Mild to Moderate Intrapulmonary Shunting in Pediatric Liver Transplantation: Is Screening Necessary?

BACKGROUND: Despite reported associations between intrapulmonary vascular shunting (IPVS) and morbidity and mortality in pediatric liver transplantation (LT), there are no guidelines for screening. OBJECTIVE: To investigate IPVS before and after pediatric LT. METHODS: Retrospective records review of all pediatric LT (n = 370) from 2005 to 2015 at a single institute in Japan. All children with cirrhosis and clinical suspicion of IPVS without cardiac or pulmonary conditions were included. 99mTechnetium labelled macroaggregated albumin (99mTcMAA) scans were performed before and after LT. The severity of IPVS was graded using shunt ratios. RESULTS: Twenty-four children fulfilled inclusion criteria and underwent Tc99MAA scans. All revealed mild (<20%) to moderate (20%-40%) grades of IPVS. Following LT, the mean shunt ratio regressed from 20.69 ± 6.26% to 15.1 ± 3.4% (P = .06). The median (range) follow-up was 17 (4-85) months. Mortality was zero. The incidence of portal vein thrombosis (4.2%) biliary strictures (12.5%) and graft loss (4.1%) in the study group was not statistically significant compared to the remainder of the 370 transplants (3.2%, 9.4% and 3%, respectively). Sub-group analysis revealed hepatopulmonary syndrome (HPS) in 2 out of 24 children. The mean shunt ratios before and after LT were 39.2 ± 0.77% and 16.2 ± 8.5%, respectively (P = .08). There was 1 complication (intra-abdominal abscess). CONCLUSIONS: HPS is less likely in mild to moderate IPVS. LT may achieve comparable results when performed in the presence of mild to moderate IPVS.

Ileal Cystic Lymphangioma presenting with Acute Appendicitis.

Mesenteric lymphatic malformations are rare benign tumors that are most commonly found in children. The presentation of these tumors can be variable. It has been suggested that mesenteric lymphatic malformations are congenital; however, there is evidence that their size may be increased by infection. A 3-year 10-month-old boy presented with rhinorrhea and cough followed by acute abdominal pain. Ultrasonography revealed a lobulated mass in the lower abdomen. Computerized tomography scan of the abdomen diagnosed an inflamed appendix with perforation. Laparotomy revealed a multicystic mass within the mesentery closely adherent to the ileum; with omentum wrapped around. The appendix was dilated and firm. An ileal resection and an appendicectomy were performed. Histology confirmed acute transmural appendicitis with concurrent cystic lymphangioma of the ileal mesentery. Although rare, cystic lymphangioma must be considered in the differential of pediatric acute abdomen. Surgeons must be aware of this association and be reminded to inspect the rest of small bowel during appendicectomy especially if the preoperative imaging is suspicious.

Total internal biliary diversion during liver transplantation for type 1 progressive familial intrahepatic cholestasis: a novel approach.

LT for PFIC type 1 is often complicated by postoperative diarrhea and recurrent graft steatosis. A 26-month-old female child with cholestatic jaundice, pruritus, diarrhea, and growth retardation revealed total bilirubin 9.1 mg/dL, gamma-glutamyl transpeptidase 64 IU/L, and TBA 295.8 µmol/L. Genetic analysis confirmed ATP8B1 defects. A LT (segment 2, 3 graft) from the heterozygous father was performed. Biliary diversion was performed by a 35-cm jejunum conduit between the graft hepatic duct and the mid-transverse colon. Stools became pigmented immediately. Follow-up at 138 days revealed resolution of jaundice and pruritus and soft-to-hard stools (6-8 daily). Radioisotope hepato-biliary scintigraphy (days 26, 68, and 139) confirmed unobstructed bile drainage into the colon (t1/2 34, 27, and 19 minutes, respectively). Contrast meal follow-through at day 62 confirmed the absence of any colo-jejuno-hepatic reflux. At 140 days, contrast follow-through via the biliary stent revealed patent jejuno-colonic anastomosis and satisfactory transit. Graft biopsy at LT, 138 days, and 9 months follow-up revealed comparable grades of macrovesicular steatosis (<20%). TIBD during LT may be a clinically effective stoma-free biliary diversion and may prevent recurrent graft steatosis following LT for PFIC type 1.

Vascular complications in pediatric liver transplantation; single-center experience from Singapore.

AIM: Vascular complications (VC) are a major cause of significant morbidity and mortality in pediatric liver transplantation (LT). We reviewed our series to study the evolution of vascular reconstructions and its effect on the incidence of VC after LT, particularly with regard to the portal vein (PV). METHODS: The medical records of 81 pediatric LT performed in 76 children (38 boys) from 1991 to 2010 in the National University Hospital, Singapore, were reviewed to identify VC pertaining to PV, hepatic artery (HA), and hepatic veins (HV) and to analyse the data for the entire series and in 2 consecutive cohorts: initial 40 LT (group 1) and subsequent 41 LT (group 2). Specific interventions in group 2 were characterized by surgical innovations for reconstruction of the difficult PV and routine use of Doppler ultrasound intraoperatively and postoperatively. RESULTS: The overall incidence of VC was 19.7% (n = 16) and individually HA thrombosis 4.9% (n = 4), HA stenosis 1.2% (n = 1), PV thrombosis 12.3% (n = 9), PV stenosis 1.2% (n = 1), and HV thrombosis 1.2% (n = 1). The overall 1- and 5-year survival rates in our series were 89% and 85%, respectively. The 1- and 5-year survival rates in patients with and without VC were 81.25% and 68.75% and 90.8% and 89.2%, respectively. The incidence of VC decreased from 27.5% in group 1 to 12.1% in group 2 (p = .08). The major contribution to this appears to be a decrease in PV complications from 17.5% in group 1 to 7.3% in group 2 (P = .1). The incidence of HA (3 vs 2) and HV (1 vs 0) complications was similar between the 2 groups. CONCLUSIONS: Vascular reconstructions in small recipients are technically challenging and associated with a learning curve. Application of meticulous techniques in general, surgical innovations to the difficult PV in particular and attention to postoperative monitoring contribute toward a major reduction in VC.

