Living donor liver transplantation for Dorfman-Chanarin syndrome with 1 year follow-up: case report.

A 27-year-old Japanese man underwent liver transplantation because of uncompensated cirrhosis due to Dorfman-Chanarin syndrome (DCS). At birth, the patient displayed ichthyosis and liver dysfunction. Moreover, mental retardation appeared and intracytoplasmic vacuoles were observed within peripheral blood neutrophils. A fatty liver was also noticed, leading to the diagnosis of DCS. When he was referred to our hospital, his American Society of Anesthesiologists score was 3. The findings of computed tomography showed liver atrophy, splenomegaly, and ascites. The Child-Pugh score was B, and the Model for End-stage Liver Disease score was 14. The pathophysiology was DCS with uncompensated liver cirrhosis. Therefore, living donor liver transplantation (LDLT) was performed from the patient's brother. The histological appearance of the resected liver revealed macrovesicular steatosis in most hepatocytes with excess fibrous tissue in the portal areas. These findings were compatible with nonalcoholic steatohepatitis. Although the patient's mental retardation and characteristic appearance have not improved, good liver function has been maintained since LDLT. An outpatient protocol liver biopsy performed at 12 months after LDLT did not show recurrence of macrovesicular steatosis.

A survival case of ABO-incompatible liver transplantation complicated with severe preoperative infection and subsequent overwhelming postsplenectomy infection.

A 47-year-old Japanese man was transferred to our hospital because of acute-on-chronic hepatitis B virus infection. On admission, he was suffering from sepsis due to a catheter infection and respiratory failure caused by pulmonary edema and pneumonia, but, as a result of preoperative intensive care, we avoided septic shock. ABO-incompatible liver transplantation (ABO-I-LT) was performed. In accordance with our ABO-I-LT protocol, we administered, rituximab and performed plasma exchange, splenectomy as well as hepatic artery infusion. The patient was discharged 80 days after living donor transplantation (LDLT). However, 136 days after LDLT, he experienced recurrent respiratory failure due to severe pneumonia. At that time, the CD19(+) B-cell count in the peripheral blood flow remained below 1%. We suspected a mixed infection involving Streptococcus pneumonia, Pneumocystis carinii, and fungus. The cause of the complication was overwhelming postsplenectomy infection (OPSI). We started administration of sulfamethoxazole and trimethoprim, ciprofloxacin hydrochloride, and micafungin sodium therapy as well as gamma-globulin. Oxygenation improved gradually; the patient was discharged at 41 days after re-admission. Although this patient survived the OPSI, it was clear that some aspects of the ABO-I-LT protocol should also be altered.

Complete neurological recovery from fulminant hepatic failure with subarachnoid hemorrhage by living donor liver transplantation: a case report.

A 29-year-old man was referred to our hospital with fulminant hepatic failure (FHF) and stage III hepatic coma (somnolence and confusion). Living donor liver transplantation (LDLT) was planned for 2 days after admission to our hospital. However, on the day after admission, he lapsed into stage IV hepatic coma: no right reflexes and no response to pain stimuli. Emergency cranial computed tomography revealed a subarachnoid hemorrhage (SAH), but no aneurysm was seen on magnetic resonance angiography. We speculated that the cause of the SAH may have been bleeding of intracranial veins secondary to coagulopathy and overextension of a vein due to brain edema. We considered that only LDLT could improve the coagulopathy and brain edema. The patient recovered consciousness on postoperative day (POD) 2 and was finally discharged from the hospital without neurological deficit on POD 85. This case suggested that SAH is not a prohibiting factor for LDLT in an FHF patient if the cause of the SAH is venous bleeding.

Neonatal hepatitis with hepatofugal portal flow and collateral veins: report of three cases.

Neonatal hepatitis, a syndrome occurring in children, has various etiologies, such as viral infection, unidentified disorders of bile salt synthesis, and other poorly understood metabolic diseases. It is characterized by jaundice, giant cell hepatitis, and, rarely, liver failure necessitating liver transplantation. We experienced 3 cases of idiopathic neonatal hepatitis with unusual progressive fibrosis presenting with retrograde portal flow and portal-systemic shunt. Clinical manifestations were hyperammonemia, hyperbilirubinemia, and coagulopathy. Characteristic histological findings were giant cell transformation of hepatocytes and progressive severe fibrosis. Two patients underwent living donor liver transplantation. We consider that liver transplantation is indicated in cases of neonatal hepatitis with hepatofugal portal flow and collateral vein formation.

A case of successful resection after long-term medical treatment of invasive pulmonary aspergillosis following living donor liver transplantation.

