Kyoko Mochizuki

Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: >20-year experience at a children's hospital.

Objective: Sequential strategies combining the Kasai operation as a first-line treatment and liver transplantation as a second-line option, if necessary, have been accepted for patients with biliary atresia (BA). To understand the role of the Kasai operation in the treatment of BA, it is necessary to analyze the long-term outcome of the operation alone and to evaluate the present status of survivors retaining their native livers. Materials and Methods: A retrospective chart review was carried out for a group of 80 patients who had undergone the Kasai operation between 1970 and 1986 at the Kanagawa Childrens Medical Center. Results: The 5-, 10-, and 20-year survival rates of patients with their native livers were 63%, 54%, and 44%, respectively. The survival rates varied significantly depending on the type of BA, age at initial Kasai operation, era of surgery, and surgical method. By age 20, nearly half of the adult survivors had already developed liver cirrhosis and its sequelae. Episodes of cholangitis and gastrointestinal bleeding occurred after 20 years of age in 37% and 17% of the adult patients, respectively, and 20% of the adult patients died of liver failure or underwent living-related partial liver transplantation in their 20s. Five female patients gave birth to a total of 9 children, and 1 male patient fathered a child. Conclusions: Although increasing numbers of patients with BA survive 20 years or more after the Kasai operation, meticulous lifelong postoperative care should be continued for the survivors because of the possibility of hepatic deterioration.

The usefulness of a high-speed 3D-image analysis system in pediatric living donor liver transplantation

BACKGROUND Since March 2010, we have used a high-speed 3D-image analysis system (SYNAPSE VINCENT) to calculate the graft volume in living donor liver transplantation (LDLT) to replace CT volumetry. The SYNAPSE VINCENT is capable of extracting each vessel territory in the liver and displaying 3D images simply, quickly, and accurately. Therefore, we report here the usefulness of the SYNAPSE VINCENT in pediatric LDLTs in overcoming issues with perfusion area of hepatic venous tributaries in monosegmental grafts. MATERIAL/METHODS The SYNAPSE VINCENT was used in three pediatric patients. In two of these cases, the possibility of monosegmental grafts was assessed when calculating graft volumetry of segment III. RESULTS The graft recipient weight ratio (GRWR) with graft volumetry measurements of the left lateral segment were 1.8-5.6%. GRWR of segment III were 2.3 and 2.0%. Since donor V2, venous branch to segment II and V3, venous branch to segment III were independently branching in one case, the monosegmental graft could be evaluated preoperatively according to the venous perfusion. CONCLUSIONS Graft volumetry using the SYNAPSE VINCENT was useful for planning the LDLT operative procedures, especially in infants possibly in need of monosegmental graft.

Partial internal biliary diversion for patients with progressive familial intrahepatic cholestasis type 1

We herein report a case of progressive familial intrahepatic cholestasis with partial internal biliary diversion (PIBD). Although by using PIBD an external stoma can be avoided, exposure of the ileocecal junction to bile reflux as well as the effects of the direct bile flow on the colonic mucosa require further investigation.

A modified Foker’s technique for long gap esophageal atresia

External traction using the Foker’s technique enables elongation in the esophageal segments within days, and allows the primary repair of the long gap. This article presents our modified Foker’s technique which was easily applicable for long-gap esophageal atresia.

Possible protection of sinusoidal endothelial cells by endothelin B receptor during hepatic warm ischemia-reperfusion.

PurposeEndothelins (ETs) are important regulators of the hepatic microcirculation. We investigated the pure biological roles of endothelin B receptors (ETB-Rs) on hepatic warm ischemia–reperfusion (I/R) injury using ETB-R deficient spotting lethal (sl) rats.MethodsHomozygous (sl/sl) and wild-type (+/+) rats were exposed to 60 min of 92% partial hepatic ischemia and then were killed at 2, 6, and 24 h, and 3 and 7 days after reperfusion. We measured the serum alanine aminotransferase (ALT) levels to assess hepatocyte injury, and the serum hyaluronic acid (HA) levels and factor VIII-related antigen (FVIIIRAg) staining to assess sinusoidal endothelial cell (SEC) injury. We also measured the concentrations of ET-1 and nitrite (NO2−) and nitrate (NO3−) of liver tissue samples.ResultsAlthough no significant difference was observed in the ALT levels, the HA levels were significantly elevated at an early stage after reperfusion in the sl/sl rats. Regarding FVIIIRAg staining, positive SECs were enhanced in the sl/sl rats. The ET-1 levels were also significantly elevated at an early stage after reperfusion in the sl/sl rats. Regarding the NO2− and NO3− levels, no significant difference was observed.ConclusionEndothelin B receptor was shown to have a protective effect on SECs through the inhibition of ET-1 during hepatic warm I/R injury.

