Selective enlargement of left lateral segment liver volume as a potential diagnostic predictor for biliary atresia.

PURPOSE: To determine an early diagnostic indicator of biliary atresia (BA), we focused on morphological left-right differences of BA livers. METHODS: Of 74 infants with suspected BA at our hospital in the last 12 years, 25 met the conditions for investigation: 15 infants with BA (BA group) and 10 with other pathologies (non-BA group). CT volumetry of the liver in each patient was performed using a 3D image analysis system. Patient characteristics, blood data, and proportion of the left lateral segment to the total liver volume (LLS ratio) were compared between the two groups. RESULTS: Among the patient characteristics and liver function tests, only γ-glutamyl transpeptidase (GGT) were significantly higher in the BA group (p < 0.001). The LLS ratio was 0.321 (0.227-0.382) in the BA group and 0.243 (0.193-0.289) in the non-BA group (p = 0.01). The summary cut-off, area under the curve, sensitivity, and specificity were 0.322, 0.813, 53.3, and 100% for the LLS ratio and 94.26, 0.95, 86.7, and 100% for the GGT × LLS ratio, respectively. CONCLUSIONS: The LLS ratio is highly specific and may be an early diagnostic predictor of BA. Moreover, this segmental LLS enlargement may be associated with the etiology of BA.

Segmental Atrophy of Explanted Livers in Biliary Atresia: Pathological Data From 63 Cases of Failed Portoenterostomy.

OBJECTIVES: Atrophy of the left lateral segment (LLS) is often encountered in liver transplantation (LT) for biliary atresia (BA). To clarify the meaning of the heterogeneous atrophy, we compared the pathological characteristics of the LLS with the right posterior segment (RPS) of BA livers obtained during LT. METHODS: Among the 116 patients with BA who underwent LT at our hospital between 2014 and 2018, 63 patients with persistent cholestasis after the Kasai portoenterostomy (KP) were selected. Three pathologists evaluated tissues from the LLS and RPS for 5 pathological parameters. Positive areas in whole-slide image observed as portal inflammation, fibrosis, cholestasis, and ductular reaction, were analyzed with automated image quantitation. Moreover, we examined the relationship between the pathological score and the Pediatric End-stage Liver Disease (PELD) score. RESULTS: The median age at LT was 7 months (range 4-26 months). Inflammation and fibrosis were significantly greater in the LLS than in the RPS (P < 0.001, for both); however, there were no differences in cholestasis, ductular reaction, and hepatocellular damage (P = 0.3, 0.3, and 0.82). The same results were obtained in automated image quantitation. Moreover, the sums of the 5 pathological scores in the LLS showed a significant positive correlation with the PELD score (P = 0.016, rs = 0.3). CONCLUSIONS: More severe inflammation and fibrosis without cholestasis were observed in the LLS. The segmental atrophy may not be associated with poor bile drainage, but with etiopathogenesis of BA. Moreover, the proper site for biopsy during KP could be the LLS.

Successful abdominal wall closure following collagen-based artificial dermis induced epithelialization for giant omphalocele: A case report.

INTRODUCTION: A giant omphalocele (GO) with marked viscero-abdominal disproportion is associated with surgical difficulty and higher morbidity and mortality rates. Despite various treatment strategies, no consensus exists on optimal GO management. We report the clinical course of a neonate with a GO who was successfully treated with abdominal-wall closure through the novel application of collagen-based artificial dermis (CAD) for epithelization. PRESENTATION OF CASE: A female neonate (estimated gestational age, 38 weeks; birthweight, 3.047 kg) with a GO where most viscera, including the liver, were completely herniated. G-band analysis showed no chromosomal abnormality and normal karyotype. Conventional silo formation was attempted, but incomplete silo was formed due to adhesion between the portal vein and fascia, and repatriation of the herniated viscera had not progressed. A new silo was formed using biomaterial, but it was infected and removed. Abdominal wall epithelialization using NPWT was attempted again but was interrupted by the occurrence of jejunal perforation. After incising the epithelialized part of the abdominal wall and repairing the perforated jejunum, the GO was covered and fixed using CAD. Epithelialization progressed well, and she was discharged on day 328. DISCUSSION: In this case, the major therapeutic challenges (including formation of an incomplete silo, silo infection, and jejunal perforation) were overcome with conventional treatment except for epithelialization of the abdominal wall, which was achieved by using CAD. CONCLUSION: The treatment with CAD for epithelialization can be considered in cases where it is extremely difficult to return the viscera in conventional management.

Liver resection for a congenital intrahepatic portosystemic shunt in a child with hyperammonemia and hypermanganesemia: a case report.

