Michihiro Sugai

Adenoma of the Nipple in an Adolescent

We recently treated a 14-year-old girl with a clinically and histologically diagnosed with adenoma of the nipple. Enucleation of a mass preserving the nipple was successfully performed. Adenoma of the nipple is a rare disease which is often mistaken clinically for Paget’s disease. About 200 cases of the tumors have been reported worldwide so far. The most common symptom is erosion of the nipple and nipple discharge. Our case had erosion of the nipple but no discharge. Adenoma of the nipple is a benign lesion which can be successfully treated by a simple surgery.

Hepatic venous outflow obstruction in living donor liver transplantation: balloon angioplasty or stent placement?

BACKGROUND The incidence of hepatic venous outflow obstruction (HVOO) has been reported to be 5%-13% when a partial graft is used for orthotopic liver transplantation (OLT). HVOO leads to graft congestion, portal hypertension, and finally cirrhosis, which jeopardizes both graft and recipient survivals. In this study, we sought to identify perioperative factors influencing HVOO and to investigate conditions that require stent placement. PATIENTS AND METHODS From February 1994 to December 2010, we performed 40 living donor liver transplantations (LDLT). HVOO occurred in 5 cases (12.5%), all of which were left lobe grafts. Because HVOO was not observed in patients with body weight (BW) <30 kg, we investigated the other 28 cases with BW >30 kg. RESULTS There was no difference from unaffected subjects except for cold ischemic time (CIT), which was significantly longer: 86.2 ± 10.4 minutes vs 46.0 ± 4.8 minutes (P = .001). Balloon angioplasty, which was selected as the initial treatment for all stricture patients, improved 2 patients after 1 and 5 treatments, respectively, but 3 subjects underwent repeated HVOO, finally being treated with self-expandable metallic stents at 9, 6, and 10 years after LDLT, respectively. All patients finally resolved their strictures. CONCLUSION HVOO reflects intimal hyperplasia and fibrosis at the anastomotic sites or compression and twisting of the anastomosis caused by graft regeneration. In addition, progression of chronic rejection and fibrosis are possibly responsible for late-onset HVOO. Longer CIT possibly reflects difficulties in the venoplasty before anastomosis. No bleeding or thrombosis complications were observed during dilatation among our cases. The selection of the stent size for each case and careful stent deployment are important to prevent complications. Stent placement should be considered in patients with chronic rejection who are refractory to several balloon angioplasties with early-onset or late-onset HVOO.

Torsion of an accessory lobe of the liver in a child: report of a case.

We report a case of torsion of an accessory lobe of the liver (ALL) in a 14-year-old girl. The patient was admitted for acute abdominal pain and laparotomy revealed an ALL, the pedicle of which was elongated with 180° torsion. The diagnosis was not made preoperatively because of the rarity of this condition. However, a close relationship between omphalocele repair and the development of ALL has been reported; thus, a history of omphalocele repair should alert the doctor to the possibility of this condition, which could be suggested by imaging findings preoperatively. Torsion of an ALL should be included in the differential diagnosis of a patient with a history of omphalocele, who presents with acute abdominal symptoms.

Liver Transplantation for Wilson's Disease in Pediatric Patients: Decision Making and Timing

Transplantation for Wilsons disease occupies 1/3 of the cases for metabolic diseases in Japan. At the end of 2009, 109 transplantations had been performed including three deceased donor cases in the Japanese registry. We herein discuss problems of transplantation for Wilsons disease as well as its indication, timing, and social care. We retrospectively reviewed four fulminant cases and two chronic cases who underwent living donor liver transplantation. There were two boys and two girls. Four adolescents of average age 11.3 years underwent living donor liver transplantation. Duration from onset to transplantation ranged from 10 to 23 days. Average Model for End-stage Liver Disease (MELD) score was 27.8 (range=24-31). All patients were administrated chelates prior to transplantation. MELD, New Wilsons index, Japanese scoring for liver transplantation, and liver atrophy were useful tools for transplantation decision making; however, none of them was an independent decisive tool. Clinical courses after transplantation were almost uneventful. One girl, however, developed an acute rejection episode due to noncompliance at 3 years after transplantation. All patients currently survive without a graft loss. No disease recurrence had been noted even using living related donors. Two adults evaluated for liver transplantation were listed for deceased donor liver transplantation. Both candidates developed cirrhosis despite long-term medical treatment. There were no appropriate living donors for them. There are many problems in transplantation for Wilsons disease. The indications for liver transplantation should be considered individually using some decision-making tools. The safety of the living donor should be paid the most attention.

Donor Quality of Life After Living Donor Liver Transplantation: Single-Institute Experience

AIM Living donor liver transplantation (LDLT) has been widely accepted because of the severe shortage of hepatic grafts. However, the healthy donor is exposed to risks of morbidity and mortality. In this study, we analyzed medical, functional, and psychological outcomes of donors after hepatectomy for liver donation. PATIENTS AND METHODS Among 41 donor hepatectomy cases for LDLT performed in our institute from January 1994 to May 2011, we reviewed the medical records (liver function tests, complications, etc) of 27 subjects who donated to recipients older than 12 years. We also performed a questionnaire survey based on the Japanese Short Form-36 version 2 Health Survey scales as a measure of physical and mental health, to which 31 subjects responded. RESULTS Six of the 27 donors experienced prolonged jaundice. Their ratios of graft volume/standard donor liver volume (GV/SDLV) were higher than those of the 21 donors without prolonged jaundice (60.0% vs 41.5%). According to the questionnaires, social functioning among those having undergone emergency hepatectomy as well as general health perceptions declined in those with postoperative complications. Physical component summary declined among those having undergone emergency hepatectomy and with postoperative complications. CONCLUSION In liver donation from a living donor, massive hepatectomy should be avoided. A ratio of GV/SDLV around 50% seems reasonable. Donors with emergency transplantations or postoperative complications must be more carefully followed after donor hepatectomy.

