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.

Efficacy of daikenchuto after laparoscopic surgery for perforated appendicitis.

BACKGROUND: Although many recent studies have scientifically verified the efficacy of the traditional herbal medicine daikenchuto (DKT) for postoperative gastrointestinal function, its efficacy has not been established in children. We retrospectively evaluated the effect of DKT in pediatric patients with panperitonitis associated with perforated appendicitis (PaPA) who underwent laparoscopic appendectomy. METHODS: Among 34 children with PaPA who underwent laparoscopic appendectomy from May 2012 to May 2021, 19 received DKT (group D) and 12 did not (group C). We compared postoperative gastrointestinal function, complications, and improvement in the inflammatory response between the two groups. RESULTS: Of the evaluation parameters for postoperative gastrointestinal function, the mean ± standard deviation time to first flatus was significantly shorter in group D than in group C (1.21 ± 0.42 and 2.17 ± 0.94 days respectively; p = 0.0005). The time to ingestion of half a meal was also significantly shorter in group D than in group C (8.42 ± 3.69 and 12.50 ± 4.96 meal occasions respectively; p = 0.01). There was no significant difference in complication rates between the two groups. CONCLUSION: Daikenchuto rapidly and safely improved postoperative gastrointestinal symptoms in children with PaPA. To the best of our knowledge, this is the first study to evaluate the effect of DKT on postoperative symptoms in laparoscopic appendectomy and in children.

Relationships Between Histopathological Findings in the Liver and Prognosis in Patients With Biliary Atresia.

Background: Biliary atresia (BA) is a progressive obstructive hepatic disease that requires early diagnosis and the prompt initiation of treatment. Although portoenterostomy (PES) is usually performed as the initial surgical procedure, the liver damage may subsequently progress, such that liver transplantation (LTx) may be required. In this study, we comprehensively evaluated the histopathology of liver samples collected during PES and retrospectively evaluated its relationship with prognosis. Methods: Forty-seven patients with BA who underwent PES between 2002 and 2021 were included. Their biopsy samples were semi-quantitatively graded according to the severity of liver fibrosis, bile duct proliferation, cholestasis, ductal plate malformation, and inflammatory cell infiltration; and the expression of cluster of differentiation (CD)3, CD20, human leukocyte antigen II-DR, and α-smooth muscle actin (α-SMA). The relationships of each with the prevalence of survival with native liver (SNL) were evaluated to identify prognostic markers. Results: The median postoperative duration of follow-up was 11.8 years (maximum, 18.0 years; minimum, 3.5 years). There were no deaths during this period, but LTx was performed in 31 patients and the final prevalence of SNL was 34.0% (16/47). There were negative correlations of liver fibrosis and α-SMA with SNL, and a positive correlation between CD20 and SNL. Multivariate analysis using a proportional hazards regression model showed that only CD20 expression was significant. Conclusions: Comprehensive histopathological analysis of liver biopsy samples obtained at the time of PES showed a positive correlation between CD20 expression and SNL, suggesting that this may represent a useful prognostic marker. Level of evidence: III.

Association between the confluent form of pancreatic and bile duct and histopathological findings in pancreaticobiliary maljunction: A case series study.

INTRODUCTION: Pancreaticobiliary maljunction (PBM) is a congenital anomaly wherein the persistent reflux of the pancreatic juice into the biliary tract induces biliary tract cancer. The prediction criteria for gallbladder carcinogenesis have been reported previously through results obtained from examining carcinogenic and non-carcinogenic cases with the parameters that classified each confluent form in PBM. This study aimed to validate these previous study results and provide new recommendations for gallbladder carcinogenesis prevention. METHODS: Twenty-four patients with PBM underwent hepaticojejunostomy. The prediction criteria for gallbladder carcinogenesis consist of three elements. The confluent forms that satisfied none or one of the three criteria were defined as a low score group, and those that satisfied two or three were defined as the high score group. Immunohistology and pathological search were performed on the gallbladders' sections in both groups to evaluate chronic inflammation. RESULTS: The cases with dysplasia, positive Ki67 labeling index, and gallbladder cancer were more common in the high score group and tended to have more lymphocyte infiltration. These findings indicate that the degree of inflammation and cell proliferation might be more severe in the high score group than in the low score group. CONCLUSIONS: There is a close relationship between the confluent form and the histopathological findings of the gallbladder in patients with PBM. The confluent forms observed in the high score group might have an additional correlation with increased proliferation activity and subsequent malignant transformation of the gallbladder epithelium.

Torsion of vermiform appendix: case report and review of the literature.

