Living Donor-Initiated Domino Split-Liver Transplantation in Pediatric Setup: A Case Report With Literature Review.

BACKGROUND: In patients undergoing liver transplantation for metabolic diseases, removing the patient's liver for transplantation to another recipient is called "domino liver transplantation." The extracted liver can be divided and transplanted into 2 recipients, which is called domino split-liver transplantation in the literature. However, in our study, the domino liver was obtained from a pediatric patient. METHODS: A patient with maple syrup urine disease (MSUD) underwent a living donor liver transplant, and the explanted liver was divided in situ into right and left lobes and transplanted to 2 separate patients. Demographic data, surgical techniques, postoperative period, and patient follow-ups were evaluated. RESULTS: The father's left lobe liver graft was transplanted into a 12-year-old boy with MSUD. The removed liver was divided in situ into right and left lobes. The left lobe was transplanted to a 14-year-old male patient, whereas the right lobe was transplanted to a 67-year-old male patient. The donor and the first recipient were discharged on postoperative days 5 and 22. The second pediatric patient who underwent domino split-left lobe transplantation was discharged on postoperative day 23. The adult patient who underwent domino split-right lobe transplantation died on postoperative day 12 owing to massive esophageal variceal bleeding. CONCLUSION: Patients who underwent liver transplantation due to MSUD are among the best donor choices for domino liver transplantation. If the extracted liver has a sufficient volume and anatomic features for a split, it can be used in "selected cases."

Complementary Roles of Cadaveric and Living Donor Liver Transplantation in Acute Liver Failure.

BACKGROUND: Living donor liver transplantation may complement cadaveric transplantation in acute liver failure (ALF) patients. METHODS: Between 2008 and 2017, 89 patients were treated for ALF; 15 patients (17%) recovered with intensive care treatment; 31 (35%) died without transplant. The records of the remaining 43 patients (median (range) age: 14 (1-62)) who underwent transplantation were evaluated. RESULTS: The etiologic factors were toxic agents (10; mushrooms: 8; herbs: 2), hepatitis viruses (7; A: 1; B: 6), Wilson's disease (7), autoimmune hepatitis (4), and Budd-Chiari syndrome (2); 13 cases were idiopathic. Cadaveric organs (whole, split, reduced) were transplanted to 32 patients; 11 patients underwent living donor transplantation. One patient (2%) died of septic shock on the second postoperative day. Bacterial infection was the most common early (< 3 months) complication in the remaining patients (31/42; 74%), followed by delirium (5/42; 12%) and acute rejection requiring steroid pulse (5/42; 12%). Seven other patients died during median (range) follow-up of 94 (14-142) months: various infections (5), leukemia (1), and acute myocardial infarction (1). The 1-, 5-, and 10-year survival rates were 100%, 96%, and 92% in children and 94%, 82%, and 65% in adults respectively. CONCLUSIONS: Cadaveric organ sharing and transplantation from living donors when appropriate yield a high survival rate, despite high early morbidity, in ALF patients whose conditions deteriorate despite intensive care treatment. Efforts to eliminate preventable causes of acute liver failure will lead to more efficient use of health care resources.

Transplantation for Large Liver Tumors in the Setting of Abernethy Malformation.

In this study, we report our experiences on the role of transplantation in 2 patients with large liver tumors in the setting of Abernethy malformation. Patient 1 was a 17-year-old boy who was referred for hepatic masses and recurrent hepatic encephalopathy episodes. Computed tomography and magnetic resonance imaging showed 2 large tumors (4 and 8 cm) in the liver. The portal vein drained directly into the vena cava. Core biopsy of the larger mass revealed fibrosis and regenerative hyperplasia. There were hyperintense signals in the T1-weighted images in the globus pallidus. The Stanford-Binet intelligence scale showed moderate mental retardation (IQ 39); however, the patient showed good ability for caring for himself. His cognitive defect was ascribed partially to chronic encephalopathy. The patient received a right hepatic lobe from his older brother. The congenital portacaval shunt was disconnected to provide inflow to the graft. Pathologic examination of the explanted liver revealed no evidence of malignancy. His IQ improved to 75 at 29 months posttransplant. The hyperintensity of the globus pallidus on magnetic resonance imaging disappeared. The patient has maintained a normal life during 9 years of follow-up. Patient 2 was a 17-year-old girl who was referred for multiple hepatic masses; she had no symptoms at admission. Magnetic resonance imaging showed type 1 Abernethy malformation and multiple hepatic masses (largest was 10 cm), which appeared to be hyperplastic lesions. Because malignancy could not be definitely excluded, she received a right lobe without the middle hepatic vein from her uncle. Pathologic examination of the explanted liver showed localized nodular hyperplasia; there was no evidence of malignancy. She has maintained normal life activities during 3 years of follow-up. Liver transplant is a curative treatment option for patients with large liver tumors, replacing the hepatic parenchyma in the setting of Abernethy malformation.

