Walid Faraj

Liver transplantation for hepatoblastoma

From October 1993 to February 2007, 25 liver transplantations were performed for hepatoblastoma. Of these 25, 18 children received cadaveric grafts, and 7 received left lateral segments from living donors. Fifteen patients were at level IV in the pretreatment extent of disease staging system for hepatoblastoma (PRETEXT IV; 11 received cadaveric grafts and 4 underwent living related liver transplantation [LRLT]) and 10 were level III (PRETEXT III; 7 received cadaveric grafts and 3 underwent LRLT). Preoperative chemotherapy was given according to the risk stratification system for children with hepatoblastoma protocols of the International Childhood Liver Tumour Strategy Group of the International Society of Paediatric Oncology (SIOPEL): SIOPEL I in the first 3 patients, SIOPEL II in 6, SIOPEL III in 10, and SIOPEL IV in 3 patients. Patient and graft survival after cadaveric transplantation was 91%, 77.6%, and 77.6%, at 1, 5, and 10 years, respectively, with no retransplantations. Patient and graft survival for children undergoing LRLT was 100%, 83.3%, and 83.3%, at 1, 5, and 10 years, respectively. All surviving children but 1 remain disease‐free, with a median follow up of 6.8 years (range, 0.9‐14.9). There were 5 deaths at a median of 13 months post‐transplantation, secondary to tumor recurrence (4) and respiratory failure (1). Liver transplantation is an established treatment for unresectable hepatoblastoma confined to the liver following chemotherapy. LRLT is a therapeutic option given that the outcome is similar to that of resection and cadaveric transplantation. Liver Transpl 14:1614–1619, 2008.

Auxiliary Liver Transplantation for Acute Liver Failure in Children

Objective:The aim of this study is to present the largest experience of auxiliary liver transplantation for acute liver failure (ALF) in children over the past 19 years. Methods:Between 1990 and 2009, a total of 128 liver transplants were performed on children with ALF. Of these, 20 received auxiliary liver transplants (19 were cadaveric and 1 living graft). The recipient median age was 12 years (range: 1 –16). Indications for auxiliary partial orthotopic liver transplantation were seronegative non-A non-B hepatitis in 16 children, drug induced in 2, and 1 autoimmune hepatitis and 1 mushroom poisoning. The median waiting time for transplantation was 2 days (range: 1–9). After native liver partial hepatectomy, 20 grafts were implanted orthotopically and included 8 right lobes, 8 left lateral segments, 3 left lobes, and 1 whole liver. Regeneration of the native liver was assessed by radiologic, nuclear medicine imaging, and histology. Follow-up imaging and biopsies were performed at intervals of 3 to 6 months and yearly. Results:Patient survival was 85% at 1, 5, and 10 years. There were 3 deaths at a median of 9 days (range: 8–52) post-transplantation. There was 1 retransplant for chronic rejection 15 months post-transplantation. There were no biliary or vascular complications. Of 17 survivors, 14 (82%) have successfully regenerated their native liver and so far 11 children (65% of the survivors) have been withdrawn from immunosuppression at a median time of 23 months (range: 4–106) after transplantation. Conclusion:Auxiliary partial orthotopic liver transplantation should be considered in children presenting with ALF who fulfill criteria for liver transplantation.

Update on totally implantable venous access devices

The use of totally implantable venous devices (TIVAD) has changed the care and quality of life for cancer patients, these devices allow chemotherapy administration, and blood sampling without the need for repeated venipuncture. These ports are used mainly when IV access is needed only intermittently over a long period of time. We are presenting a brief overview on TIVADs, with focus on the mid and long-term complications associated with these devices with their management.

Outcome of liver transplantation in hereditary hemochromatosis

Hereditary hemochromatosis (HH) is a genetic disorder of iron metabolism. It is an uncommon indication for liver transplantation (LT). It has been suggested that patients who undergo LT for cirrhosis related to HH have higher morbidity and mortality from cardiac, infectious and malignant complications. The purpose of this retrospective review was to determine whether these observations hold true in the current era. We analysed the data of 22 patients who had LT for HH from 1996 to 2007 at our center. Thirteen patients had LT for complications of end‐stage liver disease, seven for hepatocellular carcinoma (HCC) and two for subacute liver failure. Cofactors promoting liver disease were identified in 15 patients. Ten patients had iron reduction with venesection before transplantation. Patient and graft survival at 1 and 5 years were 80.7%, and 74% respectively. There were seven deaths after a median follow up of 46 months either because of multiorgan failure, or caused by HCC recurrence. Bacterial infections were the commonest cause of morbidity. Patients with HH remain at a higher risk of developing HCC. Infectious complications are common. Iron reduction with preoperative venesection reduces the risk of cardiac and infection complications postoperatively. Improved survival post‐LT reflects changes in selection, disease modification through venesection, and improvement in immunosuppression.

Inflammatory pseudo-tumor of the liver: a rare pathological entity.

