Radiological and surgical differences between congenital end-to-side (Abernethy malformation) and side-to-side portocaval shunts.

OBJECTIVE: Distinguishing retrohepatic end-to-side portocaval shunts (ES-PCS) and side-to-side portocaval shunts (SS-PCS) can be difficult, but it is essential for determining the treatment strategy. Our experience with retrohepatic PCS is analyzed. METHODS: Since 2007, 9 children (5/9 ES-PCS and 4/9 SS-PCS) were surgically treated. Radiology studies included Doppler-ultrasound, CT/MRI and angiography/occlusion test (8/9). RESULTS: CT in 5/5 ES-PCS revealed the portal vein (PV) entering the left side of the vena cava with a uniform shape. 4/4 SS-PCS showed aneurysmal PV containing the origin of the main intrahepatic portal branches (IHPB) entering the cava anterior aspect or slightly to the right with a variable length (from long to short/wide). ES-PCS anatomy showed caudate lobe absence with the fistula entering the left cava aspect free of parenchyma, but anterior through the caudate lobe in SS-PCS. With the angiography/occlusion test, the IHPB was undeveloped in ES-PCS (portal pressure > 38 mmHg) and hypoplasic in SS-PCS (portal pressure < 25 mmHg). ES-PCS treatment included: 1/5 hepatectomy and 4/5 definitive banding (one by laparoscopy); while in SS-PCS: 1/4 liver transplantation, 2/4 1-step closure (one by laparoscopy), and 1/4 definitive banding. CONCLUSION: Fistula shape, cava relationship, IHPB and portal pressures differ between the two shunt types. A question arises regarding the need for secondary complete closure after banding. LEVEL OF EVIDENCE: Level IV.

Outcomes after Split Abdominal Wall Muscle Flap Repair for Large Congenital Diaphragmatic Hernias.

INTRODUCTION: Repair of large congenital diaphragmatic hernias (CDHs) is challenging. As primary repair is not always feasible, patches are commonly used. An alternative treatment is split abdominal wall muscle flap repair, which uses vascularized autologous tissue. The aim of this study was to analyze the long-term outcome of large CDH defects undergoing split abdominal wall muscle repair. MATERIALS AND METHODS: This is a retrospective review (2003-2016) of large CDH treated by split abdominal wall muscle flap repair. RESULTS: In a total of 107 CDH patients, the abdominal muscle flap technique was used in 10 (9.3%); 7 had been prenatally treated with tracheal occlusion. Two patients experienced recurrence at 2 months and 6 years, respectively. Only one patient required abdominoplasty due to abdominal wall muscle weakness. Two patients developed progressive scoliosis; one of them required orthopaedic treatment. Minor chest wall deformities were detected in seven, but only one required orthopaedic treatment. The lung-to-head ratio was 0.79 in patients developing musculoskeletal deformities, and 1.5 in those without this complication (p < 0.05). Median follow-up was 11.2 years (3.5-14.2), and all patients were alive at the time of writing this article. CONCLUSION: The split abdominal wall muscle flap technique is a valid option for repair of large CDH. Associated musculoskeletal deformities seem to be influenced not only by the repair technique used but also by the degree of pulmonary hypoplasia and inherent pathophysiological changes.

Conversion from twice-daily to once-daily tacrolimus formulation in pediatric liver transplant recipients - a long-term prospective study.