Prevention and acute management of biliary injuries during laparoscopic cholecystectomy: Expert consensus statement.

Gallstone disease is very common and laparoscopic cholecystectomy is one of the most common surgical procedures all over the world. Parallel to the increase in the number of laparoscopic cholecystectomies, bile duct injuries also increased. The reported incidence of bile duct injuries ranges from 0.3% to 1.4%. Many of the bile duct injuries during laparoscopic cholecystectomy are not due to inexperience, but are the result of basic technical failures and misinterpretations. A working group of expert hepatopancreatobiliary surgeons, an endoscopist, and a specialist of forensic medicine study searched and analyzed the publications on safe cholecystectomy and biliary injuries complicating laparoscopic cholecystectomy under the organization of Turkish Hepatopancreatobiliary Surgery Association. After a series of e-mail communications and two conferences, the expert panel developed consensus statements for safe cholecystectomy, management of biliary injuries and medicolegal issues. The panel concluded that iatrogenic biliary injury is an overwhelming complication of laparoscopic cholecystectomy and an important issue in malpractice claims. Misidentification of the biliary system is the major cause of biliary injuries. To avoid this, the "critical view of safety" technique should be employed in all the cases. If biliary injury is identified intraoperatively, reconstruction should only be performed by experienced hepatobiliary surgeons. In the postoperative period, any deviation from the expected clinical course of recovery should alert the surgeon about the possibility of biliary injury.

A rare complication following laparoscopic Roux & Y gastric bypass: intussusception-case report.

Obesity is a growing health problem in most parts of the world. Currently only proven long term effective treatment of obesity is bariatric surgery. Roux & Y gastric bypass together with sleeve gastrectomy are the most employed surgical techniques with acceptable metabolic and surgical complication rates. In this paper we would like to present an unexpected complication of Roux & Y gastric bypass: a retrograde intussusception located in the common limb 17 months after the surgery. As intussusception in adults usually originates from a leading point, there is no such an explanation following Roux & Y gastric bypass.

Hepatic hydatid disease requiring urgent treatment during pregnancy.

BACKGROUND: Pregnant women may experience an acute presentation of hepatic hydatid disease. The available literature is limited to case reports. METHODS: The charts of 7 patients who underwent urgent treatment for hepatic hydatid disease during pregnancy between 1992 and 2010 were reviewed. RESULTS: The median patient age was 27 (range 23-39) years and median gestational age was 18 (range 13-24) weeks. The symptoms were severe abdominal pain (4), vomiting (2), jaundice (2), pruritus (2) and severe dyspepsia (1); in the asymptomatic patient, a closed intraperitoneal rupture had been detected during gynecologic ultrasonography. Surgical drainage of the cysts was performed in all cases. The two patients with frank biliary rupture underwent choledochoduodenostomy or Roux-Y hepaticojejunostomy. Four patients required postoperative tocolysis. Albendazole was not used. All mothers gave birth to healthy babies at term. The patients were followed for a median of 9 (range 4-19) years. Two patients developed recurrences at 2 and 7 years; these were treated with surgical drainage and albendazole. CONCLUSION: This entity entails the responsibility of two human beings. Although it imposes limitations on the routine diagnostic and therapeutic options due to risk of premature labor or teratogenicity, acceptable results can be obtained in collaboration with the department of obstetrics and gynecology.

