Does Congenital Biliary Dilatation Todani Type II (Diverticular Type) Really Exist?

AIM OF THE STUDY: The aim of the study is to clarify the clinicopathological and biliary morphological characteristics in reported cases of diverticular congenital biliary dilatation (CBD). METHOD: Using PubMed and the Japan Medical Abstracts Society, articles on possible diverticular CBD were extracted and the clinical pictures examined. We also sought evidence for definitions of diverticular CBD and the associated condition of pancreaticobiliary maljunction (PBM) using the original articles by Alonso-Lej and Todani. The characteristic biliary morphologies of cases with images were also investigated. RESULTS: Analyses of 211 possible cases superficially demonstrated multiple diverticula in 12 (12%) and single diverticulum in 89 (88%), with diverticula located in the upper (n = 38, 38%), middle (n = 32, 32%), or lower (n = 26, 26%) biliary tract in and presence of intra-diverticular stones, PBM, and biliary carcinoma in 23% (n = 18), 39% (n = 25), and 11% (n = 14), respectively. However, evidence defining diverticular CBD or justifying the lack of associated PBM was not demonstrated even in the original articles. Scrutiny of the biliary anatomy in 59 cases with images showed incorrect inclusions of types I or IV-A with an irregular biliary duct wall or dilated cystic duct, periampullary choledochal diverticula, or even solitary biliary cysts. Authentic diverticular CBD, representing the diverticulum connected to the middle of the common bile duct via a thin, patent stalk was seen in only 6 cases. CONCLUSION: Real diverticular CBD appears extremely rare. The lack of an objective definition allows wide interpretations of clinical pictures, creating inconsistencies in the diagnosis and treatment of CBD and raising questions regarding the utility of conventional classifications. LEVEL OF EVIDENCE: Level III.

Cyst Size in Fetuses with Biliary Cystic Malformation: An Exploration of the Etiology of Congenital Biliary Dilatation.

PURPOSE: Our aim was the longitudinal assessment of cyst size in fetuses with biliary cystic malformation (BCM) to explore its etiology and the possibility of antenatal differentiation between biliary atresia (BA) and congenital biliary dilatation (CBD). METHODS: We conducted a retrospective review of all patients diagnosed antenatally with BCM from 1994 to 2014 at our institutions. RESULTS: The study cohort comprised of three patients with BA and six with CBD. There were no significant differences in the gestational age and cyst size at the first detection of BCM between the two groups. In fetuses with CBD, the cyst size steadily increased as the gestational age advanced, while it fluctuated around 1.5 cm and remained below 2.1 cm in those with BA. However, the ratio of cystic area to fetal trunk area was approximately constant due to linear fetal growth in fetuses with CBD. CONCLUSION: Fetuses with BCM <2.1 cm in the late gestation period were more likely to have BA than CBD. Our observation of cyst enlargement with advancing gestational age in the CBD group was attributed solely to fetal growth. Biliary dilatation in fetuses with CBD and BA might be completed at the onset of BCM.

Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease, 2017.

BACKGROUND: Despite the presence of ganglion cells in the rectum, some patients have symptoms similar to those of Hirschsprung's disease. A consensus has yet to be established regarding the terminology for these diseases. We defined this group of diseases as "allied disorders of Hirschsprung's disease" and compiled these guidelines to facilitate accurate clinician diagnosis and provide appropriate treatment strategies for each disease. METHODS: These guidelines were developed using the methodologies in the Medical Information Network Distribution System (MINDS). Of seven allied disorders, isolated hypoganglionosis; megacystis-microcolon-intestinal hypoperistalsis syndrome; and chronic idiopathic intestinal pseudo-obstruction were selected as targets of clinical questions (CQ). In a comprehensive search of the Japanese- and English-language articles in PubMed and Ichu-Shi Web, 836 pieces of evidence related to the CQ were extracted from 288 articles; these pieces of evidence were summarized in an evidence table. RESULTS: We herein outline the newly established Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease. Given that the target diseases are rare and intractable, most evidence was drawn from case reports and case series. In the CQ, the diagnosis, medication, nutritional support, surgical therapy, and prognosis for each disease are given. We emphasize the importance of full-thickness intestinal biopsy specimens for the histopathological evaluation of enteric ganglia. Considering the practicality of the guidelines, the recommendations for each CQ were created with protracted discussions among specialists. CONCLUSIONS: Clinical practice recommendations for allied disorders of Hirschprung's disease are given for each CQ, along with an assessment of the current evidence. We hope that the information will be helpful in daily practice and future studies.