Invasive pulmonary aspergillosis (IPA) occurs in 1.5 to 10% of liver transplant recipients. Of the fungal infections, IPA is the most difficult to treat and the most frequently life-threatening. However, the best treatment strategy remains controversial. The patient was a 53-year-old woman who underwent living donor liver transplantation (LDLT) because of subacute fulminant hepatic failure due to autoimmune hepatitis. Aspergillus fumigatus was detected in the sputum taken intraoperatively by bronchial suction. A computed tomogram of the lung 7 days after LDLT showed fungal balls in the left lung. IPA was diagnosed. Since the patient suffered from pulmonary edema postoperatively and fungal balls occupied a greater part of the left lung, conservative therapy using micafungin, amphotericin B, and itraconazole was first selected. However, the fungus balls did not completely disappear. Moreover, brain abscess probably resulting from IPA dissemination was detected. Lung resection was performed as reduction surgery, and salvage treatment using voriconazole was done for a brain abscess. Septate hyphae of Aspergillus fumigatus were identified in the lung specimen. We concluded that for patients with IPA after LDLT, pulmonary resection should be done as soon as possible before deterioration of IPA and complication due to acute cellular rejection.

Delayed bile leakage from a remaining part of segment 8 in a posterior section graft after living donor liver transplantation: a common pitfall in harvesting a posterior section graft? A case report.

In Japan and Korea, where availability of deceased donor organs for solid organ transplantation remains rare, living donor liver transplantation (LDLT) using a posterior section graft (PSG; segments VI+VII, according to Couinaud's Nomenclature for liver segmentation) has now been accepted as a standard procedure that balances donor risk and patient benefits for cases in which right hemi-liver donation is too risky, because of marked volume imbalances between right and left hemi-livers. Compared with other types of grafts, however, the procedure requires detailed knowledge concerning hepatic vascular anatomy and meticulous manipulation during donation surgery. We present herein a case of delayed bile leakage from a remaining part of segment 8 in a PSG, which was considered to be a complication peculiar to LDLT using a PSG.

Efficacy of living donor liver transplantation for patients with methylmalonic acidemia.

Application of liver transplantation to methylmalonic acidemia (MMAemia) is controversial because MMAemia is caused by a systemic defect of methylmalonyl-CoA mutase. The clinical courses of seven pediatric patients with MMAemia undergoing living donor liver transplantation (LDLT) were reviewed. Serum and urinary methylmalonic acid (MMA) levels were found to be significantly decreased after LDLT, whereas serum and urinary MMA levels did not return to normal in any patient. One patient died of sepsis 44 days after LDLT. The other six patients are currently doing well. All patients had preoperative history of acute metabolic decompensation and/or metabolic stroke. However, no episode of acute metabolic decompensation or metabolic stroke was observed postoperatively in any surviving patients. In the preoperative period, all patients showed lethargy and cognitive deficit, both of which were eradicated after LDLT in all surviving patients. Preoperatively, all patients were subjected to dietary protein intake restriction and tube feeding, and were administered several metabolism-correcting medications. The metabolism-correcting medications being administered remained mostly unchanged after LDLT, whereas protein restriction was liberalized and tube feeding became unnecessary in all surviving patients. In addition, physical and neurodevelopmental growth delay remained in all surviving patients during the observation period, which ranged from 4 to 21 months with a median of 10.5 months.

Long-term outcome of adult-to-adult living donor liver transplantation for post-Kasai biliary atresia.

Our objective was to analyze problems in the perioperative management and long-term outcome of living donor liver transplantation (LDLT) for biliary atresia (BA). Many reports have described the effectiveness of liver transplantation (LT) for BA, particularly in pediatric cases, but little information is available regarding LT in adults (> or =16 years old). Between June 1990 and December 2004, 464 patients with BA underwent LDLT at Kyoto University Hospital, of whom 47 (10.1%) were older than 16 years. In this study, we compared the outcomes between adult (> or =16 years old) and pediatric (<16 years old) patients. The incidence of post-transplant intestinal perforation, intra-abdominal bleeding necessitating repeat laparotomy and biliary leakage was significantly higher (p < 0.0001, <0.001 and <0.001, respectively) in adults. Overall cumulative 1-, 5- and 10-year survival rates in pediatric patients were significantly higher (p < 0.005) than in adults. Two independent prognostic determinants of survival were identified: a MELD score over 20 and post-transplant complications requiring repeat laparotomy. Outcome of LDLT in adult BA patients was poorer than in pediatric patients. It seems likely that LT will be the radical treatment of choice for BA and that LDLT should be considered proactively at the earliest possible stage.

Usefulness of the prognostic score for donor safety in living donor liver transplantation.

