Yoshiki Morotomi

Biliary complications after excisional procedure for choledochal cyst

During the last 25 years, from 1969 to 1994, the authors treated 97 choledochal cysts by surgical excision. Biliary reconstruction consisted of 67 hepaticoduodenostomies and 30 hepaticojejunostomies. The common hepatic duct was the site of anastomosis in 9 of the duodenostomies and 13 of the jejunostomies and of the bifurcation of the hepatic ducts in 58 duodenostomies and 17 jejunostomies. Reoperation was required in 10 cases because of recurrent cholangitis with intrahepatic gallstones. Biliary strictures were responsible for the cholangitis in 9 children with anastomoses at the level of the common hepatic duct and in 1 with an anastomosis at the level of the bifurcation. These results suggest that biliary complications develop because of anastomotic stricture or primary ductal stricture, and may be minimised by the creation of a wide anastomosis, which is best accomplished at the hepatic hilum.

Choledochal cyst, pancreatobiliary malunion, and cancer

A review of the clinical features of biliary cancer with choledochal cyst and pancreatobiliary malunion is presented, together with a recent case report. Biliary cancer develops in about 25% of patients with choledochal cyst and pancreatobiliary malunion, and usually occurs in younger patients (usually those in their 40s) than does biliary carcinoma in the general population. The risk of malignancy in the retained cyst with internal drainage is higher than that in the primary cyst. Early excision of the retained cyst should be performed as quickly as possible, even if the patient is symptomless. Some bile acid fractions and refluxed pancreatic enzymes in bile are possibly responsible for carcinogenesis. Carcinoma generally develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic bile duct. In cystic dilatation, cancer usually occurs in the common bile duct, while in diffuse or non-dilated type, it develops in the gallbladder. Multicentric carcinomas develop in the bile duct either synchronously or metachronously. The prognosis of biliary cancer is usually dismal. However, aggressive procedures are now gaining better results than conventional approaches. The procedure of choice for choledochal cyst or malunion is to prevent the development of cancer by performing an early excision. Removal of the entire extrahepatic bile duct is necessary, even in patients with malunion and no biliary dilatation. Carcinoma rarely arises in the intrahepatic bile duct after excisional surgery, probably due to the long-standing stricture of the bile duct. Capacious anastomosis and/or ductoplasty is essential. Carcinoma may also develop in the remnant bile duct. Excision of the distal duct extending into the pancreas is also necessary.

Peritoneal Lavage Versus Drainage for Perforated Appendicitis in Children

A total of 231 children with acute appendicitis were treated at our hospitals during the 10 years between 1984 and 1993, 53 of whom had a perforated appendix. These 53 patients were randomly assigned to two groups at the time of surgery according to the different procedures performed. Thus, 29 children were managed by appendectomy followed by peritoneal lavage using a large amount of saline, and intravenous antibiotic therapy consisting of aminoglycoside and cephem (lavage group), while the other 24 children were treated by appendectomy with silicon tube drainage and the same systemic antibiotic therapy (drainage group). The mean length of hospitalization, and the mean durations of fever and the need for fasting after laparotomy in the lavage group were significantly less than those in the drainage group: 10.1 versus 18.8 days, 2.8 versus 7.7 days, and 1.8 versus 3.5 days, respectively. The operation wounds healed well in the lavage group due to the fact that there was no drain. Wound infections occurred in two children from the lavage group and six from the drainage group. Intra-abdominal abscesses occurred in two children from the drainage group. Accordingly, peritoneal lavage appears to be superior to intraperitoneal tube drainage for the management of perforated appendicitis in children.

Indications for surgical repair of funnel chest based on indices of chest wall deformity and psychological state.