Classification of pancreaticobiliary maljunction and clinical features in children.

BACKGROUND: 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. METHODS: The study group of 317 children with PBM was divided into the four types of PBM. Clinical features, preoperative complications, operations, and postoperative pancreatic complications were evaluated. RESULTS: All patients underwent excision of the extrahepatic bile duct. In type A, the age was younger and there was a higher incidence of cystic dilatation. Non-dilatation of the common bile duct was frequently seen in type B. Abdominal pain with hyperamylasemia was frequently seen in types B and C. In particular, the incidence of protein plugs and biliary perforation was high in type C (56.1% and 14.3%, respectively). The overall incidence of acute pancreatitis was 7.3%. Pancreatitis after excisional surgery was rare in the children in this study. Two patients with type D (0.6%) developed chronic pancreatitis postoperatively. CONCLUSIONS: This proposed classification is simple and correlates well with clinical features.

Duodenogastric regurgitation in hepaticoduodenostomy after excision of congenital biliary dilatation (choledochal cyst).

PURPOSE: We examined the clinical significance of duodenogastric regurgitation (DGR) as a late complication in the long-term follow-up after hepaticoduodenostomy (HD) as a reconstruction surgery for congenital biliary dilatation (CBD). METHODS: Seventeen patients with CBD were retrospectively analyzed for late complications (mean follow-up, 16.8 years). All patients had undergone total resection of the extrahepatic bile duct followed by HD. DGR was identified using endoscopic examination, intraluminal bile monitoring, and liver scanning. RESULTS: DGR was found in all 17 patients by endoscopic examination and intraluminal bile monitoring. Fourteen of the 17 (82.4%) patients with DGR had experienced abdominal symptoms since a mean of 6.9 years postoperatively. Liver scanning also revealed apparent DGR in all 14 symptomatic patients. We converted 7 of the 14 patients to hepaticojejunostomy reconstruction at a mean of 13.0 years after the initial excisional surgery. Their symptoms were completely relieved postoperatively. CONCLUSIONS: DGR is an important complication after HD. Examination of patients for the development of DGR is an essential part of long-term follow-up in patients with CBD who have undergone HD as a reconstruction surgery. Conversion surgery is recommended in patients with DGR accompanied by long-term abdominal symptoms. LEVELS OF EVIDENCE: Level IV.

Japanese clinical practice guidelines for congenital biliary dilatation.

Until now, there have been no practical clinical guidelines for congenital biliary dilatation (CBD). In this review article, the Japanese Study Group on Congenital Biliary Dilatation (JSCBD) propose to establish clinical practice guidelines for CBD. Because the evidence-based literature is relatively small, we decided to create guidelines based on the consensus of experts, using the medical literature for reference. A total of 20 clinical questions (CQs) were considered by the members of the editorial committee responsible for the guidelines. The CQs included the distinct aspects of CBD: (1) Concepts and Pathology (three CQs); (2) Diagnosis (six CQs); (3) Pancreaticobiliary Complications (three CQs); Treatments and Prognosis (eight CQs). Each statements and comments for CQs were made by the guidelines committee members. CQs were finally approved after review by members of the editorial committee and the guidelines evaluation board of CBD. These guidelines were created to provide assistance in the clinical practice of CBD management; their contents focus on clinical utility, and they include general information on CBD to make this disease more widely recognized.

Neonatal gastrointestinal perforation in Japan: a nationwide survey.