Timing for Orthotopic Liver Transplantation in Children With Biliary Atresia: A Single-Center Experience

INTRODUCTION Biliary atresia is the most common indication for orthotopic liver transplantation (OLT) in childhood. The purpose of this study was to determine predictive prognostic factors for children with biliary atresia related to the timing for OLT within 15 months after hepatoportoenterostomy (HPE). PATIENTS AND METHODS We retrospectively analyzed the medical records of 25 children (7 boys and 18 girls) who underwent HPE because of biliary atresia between January 1990 and December 2005 at our center. Data examined included age and pathologic findings at HPE, Pediatric End-Stage Liver Disease score at first admission, whether phototherapy was given, liver function test results and total bilirubin level before and 30 days after HPE, and number of cholangitis events. RESULTS Twelve children were alive with their native liver, 8 had undergone living donor OLT (all children alive), and 5 had died without OLT. Five- and 10-year survival rates without OLT after HPE were 47.4% and 26.3%, respectively. At univariate analysis, the predictive prognostic factors for children with biliary atresia were total bilirubin level at 30 days after HPE and Pediatric End-Stage Liver Disease score before HPE. At multivariate analysis, the only prognostic factor was total bilirubin level at 30 days after HPE. CONCLUSIONS In this study, the predictive prognostic factor was total bilirubin level at 30 days after HPE. Orthotopic liver transplantation within 15 months after HPE is needed in children with biliary atresia with a high total bilirubin level at 30 days after HPE.

A case of pancreatoblastoma prenatally diagnosed as intraperitoneal cyst

Pancreatoblastoma in childhood is a very rare malignant tumor, but is considered to have a relatively good prognosis because of its low metastatic potential. We report 1-day-old female infant who was recently found to have an intraabdominal cyst on prenatal ultrasound examination. The tumor was a unilocular, cystic mass without invasion or metastasis to other organs, allowing total resection. It was diagnosed postnatally with pancreatoblastoma.

Importance of Awareness of Perioperative Social and Physical Situations of Living Donors for Liver Transplantation

INTRODUCTION Transplantation in Japan still depends on living donors even after the new revised law. We must pay attention to protect living donors. PATIENTS AND METHODS Perioperative qualities of life after living donation for liver transplantation were assessed with questionnaires including the Medical Outcomes Study 36-Item Short-Form Health Survey version 2 (SF36-v2). Nonparametric Mann-Whitney tests were used to determine statistical significance. P values<.05 were considered significant. RESULTS Thirty-one among 33 donors answered questionnaires (93.9%). The 15 men and 16 women of average age of 39.7 years had a median hospital stay of 16 days and median duration after surgery of 78 months. Ten of 33 (35.7%) donors considered themselves to be the only possibility. The decision to a donor was established prior to informed consent in 23 donors (74.1%). Six months were required for them to experience a full recovery after donor surgery. Hamilton depression/anxiety score was significantly increased among donors who considered themselves to be the only possibility or those who had decided prior to informed consent. SF36-v2 revealed a significant decrease in social functioning among donors who did not have sufficient time to decide before surgery. General health was significantly decreased among donors who required more than 6 months for full recovery. Perioperative management of pain influenced general health, physical role, bodily pain, and physical functioning. CONCLUSION We must pay attention to depression and anxiety among living donors. More care should be focused on pain control and sharing of information of postoperative courses.

Two Cases of Twisted Fetal Ovarian Cysts

Two twisted fetal ovarian cysts were detected antenatally by routine ultrasonographic examination. Serial changes of twisted ovarian cysts can be monitored by ultrasonic observation. If signs of torsion appear, obstetricians should consider prompt delivery in order to preserve the patients fertility.

Monitoring of hepatic artery resistance index and optimal timing of liver transplantation for biliary atresia

PurposeBiliary atresia (BA) is diagnosed by ultrasound (US) examination of the hepatic artery and gallbladder. This study was designed to assess the usefulness of the hepatic artery resistance index (HRI) measured by Doppler ultrasonography (DUS) for prognostication of liver viability in children with BA.MethodsSeventeen patients with non-correctable BA were examined by US and DUS before and after hepatoenterostomy to evaluate hepatic artery dynamics.ResultsDilatation of the hepatic artery was demonstrated in all 17 patients. US of the gallbladder showed hypogenesis in seven patients. Preoperatively, all 17 BA patients had an HRI of <1.0. Six of the 17 patients underwent liver transplantation. Four of the six had an HRI of >0.9, and the HRI decreased after the procedure. All patients were alive at the time of writing.ConclusionsRegular US examinations are helpful for diagnosis of BA and for detecting patients at high risk. A prospective study is required to determine the optimal frequency of assessment.