Intrahepatic biliary cystic neoplasms: Surgical results of 9 patients and literature review.

AIM: To investigate the eligible management of the cystic neoplasms of the liver. METHODS: The charts of 9 patients who underwent surgery for intrahepatic biliary cystic liver neoplasms between 2003 and 2008 were reviewed retrospectively. Informed consent was obtained from the patients and approval was obtained from the designated review board of the institution. RESULTS: All patients were female with a median (range) age of 49 (27-60 years). The most frequent symptom was abdominal pain in 6 of the patients. Four patients had undergone previous laparotomy (with other diagnoses) which resulted in incomplete surgery or recurrences. Liver resection (n = 6) or enucleation (n = 3) was performed. The final diagnosis was intrahepatic biliary cystadenoma in 8 patients and cystadenocarcinoma in 1 patient. All symptoms resolved after surgery. There has been no recurrence during a median (range) 31 (7-72) mo of follow up. CONCLUSION: In spite of the improvement in imaging modalities and increasing recognition of biliary cystadenoma and cystadenocarcinoma, accurate preoperative diagnosis may be difficult. Complete surgical removal (liver resection or enucleation) of these lesions yields satisfying long-term results.

Recurrent anaphylaxis due to non-ruptured hepatic hydatid cysts.

BACKGROUND: Hydatid disease, a parasitic infestation of humans, is endemic in the Mediterranean region, Australia, New Zealand and the Middle East, and mostly involves the liver. Anaphylactic reactions, which sometimes are the first manifestations of the disease, frequently occur due to cyst rupture after a minor/major trauma, though they may also be spontaneously seen on rare occasions. In extremely few studies, anaphylactic shock has been reported in patients without macroscopic rupture of the hydatid cysts. CASE REPORT: Our patient had recurrent anaphylactic episodes without any trauma and had been misdiagnosed for several years even though the patient was living in a region endemic for hydatid disease. CONCLUSION: We emphasize that physicians should be highly aware of hydatid disease as a possible etiology for seemingly idiopathic anaphylactic reactions, especially in endemic regions.

Somatostatin and propranolol to treat small-for-size syndrome that occurred despite splenic artery ligation.

We report our success with somatostatin and propranolol to treat small-for-size syndrome that occurred despite splenic artery ligation. A 48-year-old woman with cirrhosis due to autoimmune hepatitis underwent living-donor liver transplant; her graft-to-body weight ratio of the right lobe was 0.91%. After arterial reperfusion, portal pressure and flow were 24 cm H20 and 2.22 L/min (ie, 360 mL/100g graft/min), respectively. Following splenic artery ligation, the portal pressure decreased to 16 cm H20 and portal flow to 1.74 L/min (ie, 282 mL/100g graft/min). On the second postoperative day, small-for-size syndrome was diagnosed based on the marked prolongation of prothrombin time (international normalized ratio, 4.4), hyperbilirubinemia (359.1 micromol/L), rapid escalation of transaminases (alanine aminotransferase 2488 U/L, aspartate aminotransferase 1075 U/L) and very high portal flow rate (> 90 cm/sec). Oral propranolol (40 mg/day b.i.d.) and somatostatin infusion (250-microgram bolus followed by perfusion at a rate of 250 microgram/h for 5 days) were started. Prothrombin time and transaminase levels began to decrease the following day, although the bilirubin level increased to 495.9 micromol/L before returning to normal. The patient was discharged in excellent health 5 weeks after surgery. Despite reduction of portal pressure by splenic artery ligation, small-for-size syndrome may develop in patients with persistent high portal flow. To the best of our knowledge, this is the first report of the successful treatment of small-for-size syndrome by somatostatin and propranolol in the clinical setting.

Bile duct injury during cholecystectomy requiring delayed liver transplantation: a case report and literature review.