Unusual shunt for symptomatic portal vein thrombosis after liver transplantation - Clatworthy revisited.

PV thrombosis is not an uncommon occurrence following pediatric LT. Symptomatic PHT following PV thrombosis is treated medically, surgical portosystemic shunting (mesorex, splenorenal, and mesocaval) being reserved for refractory cases. A 10-yr-old boy suffered recurrent malena and hemorrhagic shock because of chronic PV thrombosis following LT nine yr ago (1999). Extensive work-up failed to localize the bleeding source. The liver function remained normal. Initial attempts at surgical shunts failed owing to thrombosis (mesocaval 2001, splenorenal, inferior mesenteric-left renal vein, splenic-left external iliac vein 2008). In this situation, we performed a Clatworthy shunt by anastomosing the divided lower end of the LCIV to the side of SMV. There was a single, large caliber anastomosis. Post-operatively, the malena stopped completely, and clinically, there was no lower limb edema or encephalopathy. Doppler USG revealed persistence of hepatopetal flow within the portal collaterals. Follow-up at two yr reveals stable hepatic function with a patent shunt. To the best of our knowledge, we are not aware of a Clatworthy shunt being performed in a transplant setting. We reviewed the literature pertaining to this shunt in non-transplant patients with PHT.

Inflammatory tumour of the retroperitoneum--a case report.

INTRODUCTION: Neoplastic growths of myofibroblasts occurring on a background of plasma cell and lymphocytic proliferation have been designated as inflammatory myofibroblastic tumours (IMTs). These unusual tumours were first described in pulmonary location in adults. Though extrapulmonary masses have been reported in children; retroperitoneal growths are exceedingly rare. We report a case of retroperitoneal IMT that presented with constitutional symptoms without any palpable abdominal mass. CLINICAL PICTURE: A previously well 12-year-old boy presented with fever, right-sided flank pain and weight loss of 1-month duration. There were no foci of infection. The erythrocyte sedimentation rate (ESR) was raised; the white cell count was normal. An abdominal computed tomography (CT) scan revealed a right suprarenal tumour measuring 3.5 cm without any calcification. The urinary catecholamines and vanilmandelic acid were normal. TREATMENT: A laparotomy with complete excision of the tumour was performed. Final histology revealed an inflammatory myofibroblastic tumour without any correlates of aggressive behaviour. OUTCOME: Postoperatively, the constitutional symptoms of fever, weight loss and raised ESR normalised. Follow-up CT was normal and further treatment was not necessary. CONCLUSION: Although rare, IMTs should be considered in any abdominal solid tumour with associated constitutional and laboratory features of an inflammatory response. Complete surgical excision is effective treatment for biologically benign tumours.

Management of ventral hernia after giant exomphalos with external pressure compression using helmet device.

PURPOSE: The aim of this study was to evaluate an alternative technique of reducing a ventral hernia that follows the primary conservative treatment of a giant omphalocoele. METHODS: The patient is a full-term male neonate with a giant exomphalos. Initially triple dye was applied as an eschar-inducing agent. This resulted in a ventral hernia after 1 month. It was decided to achieve expansion of the abdominal cavity based on the principle of external pressure compression using a sphygmomanometer cuff over the hernia. The cuff was worn continuously, and manual pressure was applied daily. Care was taken to avoid intraabdominal hypertension using the reading of the manometer that was attached. The external pressure was corroborated with observations of respiration and circulation. RESULTS: The child did not show any ill effects of raised intraabdominal pressure. Throughout the treatment, the child was on full oral feedings and did not require any ventilator support. Reduction of the ventral hernia was achieved in 9 months. Surgical repair of the residual hernia defect was carried out by double breasting of the fascia. CONCLUSIONS: The application of controlled external pressure using a specially constructed device is a safe, noninvasive, and effective method of achieving reduction of a ventral hernia after primary conservative treatment of a giant omphalocoele.

Anorectal malformation with congenital absence of vagina: a case report and review of the literature.

Congenital anorectal malformations are relatively common, and associated genitourinary malformations have been reported in up to 40% of patients. Uterovaginal malformations are also not rare in children. They are known to occur with increased frequency in children with anorectal malformations, but the diagnosis may still be difficult. We report herein one such case of anorectal malformation associated with congenital total absence of the vagina in which the uterovaginal malformation was not diagnosed until the operative repair of the anorectal malformation. The operative procedure was thereafter directed, along with the anorectoplasty, towards restoring a functional uterovaginal tract. Review of the literature revealed that such a diagnosis is unsuspected or delayed in more than half of affected patients. Furthermore, these patients present with many diagnostic and therapeutic problems. Our report highlights the need to be aware of this condition to allow for an earlier diagnosis and appropriate operative treatment.