Inflammatory pseudo-tumor (IPT) of the liver is a rare benign neoplasm and is often mistaken as a malignant entity. Few cases have been reported in the literature and the precise etiology of inflammatory pseudotumor remains unknown. Patients usually present with fever, abdominal pain and jaundice. The proliferation of spindled myofibroblast cells mixed with variable amounts of reactive inflammatory cells is characteristics of IPT. We reviewed the literature regarding possible etiology for IPT with a possible suggested etiology.

Living related liver transplantation in children

Living related liver transplantation (LRLT) has become established for treating children with end‐stage liver disease. The aim of this study was to review a single‐centre experience of left lateral segment liver transplants from living donors in children.

Primary undifferentiated embryonal sarcoma of the liver mistaken for hydatid disease

Primary undifferentiated embryonal sarcoma of the liver is a rare tumor with a peak incidence between the ages of 6 and 10 years. We report a case of a primary hepatic undifferentiated embryonal sarcoma arising in a 21-year-old male mistaken for hydatid disease of the liver. The rapid recurrence of this tumor along the site of attempted percutaneous drainage illustrates some important management points regarding this malignancy.

Postoperative outcomes after laparoscopic splenectomy compared with open splenectomy.

Objective:To evaluate 30-day postoperative outcomes in laparoscopic (LS) versus open splenectomy (OS). Summary Background Data:LS has generally been associated with lower rates of postoperative complications than OS. However, evidence mainly comes from small studies that failed to adjust for the confounding effects of the underlying indication or clinical condition that may have favored the use of one technique over the other. Methods:A retrospective cohort study of patients undergoing splenectomy in 2008 and 2009 using data from the American College of Surgeons National Surgical Quality Improvement Program database (n = 1781). Retrieved data included 30-day mortality and morbidity (cardiac, respiratory, central nervous system, renal, wound, sepsis, venous thromboembolism, and major bleeding outcomes), demographics, indication, and preoperative risk factors. We used multivariate logistic regression to assess the adjusted effect of the splenectomy technique on outcomes. Results:A total of 874 (49.1%) cases had LS and 907 (50.9%) had OS. After adjusting for all potential confounders including the indication and preoperative risk factors, LS was associated with decreased 30-day mortality [OR (odds ratio): 0.39, 95% CI: 0.18–0.84] and postoperative respiratory occurrences (OR: 0.46, 95% CI: 0.27–0.76), wound occurrences (OR: 0.37, 95% CI: 0.11–0.79), and sepsis (OR: 0.52, 95% CI: 0.26–0.89) when compared with OS. Patients who underwent LS also had a significantly shorter total length of hospital stay and were less likely to receive intraoperative transfusions compared with patients who underwent OS. Conclusions:LS is associated with more favorable postoperative outcomes than OS, irrespective of the indication for splenectomy or the patients clinical status.

Unusually young age distribution of primary hepatic leiomyosarcoma: case series and review of the adult literature

BackgroundPrimary hepatic leiomyosarcoma is a rare disease diagnosed in older aged adults with a median age of 58 and occasionally in children with a history of immunosuppression.MethodsFrom 1998 to 2009, 215 patients were diagnosed with primary hepatic malignancies at our institution, 4 of which were diagnosed with primary hepatic sarcoma (1.8%). Three cases were primary hepatic leiomyosarcomas (LMS) and one case was primary undifferentiated embryonal sarcoma of the liver; median age 30 (range 20-39) years.ResultsOne patient is currently 12 months post-resection with no evidence of recurrence. Two patients passed away at 19 days and 22 months from small for size liver and tumor recurrence respectively.ConclusionWe have presented 3 cases of primary hepatic leiomyosarcoma diagnosed at our institution with an unusually young age distribution and no evidence of immunosuppression. These cases highlight the diagnostic and therapeutic challenges of this rare tumour.

Laparoscopic approach to symptomatic meckel diverticulum in adults.

Background: Meckel diverticulum (MD) is the most common congenital gastrointestinal malformation found in approximately 2% of the general population. MD manifests in adults as gastrointestinal bleeding, bowel obstruction, intussusception, or perforation in <5% of cases. There is no consensus on the ideal management strategy in symptomatic MD. Therefore, we searched the literature to highlight the role of laparoscopy in diagnosing and treating symptomatic MD. Method: We used PubMed, Medline, Google Scholar, Ovid, and Cochrane data search engines looking for articles containing terms such as Meckel diverticulum, ectopic gastric mucosa, laparoscopic, technetium 99m pertechnetate, and acute management. We included articles reporting on case series in the English language on adult patients only and reporting on laparoscopic approach in the management of symptomatic MD. Results: A total of 5 articles reporting on 35 cases were found. We report on the pooled data from these series with emphasis on number of patients, age, male to female ratio, length of stay, conversion to open procedure, method of resection, complications, first presentation, and confirmation of diagnosis preoperatively. We also compare the results of laparoscopic versus open approaches mentioned in the literature. Conclusions: The laparoscopic approach was found to be effective as a diagnostic and therapeutic modality in patients with symptomatic MD; however, its adoption as the gold standard still needs further studies with larger patient numbers.