PURPOSE: The purpose of this study is to identify the current clinical features of neonatal gastrointestinal perforation in Japan. METHODS: A questionnaire about cases of neonatal gastrointestinal perforation treated in recent 5 years was sent to participating institutions of the Japanese Society of Pediatric Surgeons (JSPS). RESULTS: Five hundred and thirty-six neonates with gastrointestinal perforation were treated. They consisted of 42 patients with gastric rupture/perforation (GR), 33 patients with intestinal atresia/stenosis (IA), 3 patients with malrotation (ML), 118 patients with necrotizing enterocolitis (NEC), 160 patients with focal intestinal perforation (FIP), 46 patients with meconium-related ileus (MRI), 77 patients with meconium peritonitis (MP), and 57 patients with other conditions. The total mortality rate was 20.5 %. The mortality rates of the patients with GR, IA, ML, NEC, FIP, MRI, and MP were 9.5, 9.1, 0, 33.1, 20.6, 28.2, and 9.1 %, respectively. In 263 cases involving extremely low-birth-weight neonates (ELBW), 108 died (mortality rate 41.1 %). The mortality rates for ELBW with GR, NEC, FIP, MRI, MP, and other conditions were 27.3 % (3/11), 58.5 % (48/82), 21.6 % (24/111), 70.6 % (24/34), 57.1 % (4/7), and 27.8 % (5/18), respectively. CONCLUSIONS: The mortality rates for ELBW decreased from 62.8 % in the previous survey to 41.1 % by the time of this survey.

Diagnostic criteria for congenital biliary dilatation 2015.

BACKGROUND: 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. METHODS: The committee of JSGPM prepared the diagnostic criteria for congenital biliary dilatation in 2014, and a final revised version was approved in 2015. RESULTS: Congenital biliary dilatation is defined as a congenital malformation involving both local dilatation of the extrahepatic bile duct, including the common bile duct, and pancreaticobiliary maljunction. However, cases associated with intrahepatic bile duct dilatation can also be included. Various kinds of pathological conditions can occur on hepatobiliary systems and pancreas by bile duct dilatation and pancreaticobiliary maljunction. For a diagnosis of congenital biliary dilatation, both abnormal dilatation of the bile duct and pancreaticobiliary maljunction must be evident by either imaging test or anatomical examination. Acquired or secondary dilatation of the bile duct by obstruction due to biliary stones or malignancy should be strictly excluded. CONCLUSION: Diagnostic criteria for congenital biliary dilatation 2015 were established from Japan representing a world first.

Segmental dilatation of the intestine: results of a nationwide survey in Japan.

PURPOSE: To clarify the status of segmental dilatation of the intestine (SD) using a nationwide retrospective cohort survey. METHODS: Supported by the Ministry of Health, Labour and Welfare in Japan, preliminary questionnaires requesting the numbers of SD cases from 2000 to 2009 were sent to 161 Japanese major institutes of pediatric surgery and gastroenterology. We present the results of our analysis of the data from secondary questionnaires. RESULTS: Twenty-eight cases of SD were reported, including 19 males and 9 females. The average gestational age was 30.2 weeks and the average birth weight was 2.319 g. The period of onset was neonatal in 18 cases and infancy in 6 cases, including 7 cases diagnosed prenatally. The dilated segment was ileum in 14 cases and colon in 10 cases, and symptoms included abdominal distention, vomiting, and chronic constipation. Concurrent intestinal malformation such as malrotation, intestinal atresia, and anal atresia were present in 4 cases and 27 of 28 cases were successfully treated by surgical resection. Histologically, abnormal muscle layers were found in three cases and ectopic tissue in two cases. CONCLUSION: We determined the clinical features of SD based on data from 28 cases occurring over 10 years.

Pancreatobiliary reflux in individuals with a normal pancreaticobiliary junction: a prospective multicenter study.

