Successful kidney transplantation in a small child with end‐stage renal disease due to angiotensin receptor blocker fetopathy and atretic inferior vena cava

Kidney transplantation is the treatment of choice in pediatric patients with end‐stage renal disease. This population presents technical challenges particularly in those less than 20 kg due to anomalous anatomy, vascular access issues prior to transplantation, and a generally small size for age. Standard allograft outflow is usually achieved utilizing the iliac veins or IVC. When use of the iliocaval system is not feasible, alternative anastomosis must be considered. Herein, we report a case of a pediatric kidney transplantation where successful allograft outflow was achieved using the SMV when he was found to have an atretic IVC intraoperatively. In this setting, use of the portal system was required to achieve adequate allograft outflow. We created a donor iliac graft for added length to anastomose the renal vein with the SMV. In the setting of IVC occlusion with poor drainage, we utilized a patent vessel with larger caliber for outflow to reduce the risk of high venous pressures, allograft failure, venous rotation, and thrombosis. We conclude that the SMV may serve as an alternative outflow tract in the small pediatric patient and provides the vessel caliber needed to reduce the risks of complications.     

Use of a donor iliac vein graft for reconstruction of the inferior vena cava in liver transplantation for hepatoblastoma with caval extension

Complete microscopic tumor resection is critical for successful treatment of hepatoblastoma, and this may include when liver transplantation is required. For tumors involving the IVC or PV, complete resection should include the involved IVC or PV to ensure full tumor clearance. When this is required, the venous reconstruction at transplant or post‐excision can be challenging. We present the management of an 18‐month‐old girl with PRETEXT Stage IV (P, V, F) hepatoblastoma and IVC involvement, where native caval resection and reconstruction was required. The preoperative staging following neoadjuvant chemotherapy was POSTTEXT Stage IV (P, V, F). An orthotopic liver transplantation was performed using a left lateral segment graft from a deceased adult donor. With native hepatectomy, retrohepatic IVC resection from just above the hepatic venous confluence to just above the entry of the right adrenal vein was performed. For caval reconstruction, a venous graft from a deceased donor was used. The graft included the lower IVC with the right common iliac vein and a short stump of the left common iliac vein. The common iliac was a perfect size match for the IVC, and the three natural ostia matched the upper cava, lower cava, and the outflow from the donor left hepatic vein. The patient had an uneventful postoperative course and remains well and disease‐free 2 years after transplant with continued patency of the reconstructed cava. When indicated, a donor iliac vein graft with its natural ostia should be considered in caval reconstruction for pediatric liver transplantation.     

Hepatic venous reconstruction using the superficial femoral vein in a right‐lobe living donor liver transplant patient with interrupted inferior vena cava

Anatomical abnormalities in patients with BA often include polysplenia, preduodenal portal vein, interrupted retrohepatic IVC, cardiac abnormalities, and situs inversus. In LDLT patients who had congenital vascular anomalies, additional surgical modifications for the reconstruction of hepatic venous branches are sometimes necessary to prevent venous parenchymal congestion. We report a 12‐yr‐old female with post‐Kasai BA with interrupted retrohepatic IVC who underwent right‐lobe LDLT because the left liver graft volume was insufficient. The donor right liver graft had three major hepatic branches, including the RHV, IRHV, and MHV tributary (V8). We performed hepatic venous reconstruction by creating a large, wide triple orifice consisting of the RHV and two SFVs, which were anastomosed to the V8 and IRHV using the donor's SFV as an interposition graft. In conclusion, the reconstruction of venous orifices for right‐lobe LDLT patients with the absent retrohepatic IVC is can be carried out using an SFV graft derived from the living donor or the recipient.     

Combined en bloc liver–double kidney transplantation in an infant with IVC thrombosis

McGuire MM, Jones BA, Hull MA, Misra MV, Smithers CJ, Feins NR, Jenkins RL, Lillehei CW, Harmon WE, Jonas MM, Kim HB. Combined en bloc liver–double kidney transplantation in an infant with IVC thrombosis.
Pediatr Transplantation 2011: 15: E142–E144. © 2010 John Wiley & Sons A/S. Abstract: We report a case of a pediatric en bloc liver‐double kidney transplant in a patient with IVC thrombosis below the renal veins. The patient is an 11‐month‐old girl diagnosed with congenital nephrotic syndrome at two months of age. Multifocal liver masses were identified on routine ultrasound at eight months of age. Alpha fetoprotein level was 55 319. Biopsy confirmed hepatoblastoma. CT scan confirmed multiple lesions in both lobes, which would require liver transplantation for resection. She was also found to have thrombosis of her infrarenal IVC secondary to multiple central lines. She was listed for combined liver–kidney transplant and began chemotherapy. After four cycles of chemotherapy, she underwent bilateral nephrectomies followed by a combined en bloc liver‐double kidney transplant from a size matched donor. In order to provide adequate venous outflow from the kidneys in the absence of a recipient infrarenal IVC, the donor liver and kidneys were procured en bloc with a common arterial inflow via the infrarenal aorta and common outflow via the suprahepatic IVC. Kidney transplantation in the absence of adequate recipient venous drainage may require unusual vascular reconstruction techniques. This case demonstrates a novel approach in patients who may require combined liver–kidney transplantation.     

