Liver transplantation in an adolescent with acute liver failure from acute lymphoblastic leukemia.

The most common identifiable causes of acute liver failure in pediatric patients are infection, drug toxicity, metabolic disease, and autoimmune processes. In many cases, the etiology of acute liver failure cannot be determined. Acute leukemia is an extremely rare cause of acute liver failure, and liver transplantation has traditionally been contraindicated in this setting. We report a case of acute liver failure in a previously healthy 15-yr-old male from pre-B-cell acute lymphoblastic leukemia. He underwent liver transplantation before the diagnosis was established, and has subsequently received chemotherapy for pre-B-cell acute lymphoblastic leukemia. He is currently alive 31 months post-transplantation. The published literature describing acute lymphoblastic leukemia as a cause of acute liver failure is reviewed.

Vascularized composite allotransplantation at a crossroad.

Vascularized composite allotransplantation is a relatively young field that has shown significant growth in the past decade. The subspecialty offers opportunities that are not available with solid organ transplants. However, the field also faces significant hurdles in increasing clinical volumes. The development of innovative immune-reduction strategies will likely determine the pace and direction of growth in the field in the years to come.

Composite tissue allotransplantation: current challenges.

Composite tissue allotransplantation (CTA) in the clinic is taking firm root. Success at hand, face, knee, trachea, and laryngeal transplantation has led to widespread interest and increasing application. Despite this, skepticism is common, particularly in the realm of reconstructive surgeons. The risks of immunosuppression remain a barrier to the advancement of the field, as these are perceived by many to be prohibitive. Significant progress in the field require the development of newer immunosuppressive agents with less toxicity and methods to achieve donor specific tolerance. This review focuses on the current state of CTA-both in the clinic and the laboratory. A thorough understanding of the immunology of CTA will allow the widespread application of this promising field.

Laparoscopic liver surgery: a synopsis.

The complex vascular and biliary anatomy of the liver poses great challenges even to experienced surgeons. However, with the experience accrued over the past two decades, surgery of the liver has become standardized. In the past few years innovative surgical techniques have permitted liver surgery to be performed using the minimally invasive approach. Large clinical series have been reported which demonstrate the safety and oncological integrity of the approach. This review highlights the current state of laparoscopic liver surgery with emphasis on problems unique to the procedure.

Non-Hodgkin lymphoma presenting with obstructive jaundice.

BACKGROUND: Obstructive jaundice is a rare presenting feature of non-Hodgkin lymphoma (NHL). Lymphomatous masses in the peripancreatic and hepatic hilar regions are potentially difficult to diagnose. METHODS: A retrospective analysis was undertaken of patients presenting with obstructive jaundice secondary to NHL at a tertiary care hepatobiliary unit. RESULTS: Over a 7-year period, six adults and three children with NHL were managed. The site of the lymphomatous mass was at the hepatic hilum in six patients and the peripancreatic area in three. Diagnostic procedures included a laparotomy and biopsy in four patients, hepatic trisectionectomy in two, percutaneous biopsy in two and lymph node biopsy in one patient. Percutaneous biopsy confirmed the diagnosis in both the patients in whom it was attempted. One patient died following liver resection. Chemotherapy was the mainstay of treatment and achieved complete remission in four patients, partial remission in three and no response in one patient. Two patients subsequently required operation for a benign biliary stricture after achieving complete remission. CONCLUSION: NHL must be considered in the differential diagnosis of obstructive jaundice in adults and children. Attempts must be made to diagnose the condition using non-operative techniques. Chemotherapy is the mainstay of treatment. Late benign strictures of the bile duct requiring operation may develop.

Long-term survival after spontaneous rupture of a malignant rhabdoid tumor of the liver.

A 13-month-old girl presented with a large malignant rhabdoid liver tumor that ruptured soon after admission. Six years after an emergency right hepatectomy and subsequent chemotherapy (ifosfamide, vincristine, and actinomycin D), she remains well and disease free. Previously, these rare tumors invariably have been fatal and resistant to multimodal therapy. This is the first report of long-term survival of a patient with a malignant rhabdoid liver tumor.

Segment IV liver cyst with biliary communication following laparoscopic deroofing.

Simple cysts of the liver rarely have a biliary communication. We record the development of a biliary communication following laparoscopic deroofing of a segment IV simple cyst of liver and document its successful sclerosis with tetracycline.

Choledochoduodenostomy: influence of risk factors in post-operative morbidity.

BACKGROUND: Choledochoduodenostomy is performed for a variety of lower common bile duct lesions. AIMS: To analyse the influence of risk factors on the post-operative morbidity following choledochoduodenostomy. METHODS: Relation of risk factors including age more than 60 years, medical Illness, hemoglobin less than 10 g/dL, albumin less than 3 g/dL, bilirubin more than 10m/dL, presence of cholangitis at admission (treated pre-operatively), use of pre-operative endoscopic sphincterotomy and common bile duct diameter at surgery were related to the occurrence of post-operative morbidity was studied using univariate analysis. RESULTS: Fifty patients underwent choledochoduodenostomy. One patient (2%) died; major post-operative morbidity occurred in 12 patients (24%). Presence of cholangitis at admission was the only factor related (p = 0.00012) to the occurrence of post-operative morbidity. No long-term complications were encountered in 35 patients (70%) mean with followup period of 28 (range 8-60) months. CONCLUSIONS: Choledochoduodenostomy is a safe permanent drainage procedure for benign lower biliary obstruction.

Transcholedochal cholecystolithotomy for a variant of Mirizzi's syndrome.

Obstruction of the common bile duct by impaction of a gallstone in the neck or the cystic duct of the gallbladder results into repeated attacks of cholangitis (Mirizzi's syndrome). In suspected cases preoperative diagnosis can be made by ultrasonography and cholangiography. We report two patients of a variant of Mirizzi's syndrome. The common bile duct was obstructed by a stone impacted in the neck and causing extrinsic compression from behind. Ultrasonography and endoscopic retrograde cholangiography diagnosed the condition as common bile duct stone in both the cases. The anomaly could be diagnosed only after choledochotomy. Stones were retrieved by transcholedochal cholecystolithotomy--an innovative surgical procedure for this variant of Mirizzi's syndrome.