BACKGROUND: Pancreatobiliary reflux (PBR) can occur in individuals without pancreaticobiliary maljunction. The aim of this study was to elucidate the incidence of PBR in individuals with a normal pancreaticobiliary junction and its impact on the biliary tree. METHODS: Data were prospectively collected from 15 centers on 420 patients with a morphologically normal pancreaticobiliary junction who had undergone bile sampling and measurement of the biliary amylase level. We investigated the incidence and predictive factors for high biliary amylase levels (HBAL), as well as the relationship of HBAL with biliary malignancy. RESULTS: Twenty-three patients (5.5%) showed HBAL (≥10,000 IU/L). Univariate analysis showed that risk factors for the elevation of biliary amylase levels were the existence of a relatively long common channel (≥5 mm), acute pancreatitis, and papillitis. Multivariate analysis revealed that only the existence of a relatively long common channel was a significant factor for PBR. Biliary amylase levels in patients with a relatively long common channel were significantly higher than in patients without a long common channel (12,333 vs. 2,070 IU/L, P = 0.001). The incidence of HBAL (P < 0.001), as well as the overall biliary amylase levels (P = 0.007) were significantly higher in patients with gallbladder cancer than in those without gallbladder cancer. CONCLUSIONS: The PBR was frequently observed in individuals with a relatively long common channel. Patients showing HBAL with normal pancreaticobiliary junction are at high risk for gallbladder cancer.

Recent advances and problems in the management of pancreaticobiliary maljunction: feedback from the guidelines committee.

Clinical practice guidelines on how to deal with pancreaticobiliary maljunction (PBM) were made in Japan in 2012, representing a world first. Using a narrow definition, congenital biliary dilatation involves only Todani type I (except type Ib) and type IV-A, both of which are accompanied by PBM in almost all cases. Prospective ultrasonographic study revealed that the maximum diameter of the common bile duct increased with age. Pathophysiological conditions due to pancreatobiliary reflux occur in patients with high confluence of the pancreaticobiliary ducts, a common channel ≥ 6 mm long and occlusion of communication during contraction of the sphincter of Oddi. Since PBM can be diagnosed by magnetic resonance cholangiopancreatography, multi-planar reconstruction multi-detector row computed tomography and endoscopic ultrasonography, the current diagnostic criteria should be revised to take these diagnostic imaging modalities into consideration. According to a nationwide survey, biliary cancer occurred in 21.6% of adult patients with PBM with biliary dilatation and 42.2% of patients with PBM without biliary dilatation. In biliary cancer associated with PBM without biliary dilatation, 88.1% were gallbladder cancer. Treatment for PBM with biliary dilatation is prophylactic flow-diversion surgery, but further investigations and surveillance studies are needed to clarify the appropriate surgical strategy for PBM without biliary dilatation.

Temporary umbilical loop colostomy for anorectal malformations.

Transumbilical surgical procedures have been reported to be a feasible, safe, and cosmetically excellent procedure for various pediatric surgical diseases. Umbilical loop colostomies have previously been created in patients with Hirschsprung's disease, but not in patients with anorectal malformations (ARMs). We assessed the feasibility and cosmetic results of temporal umbilical loop colostomy (TULC) in patients with ARMs. A circumferential skin incision was made at the base of the umbilical cord under general anesthesia. The skin, subcutaneous tissue, and fascia were cored out vertically, and the umbilical vessels and urachal remnant were individually ligated apart from the opening in the fascia. A loop colostomy was created in double-barreled fashion with a high chimney more than 2 cm above the level of the skin. The final size of the opening in the skin and fascia was modified according to the size of the bowel. The bowel wall was fixed separately to the peritoneum and fascia with interrupted 5-0 absorbable sutures. The bowel was opened longitudinally and everted without suturing to the skin. The loop was divided 7 days postoperatively, and diversion of the oral bowel was completed. The colostomy was closed 2-3 months after posterior saggital anorectoplasty through a peristomal skin incision followed by end-to-end anastomosis. Final wound closure was performed in a semi-opened fashion to create a deep umbilicus. TULCs were successfully created in seven infants with rectourethral bulbar fistula or rectovestibular fistula. Postoperative complications included mucosal prolapse in one case. No wound infection or spontaneous umbilical ring narrowing was observed. Skin problems were minimal, and stoma care could easily be performed by attaching stoma bag. Healing of umbilical wounds after TULC closure was excellent. The umbilicus may be an alternative stoma site for temporary loop colostomy in infants with intermediate-type anorectal malformations, who undergo radical anorectoplasty via a non-abdominal approach.