Asymptomatic inferior vena cava abnormalities in three children with end-stage renal disease: risk factors and screening guidelines for pretransplant diagnosis

We report two children with end-stage renal disease (ESRD) found to have inferior vena cava (IVC) thrombosis at the time of renal transplantation. The children suffered from renal diseases that included congenital hepatic fibrosis and portal hypertension as part of their pathophysiology. Neither child had evidence of hypercoaguability or clinical symptoms of IVC thrombosis. Prior to transplantation, the renal replacement therapy consisted primarily of peritoneal dialysis. During their hospital courses, these children had central venous catheters placed for temporary hemodialysis, episodes of peritonitis and numerous abdominal surgeries. The medical literature to date has not identified a link between IVC thrombosis and portal hypertension, nor has an association between the patients' primary renal disease and IVC thrombosis been found. We also report the finding of asymptomatic IVC narrowing in a third patient with obstructive uropathy, colonic dysmotility and numerous abdominal surgeries. IVC narrowing was diagnosed by CT scan during his pretransplant evaluation. In this paper, we consider similarities between these three patients that may have predisposed each of them to asymptomatic IVC pathology, including large-bore central venous access as young children and/or recurrent scarring abdominal processes. A discussion regarding appropriate screening of the 'high-risk patient' for IVC pathology prior to kidney transplantation and surgical options for children with this rare complication are presented.     

Fatal hyperammonemia associated with disseminated Serratia marcescens infection in a pediatric liver transplant recipient

Hyperammonemia is a rare and important complication post‐liver transplantation. We review a case of a 5‐month‐old boy with biliary atresia who received a split liver transplant following a variceal bleed. The transplant was complicated by recurrent portal vein thrombosis. Colonized with Serratia marcescens pretransplant, he developed disseminated infection associated with very high levels of ammonia that led to his death. It is important to be aware of serum ammonia levels in patients with portal vein thrombosis, particularly in the setting of gastrointestinal bleeding and sepsis.     

Renal transplant in children with previous inferior vena cava thrombosis

Our experience with renal transplantation in children with inferior vena cava thrombosis is presented in this study. Of the 238 children who have received renal transplants at our institution, four had IVC thrombosis (discovered during pretransplant evaluation: three patients; found at surgery: one patient). The pretransplant US evaluation diagnosis of IVC thrombosis in three patients was confirmed by transjugular retrograde cavography. There were no signs of hypercoagulability or IVC thrombosis symptoms prior to diagnosis in any patient. The graft was implanted in a left orthotopic position in three patients. Venous drainage was attained to the infrahepatic vena cava or native renal vein after ipsilateral nephrectomy. The renal artery of the graft was anastomosed to the aorta. In one patient, the graft was placed in the left iliac fossa. Patient and graft survival are 100%. Three grafts are functioning normally after a mean follow-up of 3.7 yr. The graft placed in the iliac fossa has moderate dysfunction due to high pressure venous outflow. Children with IVC thrombosis can be successfully transplanted orthotopically. Candidates with any suspicious-looking occlusion on ultrasound should be studied by retrograde venography to confirm diagnosis prior to transplantation.     

Combined split liver and kidney transplantation in a three‐year‐old child with primary hyperoxaluria type 1 and complete thrombosis of the inferior vena cava

Khan Z, Sciveres M, Salis P, Minervini M, Maggione G, Cintorino D, Riva S, Gridelli B, Emma F, Spada M. Combined split liver and kidney transplantation in a three‐year‐old child with primary hyperoxaluria type 1 and complete thrombosis of the inferior vena cava.
Pediatr Transplantation 2011: 15: E64–E70. © 2009 John Wiley & Sons A/S. Abstract: PH1 is an inborn error of the metabolism in which a functional deficiency of the liver‐specific peroxisomal enzyme, AGT, causes hyperoxaluria and hyperglycolic aciduria. Infantile PH1 is the most aggressive form of this disease, leading to early nephrocalcinosis, systemic oxalosis, and end‐stage renal failure. Infantile PH1 is rapidly fatal in children unless timely liver‐kidney transplantation is performed to correct both the hepatic enzyme defect and the renal end‐organ damage. The surgical procedure can be further complicated in infants and young children, who are at higher risk for vascular anomalies, such as IVC thrombosis. Although recently a limited number of children with IVC thrombosis have underwent successful kidney transplantation, successful multi‐organ transplantation in a child with complete IVC thrombosis is quite rare. We report here the interesting and technically difficult case of a three‐yr‐old girl with a complete thrombosis of the IVC, who was the recipient of combined split liver and kidney transplantation for infantile PH1. Although initial delayed renal graft function with mild‐to‐moderate acute rejection was observed, the patient rapidly regained renal function after steroid boluses, and was soon hemodialysis‐independent, with good diuresis. Serum and plasma oxalate levels progressively decreased; although, to date they are still above normal. Hepatic and renal function indices were at, or approaching, normal values when the patient was discharged 15‐wk post‐transplant, and the patient continues to do well, with close and frequent follow‐up. This is the first report of a successful double‐organ transplant in a pediatric patient presenting with infantile PH1 complicated by complete IVC thrombosis.     