Expression of alpha smooth muscle actin in living donor liver transplant recipients

Recently, there have been reports from liver biopsies that showed the progression of liver fibrosis in liver transplant patients after the cessation of immunosuppression. Herein, we focused on activated hepatic stellate cells expressing alpha smooth muscle actin (α-SMA) to understand the correlation between immunosuppressant medication and liver fibrosis. The study enrolled two pediatric patients who underwent living donor liver transplantation and ceased immunosuppressant therapy. The number of α-SMA-positive cells in the specimens obtained by liver biopsy from these two patients showed a three-fold increase compared with the number from four transplanted pediatric patients who were continuing immunosuppressant therapy. In addition, the α-SMA-positive area evaluated using the WinRooF image processing software program continued to increase over time in three adult transplanted patients with liver fibrosis, and the α-SMA-positive area was increasing even during the pre-fibrotic stage in these adult cases, according to a retrospective review. Therefore, α-SMA could be a useful marker for the detection of early stage fibrosis.

Yolk sac tumor of the vulva: a case report with recurrence after long-term follow-up

A yolk sac tumor (YST) of the vulva is extremely rare and highly malignant with recurrence frequently occurring within a year. This report presents the 13th known case of vulvar YST, with recurrence occurring after the longest known follow-up period so far reported in the literature.

Hemi-hepatectomy in pediatric patients using two-surgeon technique and a liver hanging maneuver

AIM To evaluate the efficacy of the two-surgeon technique with the liver hanging maneuver (LHM) for hepatectomies in pediatric patients with hepatoblastoma. METHODS Three pediatric patients with hepatoblastoma were enrolled in this study. Two underwent right hemi-hepatectomies and one underwent a left hemi-hepatectomy using the two-surgeon technique by means of saline-linked electric cautery (SLC) and the Cavitron Ultrasonic Surgical Aspirator (CUSA; Valleylab, Boulder, CO) and the LHM. RESULTS The mean operative time during the parenchymal transections was 50 min and the mean blood loss was 235 g. There was no bile leakage from the cut surface after surgery. No macroscopic or microscopic-positive margins were observed in the hepatic transections. CONCLUSION The two-surgeon technique using SLC and CUSA with the LHM is applicable to even pediatric patients with hepatoblastoma.

Mediastinoscopic extended thymectomy for pediatric patients with myasthenia gravis.

BACKGROUND Extended thymectomy is indicated for children with myasthenia gravis (MG) when drug-resistance or dependence is seen. We have employed a technique for mediastinoscopic extended thymectomy (MET) on children with MG. METHOD A total of 14 children underwent MET at Kanagawa Childrens Medical Center between 2005 and 2013. A mediastinal operation field was made by a V-shaped hook infrasternally to extirpate the thymus with adipose tissue around the thymus. RESULTS The operation time and the amount of blood loss were 182±44 minutes and 34±43 ml, respectively. Postoperative complications, in the form of transient paralysis of the right recurrent nerve, occurred in 2 patients. The median length of postoperative hospital stay was 4.5 days. After MET, 6 patients achieved complete remission and 7 patients achieved steroid dose reduction, but no improvement was seen in 1 patient. CONCLUSIONS This procedure offers the advantage of good surgical access for dissection around the bilateral phrenic nerves in extended total thymectomy, while achieving good cosmetic results.

Anorectal malformation associated with a perineal protrusion of the rectal mucosa: case presentation

We present a rare case of a female neonate with an imperforate anus associated with a perineal mass which may correspond to an extrophied rectal duplication. Associated anomalies were thoracic hemivertebrae and a multicystic dysplastic kidney. Excision of the perineal lesion followed by anal transplantation and perineal reconstruction corrected the anomaly.