BACKGROUND: Torsion of the vermiform appendix is a rare disease with symptoms very similar to those of acute appendicitis. We herein report a case of torsion of the vermiform appendix diagnosed by intraoperative findings. CASE PRESENTATION: A 4-year-old boy presented to our hospital because of abdominal pain and vomiting. Laboratory data revealed a C-reactive protein level of 0.08 mg/dL and white blood cell count of 19,300/µL (neutrophils, 88.9%). Abdominal ultrasound showed a target sign-like finding in the ileocecal region. A computed tomography scan showed swelling of the appendix. We performed an emergency operation under suspicion of acute appendicitis. Laparoscopic examination showed that the appendix had twisted 720° in the clockwise direction. Appendectomy was performed, and the postoperative course was uneventful. CONCLUSIONS: Although torsion of the vermiform appendix is a very rare disease and difficult to differentiate from appendicitis, an inappropriate treatment plan could lead to necrosis and perforation of the appendix. It is important to consider this disease as a differential diagnosis in patients with right lower abdominal pain.

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.

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.

Effective Surveillance to Identify the Surgical Patients Carrying Methicillin-resistant Staphylococcus aureus on Admission in a Pediatric Ward.

Background To effectively detect surgical patients carrying methicillin-resistant Staphylococcus aureus (MRSA) in a pediatric ward, we investigated who should be tested through bacteriological surveillance on admission. Methods In 1124 patients, bacteriological examination of throat swabs and stool samples was performed within 48 hours after admission. The relations between carrier rate (CR) of MRSA and a history of hospitalization, neurological disorder (ND), and age group were retrospectively investigated. Results The CR of all patients was 7.8%. The CRs of the patients with no history of hospitalization, a history of hospitalization before the previous year, and a history of hospitalization within the past year (HWPY) were 2.3%, 3.4%, and 14.5%, respectively; the CR of those with HWPY was significantly higher than the CRs of the other 2 groups (p<0.0001). The CR of the patients with an ND (19.2%) was significantly higher than that of the patients without an ND (6.1%) (p<0.0001). The CRs of the patients <3 (11.7%) and 15 (11.9%) years old were higher than that of other patients (3.8%) (p< 0.05). With multivariate logistic regression analysis, HWPY, an ND and age groups <3 and k15 were independent risk factors. Conclusions - HWPY, an ND, and being <3 or k 15 years old were found to be risk fqctors for carrying MRSA on admission. Active bacteriological surveillance on admission should be performed for patients with these risk factors.

Successful emergency combined therapy with partial splenic arterial embolization and endoscopic injection therapy against a bleeding duodenal varix in a child.

There is no consensus guidelines for treating duodenal variceal bleeding, which is a rare and life-threatening complication of portal hypertension. Here we report an exceedingly unusual case in a 9-year-old boy who had developed left-sided portal hypertension after surgical treatment for pancreatoblastoma followed by a duodenal variceal bleeding with massive melena, severe anemia (hemoglobin 4.5 g/dL) and hypovolemic shock. Emergency partial splenic arterial embolization (PSE) provided a reduction of variceal bleeding and improved blood pressure. Endoscopic injection sclerotherapy (EIS) was subsequently performed and stopped the duodenal variceal bleeding without the complication of portal vein thrombosis caused by injected sclerosant under hepatopetal flow. Our case demonstrates that emergency combined therapy with PSE and EIS can be considered as the therapeutic option for the management of left-sided portal hypertension-induced ectopic variceal bleedings in order to avoid the complication of portal embolization by EIS and provide effective hematostasis.

A case of fetal intestinal volvulus without malrotation causing severe anemia.

Fetal intestinal volvulus without malrotation is a rare, life-threatening disease. Left untreated, hemorrhage from necrotic bowel tissue will lead to severe fetal anemia and even intrauterine death. We encountered a case of fetal intestinal volvulus causing severe anemia, which was diagnosed postnatally and successfully treated with surgical intervention.

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

BACKGROUND: 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. METHODS: In 184 PBM patients and 22 HCPBD patients who were diagnosed by direct cholangiography and underwent MRCP, the length of the common channel was measured. RESULTS: The length of the common channel was 16.2 ± 6.9 mm on direct cholangiography and 13.9 ± 6.2 mm on MRCP in PBM patients, and 7.7 ± 1.5 mm and 6.6 ± 1.4 mm in HCPBD patients. The optimal cut off value of the length of the common channel to distinguish PBM from HCPBD was identified to be 9 mm. CONCLUSIONS: Pancreaticobiliary maljunction can be diagnosed from an abnormally long common channel on MRCP, but in cases of the common channel ≤9 mm on MRCP, direct cholangiography is needed to confirm PBM.

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.

Single-incision laparoscopic percutaneous extraperitoneal closure for inguinal hernia in children: an initial report.