This study sought to determine whether a prognostic score is a useful indicator of donor safety using 13 consecutive donors enrolled for liver transplantation. The donor operations were right hepatic lobectomies (n = 10) and left hepatic lobectomies (n = 3). The postoperative maximal level of serum total bilirubin was used to assess the magnitude of surgical stress. Variables such as donor age, percentage of liver resection (PLR), indocyanine green 15-minute retention rate (ICGR15), operative blood loss, operation time, prognostic score and graft weight were evaluated as predictors of the magnitude of surgical stress. The PLR and prognostic score (PS) were calculated according to the following formulae: PLR (%) = 100*Graft weight (g)/standard liver volume of the donor (mL); PS = -84.6 + 0.933*PLR (%) +1.11*ICGR15 (%) +0.999*age (years); Standard liver volume (mL) = 706.2*body surface area (m2) + 2.39. No serious complications occurred after the donor operations. Maximal bilirubin ranged from 1.9 to 10.9 mg/dL. There were no mortalities, although there were two morbidities, bile leakage and prolonged liver dysfunction. Postoperative hyperbilirubinemia was observed in two donors and in one Gilbert's syndrome donor. Linear regression analysis of each variable indicated poor correlations between those variables and maximal bilirubin. However, close correlations were seen between maximal bilirubin and both donor age and PS except for the three patients who showed postoperative hyperbilirubinemia. In these uneventful donors, statistical formulae were obtained as follows: maximal bilirubin (PMB) = 0.271 + 0.056*donor age (correlation coefficient 0.612, P < .008), PMB = 1.541 + 0.059*PS (correlation coefficient 0.597, P < .009). In conclusion, PS is useful to predict maximal bilirubin and to ensure donor safety.

Indications for hepatic vein reconstruction in living donor liver transplantation of right liver grafts.

BACKGROUND: To overcome problems arising from a graft of insufficient size, right liver grafts have been used extensively for adult-to-adult living donor liver transplantation (LDLT). However, there are reports of severe congestion in the anterior segment of the graft after transplantation. CASE REPORTS: Right liver transplantation without the middle hepatic vein was performed in six cases. In the second and third cases, the inferior right hepatic vein was reconstructed because it was thick (whereas the middle hepatic vein was not). Abdominal CT revealed congestive infarction of the anterior segment in the second case and of the posterior segment in the third. It was suspected that the former resulted from the lack of an middle hepatic vein, and the latter from obstruction of the reconstructed inferior right hepatic vein. Both patients died without improvement in liver function. Accordingly, in the fifth case, the middle hepatic vein was reconstructed. The postoperative course of this case was uneventful. Doppler ultrasonography showed profuse blood flow in the interposition graft. In the sixth case, the middle hepatic vein was not reconstructed because of technical difficulties. Although abdominal CT showed a congestive area in the anterior segment, the patient recovered uneventfully, probably because the volume of functional graft was sufficient even without the congestive area. CONCLUSION: When the color becomes dark in more than half of the surface of the anterior segment following clamping of middle hepatic vein tributaries and the hepatic artery, the middle hepatic vein should be reconstructed. When the diameter of the inferior right hepatic vein is more than 5 mm, its reconstruction is also recommended.

Surveillance of perioperative infections after adult living donor liver transplantation.

AIM: This study was conducted to clarify the management of perioperative infectious complications after adult living donor liver transplantation (LDLT). PATIENTS AND METHODS: Fourteen adult LDLT patients were enrolled in this study. We examined the occurrence of infectious complications in these cases and the relationships of infectious complications to UNOS status and MELD score. Surveillance culture and immunoserologic analyses were performed. From the results of these analyses, we made a diagram of infection surveillance using a matrix of time and sampling site. Using the diagram, we chose sensitive antibiotics as soon as possible. RESULTS: The infection site and its pathogen were able to be detected in four (28.5%) patients, all of whom had MRSA infections, together with lung aspergillosis in one case, pseudomonas pneumonia in another, and both in another. Two patients died of lung aspergillosis. Bacteria detected in the airway tended to spread to other sites during the postoperative period. In all four patients in whom infectious diseases were detected, and in a fifth patient in whom the site of infection was not known, the UNOS status was 1. The MELD score was calculated in eight patients, six of whom had high MELD scores (>20). CONCLUSION: Most cases were manageable by choosing and changing antibiotics and antifungal drugs according to the results of surveillance cultures twice a week. However, aspergillosis had an extremely poor prognosis. Patients with a high MELD score or low UNOS status, or both, showed poor prognosis; and in them, multiple drug resistance bacteria caused severe perioperative infectious complications.

Usefulness of artificial liver support for pretransplant patients with fulminant hepatic failure.

