostomy, and a resection of the aganglionic bowel is therefore recommended to allow for adequate lavage of the segment distal to the enterostomy site.

Treatment of laryngotracheoesophageal cleft

Four patients with laryngotracheoesophageal cleft were treated in our institution. Two of the patients survived and two died. Cleft levels ranged from partial 15 mm laryngotracheoesophageal to total with extended right bronchoesophageal cleft.

A new type of laryngotracheoesophageal cleft with extended bronchoesophageal cleft

A case of total laryngotracheoesophageal cleft with extended fronchoesophageal cleft is reported. The baby had a long cleft not only on the whole length of the laryngotracheoesophagus but it also extended to the right lower bronchus and esophagus. It was associated with right upper bronchial stenosis, sequestration of right lower lobe, and a complex cardiovascular anomaly. There have been no reports of this extended type of cleft in the 59 reported cases.

Ureteritis and pyelitis emphysematosa in a neonate

We describe a case of ureteritis and pyelitis emphysematosa in a neonate. The radiographic features were bilateral linear gas shadows of the ureters joining with gas outlining the renal pelves and calyces.

Infantile hemangioendothelioma of the thymus with massive pleural effusion and Kasabach‐Merritt syndrome: Histopathological, flow cytometrical analysis of the tumor

Infantile hemangioendothelioma of the thymus is a rare disease. We describe a patient who developed a large anterior mediastinal mass, severe thrombocytopenia and massive pleural effusion at 1 month of age. Glucocorticosteroid and irradiation therapy had no effect on either the tumor size or clinical symptoms and the tumor was resected subtotally. Three months after the subtotal resection, the remaining tumor had almost disappeared and the symptoms had resolved. The patient has now been well for 1 year after surgery without evidence of recurrence. The tumor tissue was characterized by prominent vascular endothelial proliferation intermixed with a normal thymic structure, producing a picture consistent with that of an infantile hemangioendothelioma in the thymus, lmmunohistochemically, the tumor cells showed positive staining for vimentin, factor VIII and CD34. The DNA stemline and proliferative activity were examined by flow cytometry, which revealed a diploid stemline with a low growth fraction. DNA content and cell cycle analyses of the tumor tissue may be useful for predicting the biological behavior of the tumor.