Simultaneous development of adenocarcinoma and gastrointestinal stromal tumor (GIST) in the stomach: case report.

BACKGROUND: Gastrointestinal stromal tumors (GISTs) and adenocarcinoma are distinct neoplasms originating from different cell layers. Approximately 20% of patients with GIST develop other cancers. CASE PRESENTATION: We report a case of the coexistence of adenocarcinoma and gastrointestinal stromal tumor (GIST). Gastric endoscopy showed the ulcerated tumor with bleeding along the lesser curvature of the proximal stomach and a submucosal nodule that measured about 3 cm in diameter in the lower part of the stomach body. Their pathological examination showed gastric cancer (poorly differentiated diffuse adenocarcinoma) and GIST (low-risk category). Further, immunohistochemical staining for C-kit and CD34 was positive, while that for SMA and S-100 was negative. CONCLUSION: Although it is not easy to speculate on the coexistence of adenocarcinoma and GIST, pre-and post-operative diagnoses may be essential, and such cancer development is not considered to be unusual.

[Delayed hepatic resection for synchronous liver metastases from colorectal cancer].

BACKGROUND: This study aimed to compare the surgical outcome and long-term survival between simultaneous and delayed resection of liver metastases from colorectal cancer (LM). METHODS: Seventy-four patients with LM were divided into two groups, i. e., 32 patients who underwent hepatectomy at the time of colorectal surgery (simultaneous group) and 35 patients who underwent delayed liver resection (delayed group). RESULTS: In 12 of the 35 (34%) patients from the delayed group, new metastatic lesions were found in the same and/or different segments after re-evaluation during the interval between operations. These patients had a shorter interval between procedures, and larger tumors than patients without tumor progression. There were significant differences (p=0.0454 and 0.0077) of hepatic disease-free survival between the metachronous or delayed groups and the simultaneous group. Multivariate analysis showed that simultaneous resection was one of three independent prognostic indicators with an influence on hepatic disease-free survival. CONCLUSIONS: Tumor progression could be recognized and occult metastases were detected during the interval between operations. Delayed resection of synchronous LM may be useful to reduce the risk of rapid recurrence in the remnant liver.

Timing of resection for synchronous liver metastases from colorectal cancer.

BACKGROUND: This study aimed to compare the surgical outcome and long-term survival between simultaneous and delayed resection of liver metastases from colorectal cancer (LM), and to identify the factors influencing hepatic disease-free survival in patients with synchronous LM. METHODS: Seventy-four patients with LM were divided into two groups, i.e., 32 patients who underwent hepatectomy at the time of colorectal surgery (simultaneous group) and 42 patients who underwent delayed liver resection (delayed group). RESULTS: The hepatic disease-free survival rates of patients from the delayed group with either ≥ 3 or <3 liver metastases were significantly better than that of the simultaneous group. Multivariate analysis showed that simultaneous resection was one of three independent prognostic indicators with an influence on hepatic disease-free survival. In 13 of the 42 (31%) patients from the delayed group, new metastatic lesions were found in the same and/or different segments after re-evaluation during the interval between operations. These patients had a higher incidence of poorly differentiated or mucinous adenocarcinoma, shorter interval between procedures, and larger tumors than patients without tumor progression. CONCLUSIONS: Tumor progression could be recognized and occult metastases were detected during the interval between operations. Delayed resection of synchronous LM may be useful to reduce the risk of rapid recurrence in the remnant liver. Patients with poorly differentiated or mucinous adenocarcinoma and those with larger tumors who undergo delayed liver resection should receive neoadjuvant chemotherapy during the interval between operations.

Metastatic gastric tumor from renal cell carcinoma.