To assess the safety and efficacy of conversion from twice-daily tacrolimus to once-daily tacrolimus in pediatric liver transplant recipients. Conversion from twice-daily to once-daily tacrolimus was made in stable pediatric liver transplant recipients. Doses and serum levels of tacrolimus, liver, and renal function were recorded on the day before the conversion and at days 5, 30, 90, and 180 postconversion. Patients were controlled every 2-3 months thereafter. Fifty-five patients were enrolled in the study. The mean age at conversion was 10.2 ± 3.6 years. The mean tacrolimus trough level was 4.7 ± 1.9 ng/dl preconversion, followed by a significant decline to 4.2 ± 1.7 30 days after the switch (P < 0.004). Mean daily tacrolimus dose was 0.09 ± 0.046 mg/Kg preconversion with a significant increase to 0.11 ± 0.060 3 months postconversion (P < 0.001). Fifteen patients with calculated glomerular filtration rate between 60 to 80 ml/min/m2 preconversion showed a significant improvement one and 3 years after the switch (73 ± 4.1, 83 ± 4.3 and 90.3 ± 7.3 ml/min/m2 , respectively (P < 0.001). The mean follow-up was 5.2 ± 2.4 years. Conversion to once-daily tacrolimus is safe and effective in a cohort of stable pediatric liver transplant patients.

Successful long-term outcome of pediatric liver-kidney transplantation: a single-center study.

INTRODUCTION: Liver-kidney transplantation is a rare procedure in children, with just ten to 30 cases performed annually worldwide. The main indications are autosomal recessive polycystic liver-kidney disease and primary hyperoxaluria. This study aimed to report outcomes of liver-kidney transplantation in a cohort of pediatric patients. METHODS: We retrospectively analyzed all pediatric liver-kidney transplantations performed in our center between September 2000 and August 2015. Patient data were obtained by reviewing inpatient and outpatient medical records and our transplant database. RESULTS: A total of 14 liver-kidney transplants were performed during the study period, with a median patient age and weight at transplant of 144.4 months (131.0-147.7) and 27.3 kg (12.0-45.1), respectively. The indications for liver-kidney transplants were autosomal recessive polycystic liver-kidney disease (8/14), primary hyperoxaluria -1 (5/14), and idiopathic portal hypertension with end-stage renal disease (1/14). Median time on waiting list was 8.5 months (5.7-17.3). All but two liver-kidney transplants were performed simultaneously. Patients with primary hyperoxaluria-1 tended to present a delayed recovery of renal function compared with patients transplanted for other indications (62.5 vs 6.5 days, respectively, P 0.076). Patients with liver-kidney transplants tended to present a lower risk of acute kidney rejection than patients transplanted with an isolated kidney transplant (7.2% vs 32.7%, respectively; P < 0.07). Patient and graft survival at 1, 3, and 5 years were 100%, 91.7%, 91.7%, and 91.7%, 83.3%, 83.3%, respectively. No other grafts were lost. CONCLUSION: Long-term results of liver-kidney transplants in children are encouraging, being comparable with those obtained in isolated liver transplantation.

Duodenal lengthening in short bowel with dilated duodenum.

UNLABELLED: Although duodenal dilatation occurs in children with short bowel syndrome (SBS) facilitating dismotility and bacterial overgrowth, the duodenum has been an untouchable intestinal segment for lengthening procedures owing to its close relationship with bilio-pancreatic structures and blood supply shared with the pancreas. Three children (age range, 0.5-7 years) with SBS and dilated duodenum underwent a novel surgical procedure of duodenal lengthening combined with a technical modification of serial transverse enteroplasty (STEP). Pre-STEP, jejunum length was 5, 35 and 45cm, respectively. Duodenal lengthening was performed with sequential transverse applications of an endoscopic stapler on the anterior and posterior wall of the duodenum to avoid bilio-pancreatic structure injury. Two patients underwent 3 duodenal firings (stapler of 35mm) and the third 5 firings (stapler of 45mm). Duodenal firings were 17%, 21% and 83% of the total firings. RESULTS: No surgical complications occurred. One patient developed transient episodes of D-lactic acidosis. Two patients (5 and 45cm) were weaned off parenteral nutrition at 12months post-surgery and the remaining patient´s (35cm) parenteral calorie requirements have decreased by 60%. CONCLUSION: Duodenal lengthening is effective since it tailors and increases the absorptive surface of the duodenum, even in cases of extreme SBS.