Major bile duct injury during cholecystectomy represents potentially severe complications with unpredictable long-term results. If these lesions are not treated adequately, they can lead to hepatic failure or secondary biliary cirrhosis therefore requiring liver transplantation. We report a patient who required liver transplantation 15 years after open cholecystectomy. A l0-year old girl underwent open cholecystectomy and duodenal repair for cholelithiasis and cholecystoduodenal fistula. She required two surgical interventions, hepaticojejunostomy which was performed in another center and portoenterostomy for biliary stricture at our institution seven years after the cholecystectomy. Eight years after the third operation, she required recurrent hospitalization for treatment of hepatic abscesses. The extremely short intervals between the three life threatening episodes and the rapid progression to severe sepsis were taken into consideration and liver transplantation was performed at the age of 25. She is leading a healthy life at 4 years post transplantation. Although iatrogenic biliary injury can usually be treated successfully by a combination of surgery, radiological and endoscopic techniques, patients with severe injuries develop irreversible liver disease. This case report and review of the literature suggest that liver transplantation is a treatment modality for a selected group of patients with end-stage liver disease secondary to bile duct injury.

Hepatic atrophy-hypertrophy complex due to Echinococcus granulosus.

Obstruction of a major hepatic vein, or major portal vein, or biliary tree branch causes atrophy of the related hepatic region, and frequently, hypertrophy in the remaining liver-the atrophy-hypertrophy complex (AHC). Whether hydatid cysts can cause AHC is controversial. The records of 370 patients who underwent surgery for hepatic hydatid disease between August 1993 and July 2002 were evaluated retrospectively. Excluding six patients with previous interventions on the liver, AHC had been recorded in the operative notes of 16 patients (4.4%); for all patients, a cyst located in the right hemiliver had caused atrophy of the right hemiliver and compensatory hypertrophy of the left hemiliver. The computed tomography images of seven patients were suitable for volumetric analysis. The median (range) right and left hemiliver volumes were 334 (0-686) ml and 1084 (663-1339) ml, respectively. The median (range) cyst volume was 392 (70-1363) ml. AHC due to Echinococcus granulosus was confirmed by objective volumetric analysis. The presence of AHC should alert the surgeon to two implications. First, pericystectomy may be hazardous due to association with major vascular and biliary structures. Second, in patients with AHC, the hepatoduodenal ligament rotates around its axis; this should be considered to avoid vascular injury if a common bile duct exploration is to be performed.

Endoscopic and radiologic interventions as the leading causes of severe cholangitis in a tertiary referral center.

BACKGROUND: Iatrogenic factors became the leading mechanisms of severe cholangitis in a referral center. PATIENTS AND METHODS: The records of the 58 patients treated for severe cholangitis between 1996 and May 2004 (inclusive) were evaluated. RESULTS: The most frequent underlying diseases were periampullary tumors and mid-bile duct carcinomas (22), followed by proximal cholangiocarcinomas (14). The triggering mechanism was an incomplete endoscopic retrograde cholangiopancreatography (ERCP) in 32 patients, incomplete or inappropriate percutaneous transhepatic biliary drainage (PTBD) in 6, apparently successful ERCP and stenting in 1, and percutaneous transhepatic cholangiography in 1. PTBD was the treatment of choice (38). Mortality was 29% (17/58); the major causes were refractory sepsis (8) and incomplete biliary drainage (advanced tumor, technical failure, or hemobilia) (8). CONCLUSIONS: In this series composed predominantly of patients referred after development of sepsis, ERCP and PTBD complications were the leading mechanisms of severe cholangitis. Nonoperative biliary manipulations are invasive procedures with potentially fatal complications. The decisions to perform such procedures and periprocedural management are responsibilities of an experienced multidisciplinary team.

Definitive treatment of traumatic biliary injuries.