Metastatic tumors of the stomach are rare, with an incidence of 0.2%-0.7%, and they have been reported to result mainly from primary breast cancers, lung cancers, and melanoma. Further, among such metastatic tumors, the metastasis of renal cell carcinoma (RCC) to the stomach is an extremely rare disease, and it is usually reported in autopsy series. We report a rare case of metastatic gastric tumor derived from right renal carcinoma. Gastric endoscopy confirmed a large, polypoid, friable mass (type 1 tumor, about 7 cm in diameter) in the middle part of the stomach body. The mass was surgically excised and pathological examination showed that the gastric tumor was derived from a metastasis from the right kidney, because it was composed of malignant cells that were identical to those from the removed RCC. In addition, the tumor cells were immunoreactive for CD10, CD15, Ecadherin, early membrane antigen (EMA), and vimentin, but no reactivity was observed for cytokeratins 7 and 20 or c-KIT. Although gastric metastatic tumor derived from renal carcinoma is rare, the precise pre- and postoperative diagnosis may be important; thus, investigation for such metastatic tumors should be performed routinely in the follow up of patients who have been treated for RCC.

Choledochal cyst associated with duodenal atresia: case report and review of the literature.

We report a rare case of choledochal cyst (CC) associated with congenital duodenal atresia (DA) and annular pancreas (AP). A girl was born at 37 weeks of gestation weighing 2,974 g with a prenatal diagnosis of DA. She underwent a duodenoduodenostomy for type III DA with an AP 1 day after birth. At 4 years of age, she was admitted for evaluation of cholangitis and pancreatitis. Radiological studies demonstrated a fusiform-type CC with pancreaticobiliary maljunction (PBMJ). Excision of the CC and hepaticojejunostomy were performed. The patient was discharged without complications. Despite the fact that CC, DA, and AP are embryologically closely related entities, to the best of our knowledge, only eight such cases have been documented. We must be aware of the possible combination of CC in the follow-up of the patients with DA associated with AP.

Pentalogy of Cantrell with a double-outlet right ventricle: 3.5-year follow-up in a prenatally diagnosed patient.

Pentalogy of Cantrell is a rare congenital defect associated with five ventral midline anomalies and high mortality. An obstetric sonogram revealed a fetus with a body wall defect suggesting a diagnosis of this condition. Soon after birth, the infant underwent a closure of the upper abdominal wall defect followed by the successful repair of double-outlet right ventricle and pulmonary valve stenosis at the age of 5 months. The patient is currently alive and well 3.5 years after surgery. It is concluded that a deliberate therapeutic strategy based on the intrauterine diagnosis may alter the natural history of this devastating disorder.

A case of splenosis after laparoscopic splenectomy.

Splenosis is the heterotopic autotransplantation of splenic tissue usually following traumatic rupture of the spleen or surgical manipulation of splenic tissue. Although it is generally asymptomatic, it may present with abdominal pain or mass. Furthermore, there may be recurrence of the hematological disorders for which the patient underwent splenectomy. We report a rare case of splenosis in a 9-year-old girl after laparoscopic splenectomy for hereditary spherocytosis, which required laparotomy due to bowel obstruction.

Cecal volvulus in children with mental disability.

Two cases of cecal volvulus in children with mental disability are described. Case 1: a 3-year-old girl with trisomy 18 was admitted with abdominal pain and vomiting. She had received left lateral segmentectomy 6 months earlier because of hepatoblastoma. Release of the cecal volvulus followed by the fixation of the cecum and ascending colon to the right retroperitonium was performed. Case 2: a 15-month-old boy with Cornelia de Lange syndrome who had undergone a standard Nissen's fundoplication. On the sixth postoperative day, progressive abdominal distention developed. Abdominal color Doppler ultrasonography from the right rear side revealed a clockwise-twisted ileocecal artery and vein and a dilated colon with tapering configuration. On the ninth postoperative day, emergent release of the cecal volvulus followed by ascending colostomy through a perforation site was performed. Approximately 40 children with cecal volvulus have so far been reported, of whom 13 are mentally disabled. We speculated that in the cases reported here, distention of the intestine accompanying the mental disability and the previous surgery contributed to the development of cecal volvulus in addition to the prerequisite of abnormal mobility of the cecum. Pediatric surgeons should consider the cecal volvulus as a cause of intestinal obstruction in mentally disabled children.