BACKGROUND: This report describes the first use of single-incision, two-port access for single-incision laparoscopic percutaneous extraperitoneal closure (SILPEC) to manage inguinal hernia in children. METHODS: Between December 2009 and April 2010, 92 SILPECs of inguinal hernia and hydrocele were performed for 62 patients at Osaka City University Graduate School of Medicine. The SILPEC technique was performed using two ports (a 4.2-mm port placed using the open technique and an additional 4.2-mm port) inserted through the same periumbilical skin incision with different entrances through the abdominal wall. A 19-gauge LPEC needle (a special instrument with a wire loop at the tip to hold the material for circuit suturing around the internal inguinal ring) was used to close the orifice of the hernial sac extraperitoneally with circuit suturing around the internal inguinal ring. Data regarding patient demographics, type of hernia, operating time, complications, postoperative hospital stay, and recurrence were prospectively collected. RESULTS: The mean operative time was 26.9 min. The estimated blood loss was almost nil, and no intraoperative complications occurred. CONCLUSION: The use of SILPEC for inguinal hernia and hydrocele in children appears to be safe, effective, and reliable.

Clinical and pathological features of ascending testis.

BACKGROUND: The clinical features and principles of surgery of the Ascending Testis (AT), in which the testis ascends from the scrotum with age, are controversial. We selected AT that met the treatment guidelines and examined the clinical features and principles of surgery. METHODS: We retrospectively reviewed the medical charts of 12 AT and 530 Congenital Undescended Testis (CUDT) patients to investigate the statistics of AT among all CUDT, the side of the maldescent, the associated disorders, and the clinical course. The patients were compared in terms of testicular volume (TV), mean tubular diameter (MTD), and testicular fertility index (TFI) between the AT group and the CUDT group. RESULTS: AT was observed in 2.2% of all CUDT patients: 10 had left AT and 2 had bilateral AT. Among the 12 AT patients, 8 patients had associated disorders. The TV, MTD, and TFI of 7 AT and 7 CUDT were 0.74 +/- 0.20 and 0.77 +/- 0.59 cm3, 38.7 +/- 3.7 and 35.9 +/- 9.1 microm, and 0.19 +/- 0.07 and 0.11 +/- 0.11, respectively. CONCLUSIONS: In order to examine the clinical features of AT, it is essential to strictly rule out CUDT and retractile testis. In our study, the clinical features are that AT is a rare disorder, occurs more frequently on the left side, and many patients with AT have associated disorders. Since AT causes histological damage equivalent to that caused by CUDT, we should perform orchidopexy for patients with AT immediately after diagnosis.

Classification of congenital biliary cystic disease: special reference to type Ic and IVA cysts with primary ductal stricture.

Congenital biliary cysts occur not only in the choledochus but also everywhere in the biliary tree, and are frequently accompanied by pancreatobiliary malunion. Alonso-Lej and colleagues first classified choledochal cysts into three types, in 1959. Due to the recognition of intrahepatic involvement, we refined their classification into six types, in 1977, and this has become the reference. However, this classification does not include the concept of pancreatobiliary malunion and has caused some confusion, especially in regard to types Ic and IVA. Type Ic, showing fusiform dilatation, has a pancreatobiliary malunion and often extends continuously to the intrahepatic duct. Type IVA, with intrahepatic involvement, shows primary ductal stricture, especially around the hepatic hilum and umbilicus. The incidence of the stricture is considerably greater than was previously assumed, and upstream intrahepatic ducts often join the dilated duct, with distinct caliber change. Intrahepatic dilatation may develop primarily, due to the stricture, combined with weakness of the duct wall. This stricture can be corrected by portal dissection in the liver to obtain a large anastomosis. In patients with intrahepatic involvement, we should carefully find the stricture by suitable cholangiograms, or by intraoperative cholangioscopy. Detecting the ductal stricture is obviously important, rather than classifying the cyst as type Ic or IVA.

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

PURPOSE: 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 patient's 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.

Comparison of the Nuss procedure for pectus excavatum by age and uncommon complications.

BACKGROUND: We summarized our experience of the Nuss procedure for pectus excavatum with comparison by age and uncommon complications. METHODS: Twenty-three patients underwent the Nuss procedure. Their age ranged from 3 to 19 years old. The outcome was compared between two groups divided by age: teenagers (> or = 13 yrs old, n = 5, Group 1) and younger patients (n = 18, Group 2). RESULTS: Cosmetic results were significantly better, and the operating time and postoperative hospital stay in Group 2 were significantly shorter than in Group 1. Complications were more frequently seen in Group 1 than in Group 2. In all three younger patients whose bars were removed after 2 years, the ribs holding the bar were deformed. Uncommon complications occurred in two cases. In one case, pneumothorax occurred due to laceration by a fragment of wire after 12 months, and the fragment fell into the thoracic cavity. In another case, pneumothorax occurred due to injury by the port of a thin thoracoscope. CONCLUSION: The teenaged patients had more complications and poorer cosmetic results. Therefore, this operation should be performed before the teenage years. In younger patients with rapid growth, the bar should be removed less than 2 years to prevent restriction of costal growth.