This study assessed the usefulness of artificial liver support (ALS) for pretransplant patients with fulminant hepatic failure (FHF). Five patients (age 14 to 52 years, 3 men and 2 women) with FHF who were being prepared for living donor liver transplantation (LDLTx) were enrolled in this study. ALS included plasma exchange, using 40 to 50 units of fresh frozen plasma per session, and high-flow hemodiafiltration, using a high-performance polysulfone membrane. Variables such as circulatory and respiratory function, coma grade, and neurologic disorders were evaluated. Although systolic and diastolic blood pressures showed no statistical differences between pre-ALS and post-ALS, the difference in heart rates was statistically significant. After ALS initiation in the pre-LDLTx period, one of the three patients who needed mechanical ventilation was weaned from it. After LDLTx, all patients recovered neurologically; no neurologic disorder was observed. These results suggested that ALS could predict neurologic status after LDLTx. The difference in coma grades also achieved statistical significance. Our study indicates that short-term ALS is useful for improving circulatory and respiratory function prior to liver transplantation, as well as for predicting posttransplantation neurologic status. Although some patients recover by ALS alone, the survival rate of ALS-only patients is less than 50%. ALS prolongs intensive treatment, thus increasing both the risk of infection and the medical costs. Further investigation to determine a precise marker for liver regeneration will be needed to establish a consensus on the indications for long-term ALS. We conclude that ALS is useful to improve circulatory and respiratory functions among pretransplant patients, and to predict neurologic status after LDLTx.

Remaining caudate lobe in the right lobe graft in living donor liver transplantation: a blind spot?

The right margin of the caudate lobe is obscure. Therefore, a part of the caudate lobe (a part of the right side of the paracaval portion) seems almost always to remain with the right lobe graft during the standard harvesting procedure. We reviewed the intraoperative findings and the postoperative courses of donors and recipients of 11 consecutive living donor liver transplantations using right lobe grafts. Further, we used computed tomography during the postoperative course to investigate whether the remaining caudate lobe was present in the right lobe graft and whether it produced serious complications. Four recipients displayed an intraoperative bile leak from a remaining part of the caudate lobe after the completion of biliary reconstruction. With the exception of one case who developed repeated bile leakage from the same origin which eventually healed during a long-term postoperative course, Most recipients showed no postoperative biliary complications. Although a remaining caudate lobe was detected on postoperative computed tomography in all recipients, it produced no serious complications. In conclusion, a part of the right side of the paracaval portion of the caudate lobe almost always remains with a right lobe graft during the standard harvesting procedure. However, the implications of this phenomenon seem to be benign.

[Indications for combined resection and reconstruction of the hepatic artery in biliary tract carcinoma].

More than 10 years have passed since hepatic artery resection was first performed for the treatment of biliary tract cancer. The safety of this procedure has been established with the introduction of the microsurgery technique. However, the benefits of and indications for this treatment have not yet been clarified. Twenty-three patients underwent vascular resection (portal vein in 7, portal vein + hepatic artery in 9, hepatic artery in 7) among 114 resected patients with biliary tract cancer in our institution. The right hepatic artery was reconstructed by end-to-end anastomosis in most cases. The curative resection rate was 88.9% in hilar bile duct cancer. However, it was less than 50% in other carcinomas. Cumulative 5-year survival rates of vascular resection patients with hilar bile duct cancer, lower bile duct cancer, gallbladder cancer, and cholangiocarcinoma were 14.8%, 25%, 0%, and 0%, respectively. On the other hand, the rates were 38.9%, 0%, 0%, and 0%, in the stage III + IV patients who did not undergo vascular resection. The longest survival period among patients with hilar bile duct cancer and lower bile duct cancer was 85 months and 65 months, respectively, whereas it was 15 months in gallbladder cancer and 20 months in cholangiocarcinoma patients. No hilar bile duct cancer patient who survived for more than 3 years had lymph node metastasis. The longest surviving cholangiocarcinoma patient has received adjuvant chemotherapy consisting of 5-fluorouracil and cisplatin. It is concluded that patients with hilar bile duct cancer are good candidates for vascular resection. Adjuvant chemotherapy should be administered to gallbladder cancer and cholangiocarcinoma patients, because vascular resection alone does not result in prolongation of life in these patients.

Aesthetic and plastic surgery for trichorhinophalangeal syndrome.

Trichorhinophalangeal syndrome is an autosomal dominant disorder characterized by a number of clinical features including short stature, sparse scalp hair, a pear-shaped bulbous nose, upper lip deformity, protruding ears, mandibular hypoplasia, and cone-shaped epiphyses of the phalanges. The syndrome has three subgroups: types I, II, and III. Although a few authors have pointed out the importance of aesthetic and plastic surgery in this syndrome, it has attracted relatively little attention. This review of the literature indicates that many patients have had various surgical corrections for associated abnormalities, including otoplasty and rhinoplasty. Unlike aesthetic or plastic corrections in other well-known congenital disorders, most corrections in trichorhinophalangeal syndrome have been performed prior to a diagnosis of the syndrome. Accurate identification of this syndrome is important to provide appropriate aesthetic treatments. Careful evaluation of patients is required when they present for surgery or aesthetic counseling, because they may have a variety of occult associations such as recurrent respiratory tract infections and urogenital anomalies.