BACKGROUND: Congenital portosystemic shunt (CPSS) is a rare malformation that leads to hyperammonemia, hypermanganesemia, and various symptoms. CPSSs are divided into intrahepatic and extrahepatic shunts. In patients with persistent CPSS including an intrahepatic portosystemic shunt (IPSS), early intervention to occlude the shunt reverses the associated complications. CASE PRESENTATION: The patient was a 1-year-and-7-month-old girl. She presented with hypergalactosemia and elevation of blood ammonia level (75 µg/dL) and total bile acid levels (68.2 µmol/L) during the neonatal period. Two IPSSs were detected using ultrasound and enhanced computerized tomography. Magnetic resonance imaging (MRI) at 1 year and 3 months of age showed abnormally high signal intensity in the pallidum of her brain. Spontaneous closure was not observed. We performed a right hepatectomy at 1 year and 7 months of age. The portal vein pressure was 16 mmHg after temporary occlusion of the right portal vein. Blood ammonia and serum manganese levels decreased immediately after the operation. The abnormal signal on brain MRI disappeared. She had a favorable course with no sign of recurrence of IPSS 5 years postoperatively. CONCLUSION: Liver resection for an IPSS to control the symptoms of a portosystemic shunt is reasonable in a child for whom interventional radiological treatment is not indicated.

Ultrasound-guided hydrostatic enema for meconium obstruction in extremely low birth weight infants: a preliminary report.

Meconium obstruction (MO) in extremely low birth weight (ELBW) infants is a challenging disease to treat. We performed ultrasound-guided hydrostatic enema on six ELBW infants diagnosed with MO. We consider this procedure to be safe and effective, and recommend it as a treatment for MO in ELBW infants.

Implantable central venous access device in infants: Long-term results.

BACKGROUND: The management of central venous access device (CVAD) is extremely difficult in babies and small infants (BSI). We therefore compared the long-term results and complications of CVAD in BSI with those in children. METHODS: One hundred and twenty patients were divided into two groups as follows: age <1 year or weight <10 kg (group A; 25 patients); and age ≧1 year and weight ≧10 kg (group B; 95 patients). The clinical results were retrospectively compared between the groups. RESULTS: Mean age and weight were 12.5 ± 5.9 months and 8.2 ± 1.2 kg in group A, and 78.8 ± 58.9 months and 20.9 ± 13.6 kg in group B. Operation time was 57 ± 29 min in group A and 52 ± 21 min in group B (P = 0.38). The catheter was advanced with difficulty into the central vein in five and in 16 patients (P = 0.77), and surgical complications occurred in one and in seven patients in groups A and B, respectively (P > 0.99). The CVAD remained in place for 627 ± 494 and 550 ± 414 days (P = 0.47) and was removed before treatment completion in five and in 14 patients in groups A and B, respectively (P = 0.54). CONCLUSION: The clinical results for CVAD in BSI did not differ from those in children. CVAD are useful and safe for the treatment of BSI with serious diseases.

Early Detection and Treatment of Neuroblastic Tumor with Opsoclonus-Myoclonus Syndrome Improve Neurological Outcome: A Review of Five Cases at a Single Institution in Japan.

INTRODUCTION: Opsoclonus-myoclonus syndrome (OMS) is a paraneoplastic neurological disorder associated with neuroblastic tumor (NT) in childhood. Half of patients have neurological sequelae after the neurological and oncological treatment. We reviewed the neurological and oncological outcomes of NT with OMS, and discussed whether the treatment of NT would contribute to improving the neurological prognosis. METHODS: We retrospectively assessed NT patients with OMS from January 2001 to December 2013 at a single institution in Japan. Demographic data, neurological and oncological status, histopathology, treatments, prognosis, and diagnosis and treatment timing were retrospectively reviewed from the records. The timings assessed were the interval between OMS onset and NT detection, initial NT therapy, and initial OMS therapy, the interval between NT therapy and OMS remission, and duration of OMS. RESULTS: A total of 73 patients with NT were treated during the study period, and 5 of 73 patients were diagnosed as having NT with OMS. The median age at onset of OMS was 22 months (range, 18-30 months). The median age at detection of NT was 29 months (range, 21-33 months). Three of five cases showed no uptake on meta-iodobenzylguanidine scintigraphy. The tumor histopathology was neuroblastoma in two patients, ganglioneuroblastoma in two patients, and ganglioneuroma in one patient. Primary resection was performed in three cases. All patients survived. Two of five cases presented with atypical neurological symptoms without opsoclonus. The initial neurological therapy was started within a mean of 20 days (range, 3-76 days) from the onset of OMS in all cases. Four patients received intravenous immunoglobulin, and one with persistent neurological problems received rituximab. Neurological symptoms resolved in three cases. The mean interval between the onset of OMS and the detection of NT in case without neurological sequelae was 57 days (range, 25-113 days), while in case with neurological sequelae it was 365 days (range, 271-458 days). The mean interval between onset of OMS and initial therapy for NT in case without neurological sequelae was 88 days (range, 47-145 days), while in case with neurological sequelae it was 389 days (range, 292-486 days). CONCLUSION: The interval between the onset of OMS and the detection and initial therapy of NT tended to be longer in patients with neurological sequelae than in those without neurological sequelae. This study suggested that early detection and treatment of NT with OMS might improve the neurological outcomes.