BACKGROUND: We presented our experience with definitive treatment of traumatic biliary injuries. METHODS: Six male patients (mean age 13 years; range 2 to 32 years) who were referred to our unit for definitive treatment of traumatic biliary injuries were retrospectively evaluated. Data were analyzed in terms of demographic characteristics, mechanisms of injuries, associated injuries, previous treatments, symptoms on admission, treatment at our unit, and the results of treatment. Outcome was assessed using modified Schweiser and Blumgart criteria. RESULTS: The injuries were due to blunt abdominal trauma in all the patients but one who had a gunshot wound. In three patients, biliary injuries were missed at the initial operation. On admission, three patients had external biliary fistulas, two had biliary strictures. One patient was sent following inadvertent ligation of the hepatoduodenal ligament during attempts to control hemorrhage. Roux-en-Y hepaticojejunostomy was performed in three patients. Percutaneous biloma drainage was performed in two patients, resulting in fistula closure in 13 and 40 days, respectively. One patient was treated by endoscopic retrograde cholangiopancreatography and papillotomy, which enabled fistula closure in three days. One patient was lost to follow-up. One patient died from hepatic failure 11 years after the trauma. At the end of a mean follow-up of 49 months (range 15 to 75 months), three patients were in excellent condition, while one patient experienced occasional attacks of cholangitis. CONCLUSION: In patients with undetected biliary injuries and in those with unsuccessful repair attempts, biliary reconstruction should be performed in experienced hepatopancreatobiliary surgery units.

Alveolar echinococcosis in Turkey. Experience from an endemic region.

BACKGROUND: Radical resection is the only potentially curative treatment for hepatic alveolar echinococcosis (AE). Although Turkey is an endemic region, population screening is not performed and early diagnosis is rare. Consequently, surgeons are compelled to explore possibilities such as near-total resection and biliodigestive anastomosis for palliation of jaundice. METHODS: Surgery was performed in 32 patients with hepatic AE with the following indications: (1) resection; (2) palliation of jaundice; (3) definite assessment of operability; (4) failure in the management of cavity infection by percutaneous methods. Curative resection (R0 = complete resection of all parasitic mass [n = 9], and R1 = a resection in which a small remnant was left on a vital structure [n = 8]) were performed in 17 patients, intrahepatic cholangiojejunostomy in 7, laparotomy-external drainage in 7, and debulking in 1. RESULTS: Perioperative mortality rates were 2/17, 0/7, 2/7 and 1/1, respectively. Twelve patients in the curative resection group are alive without recurrence/progression of the small remnant during a median follow-up of 59 (range 27-116) months. One patient developed an inoperable recurrence that was treated with albendazole. One patient was lost to follow-up. Long-term albendazole treatment was effective in all R1 patients except a patient who had slow asymptomatic progression. Successful palliation of jaundice was achieved in 5 of the 7 intrahepatic cholangiojejunostomy patients. CONCLUSIONS: The results of R1 resection in alveolar hydatid disease are similar to those of R0 resection; a small remnant is successfully controlled by albendazole. In patients with jaundice due to hilar invasion, biliary diversion from segment 3 or 5 is effective for palliation of the jaundice and facilitates albendazole treatment.

The effect of concomitant vascular disruption in patients with iatrogenic biliary injuries.

BACKGROUND AND AIMS: To evaluate treatment results in iatrogenic biliary injuries with concomitant vascular injuries. PATIENTS/METHODS: Between January 1998 and May 2002 (inclusive), angiography was performed in 45 of the 105 patients treated for iatrogenic biliary tract injury. The charts of these 45 patients and 5 other patients in whom vascular injury was diagnosed at operation were evaluated retrospectively. Twenty-nine patients had concomitant vascular injury, the biliovascular injury group (BVI), and the remaining 21 patients had isolated biliary tract injury (IBTI). RESULTS: The most frequent initial operation was a cholecystectomy. The frequency of high-level (Bismuth III or IV) strictures was 90% in the BVI group and 62% in the IBTI group ( P<0.05). Perioperative mortality was 7% in the BVI group and 5% in the IBTI group ( P>0.05). The morbidity in the BVI group was significantly higher ( P<0.05). Two patients in each group were lost to follow up. During a median (range) follow up of 31 months (5-51 months), a successful functional outcome was achieved in 96% of the BVI group and 100% of the IBTI group with a multimodal approach ( P>0.05). CONCLUSIONS: The frequency of high-level biliary injury and morbidity were significantly higher in the BVI group. However, concomitant vascular injury had no significant effect on mortality and medium-term outcome of biliary reconstruction. Thus, routine preoperative angiography is not recommended.