AbstractPurpose. We examined the surgical indications for funnel chest, taking psychological factors into consideration. Methods. We assessed 36 young people with funnel chest who were seen as outpatients, including 31 boys and 5 girls aged from 1 to 22 years old. Respondents were asked whether they suffered psychological distress, and if they wanted surgery. The severity of the deformity was evaluated using the Vertebral Index (VI) and the Frontosagittal Index (FSI) calculated from chest roentgenograms. Results. The VI in 11 patients without distress (23.7 ± 4.1) was lower than that in 25 patients with distress (32.8 ± 8.2), and the FSI in the patients without distress (33.5 ± 5.3) was higher than that in the patients with distress (23.6 ± 8.6). The VI in 19 patients who did not want surgery (26.9 ± 7.9) was lower than that in 17 patients who did (33.5 ± 7.5), and the FSI in the patients who did not want surgery (30.4 ± 8.1) was higher than that in the patients who did (22.4 ± 8.1). The distressed patients suffered many psychological problems, such as being the object of bullying. Conclusion. The severity of the deformity affected the patients psychological state. We consider that a VI ≫28 or an FSI ≪28 are indications for surgery, based on the mean VI + SD and the mean FSI-SD of patients not suffering distress.

Diagnostic criteria for congenital biliary dilatation 2015

The Diagnostic Criteria for Pancreaticobiliary Maljunction 2013 were published by the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) in 2014. The committee of JSGPM for diagnostic criteria for pancreaticobiliary maljunction has established the standard diameter of the bile duct, and a definition of dilatation of the bile duct was proposed in 2014.

A case of TUBA1A mutation presenting with lissencephaly and Hirschsprung disease

Gene mutation of tubulin alpha-1A (TUBA1A), a critical component of microtubules of the cytoskeleton, impairs neural migration and causes lissencephaly (LIS). The approximately 45 cases of disease-associated TUBA1A mutations reported to date demonstrate a wide spectrum of phenotypes. Here we describe an 8-year-old girl with lissencephaly, microcephaly, and early-onset epileptic seizures associated with a novel mutation in the TUBA1A gene. The patient developed Hirschsprung disease and the syndrome of inappropriate antidiuretic hormone secretion (SIADH), which had not previously been described in TUBA1A mutation-associated disease. Our case provides new insight into the wide spectrum of disease phenotypes associated with TUBA1A mutation.

Alveolar rhabdomyosarcoma of the lung in a child

The case of a 22-month-old boy with alveolar rhabdomyosarcoma of the lung is presented. Brain metastasis and recurrence of the right pulmonary hilum and parietal pleura developed 6, 11, and 24 months (respectively) after tumor resection. Chemotherapy and radiotherapy were effective. Neuron-specific enolase was very helpful in detecting metastasis and disease recurrence. Primary pulmonary rhabdomyosarcoma can be divided into two groups: tumor in the normal lung, and tumor in cystic lesions of the lung.

Exploring the length of the common channel of pancreaticobiliary maljunction on magnetic resonance cholangiopancreatography

In the revised diagnostic criteria for pancreaticobiliary maljunction (PBM), PBM can be diagnosed from a long common channel by magnetic resonance cholangiopancreatography (MRCP). However, it is necessary to differentiate from high confluence of pancreaticobiliary ducts (HCPBD) with a relatively long common channel (≥6 mm) and effect of the sphincter in the pancreaticobiliary junction. This multicenter study aimed to explore definite value of the length of an abnormally long common channel, which enables to distinguish PBM from HCPBD on MRCP.

Modified Kasai's procedure for a choledochal cyst with a very narrow hilar duct

Excision has been widely recognized as the treatment of choice for choledochal cysts. For biliary reconstruction after cyst excision, we have strongly recommended hepaticoenterostomy at the hepatic hilum with a wide anastomotic stoma to prevent postoperative cholangitis. However, we recently treated two infants in whom a wide anastomotic stoma could not be made due to a narrow hilar duct. Therefore, a hepatic portojejunostomy with Roux-en-Y anastomosis (Kasais procedure) was performed after cyst excision in both case to permit free drainage of bile. The mucosa of the hilar duct was everted and fixed to the liver parenchyma to prevent stricture formation. Both babies have done well since the surgery.

Classification of pancreaticobiliary maljunction and clinical features in children

In 2015, the Committee on Diagnostic Criteria of the Japanese Study Group on Pancreaticobiliary Maljunction (PBM) proposed a classification of PBM into four types: (A) stenotic type, (B) non‐stenotic type, (C) dilated channel type, and (D) complex type. To validate this classification and clarify the clinical features of the four types of PBM, a retrospective multicenter study was conducted.