Abdominal Doppler sonography in childhood

With the help of pulsed Doppler sonography and colour coded Doppler sonography noninvasive measurements of blood flow within the abdominal vessels are possible. Cystic and tubular abdominal masses and vascular structures can be differentiated. Laminar and turbulent flow as well as arterial and venous flow can be distinguished. The direction of blood flow can be shown. Regarding liver circulation Doppler sonography of portal hypertension, portal vein thrombosis, veno-occlusive-disease, Budd-Chiari-Syndrome and transplant rejection or other vascular complications after liver transplantation can be shown. The circulation of the spleen should be examined if aneurysms, thrombosis or portal hypertension are suspected. Mesenterial circulation should be investigated in suspected vascular occlusion, arterial stenosis and necrotizing enterocolitis. Renal Doppler sonography is helpful for the diagnosis of renal artery stenosis, arterio-venous fistulas, renal vein thrombosis and acute transplant rejection.     

Congenital absence of the portal vein: Case report and MR demonstration

Congenital absence of the portal vein (CAPV) is a rare anomaly that results from aberrant venous development in early embryonic life. The intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava (IVC), the left renal vein, or the left hepatic vein. We describe a case where prenatal ultrasonography demonstrated an unusual C-shaped vessel between the umbilical vein and a dilated IVC, and failed to show a portal vein. Ultrasonography and magnetic resonance imaging and magnetic resonance angiography at 4 months of age again found no portal vein. The superior mesenteric vein drained into the left renal vein.     

Alternatives for vascular reconstruction in pediatric living donor liver transplantation

Many publications discuss the various strategies for vascular reconstruction (VR) in pediatric LDLT. Having knowledge of alternative techniques is helpful in planning transplants. This article presents three case reports that illustrate some of the alternative techniques for HV, PV, and HA reconstruction in pediatric LDLT. It also reviews the available alternative strategies reported for VR in pediatric LDLT. In the first case, a 13‐month‐old girl presented a PRETEXT III HB with invasion of the retrohepatic vena cava. An LLS graft HV was anastomosed to a DD iliac vein graft and subsequently implanted in a “standard” fashion in the recipient. In the second case, a 44‐month‐old boy presented with multifocal HB and portomesenteric thrombosis and the portal inflow was done through a renoportal anastomosis. In the third case, a 22‐month‐old child with a failed Kasai procedure had extensive HA thrombosis. The HA reconstruction was performed with an interposition of the recipient's IMV graft. The use of alternative techniques for VR in pediatric LDLT is paramount to the success of such a complex procedure. Imaging studies can help transplant surgeons outline surgical strategies and define the best technique to be used in each case.     

Utility of thrombophilia screening in pediatric renal transplant recipients

Thrombosis after kidney transplantation may result in catastrophic outcomes, including graft loss. Thrombophilia has been implicated in post‐transplant thrombosis; data, however, are inconclusive on the impact of acquired and inherited thrombophilia and resultant thrombosis in renal graft recipients. We aimed to evaluate whether identifying children with thrombophilia during the pretransplant evaluation predicted post‐transplant outcomes. We reviewed 100 kidney transplants performed in 100 children, aged 1‐18 years, in a single‐center retrospective study. Routine pretransplant comprehensive thrombophilia evaluation was completed. Thrombophilia was demonstrated in 36% patients (N = 36). TEs occurred in 11 patients before kidney transplant. Low PS and antithrombin were found in 9/86 (10.5%) and 2/89 (2.2%) children, respectively. Heterozygosity for FLV and PGM were found in 5/81 (6.2%) and 1/93(1.1%) children, respectively. A post‐transplant thrombotic event occurred in 10 children (10%); six involved the renal transplant. The association between a history of a pretransplant thrombotic event and post‐operative renal graft thrombosis approached, but did not reach significance (P = 0.071). There was no association between preoperative screening abnormalities and post‐operative TEs. Graft loss due to a thrombotic event occurred in two patients; none had underlying thrombophilia. Our data suggest that the utility of universal, comprehensive preoperative thrombophilia testing is not beneficial in determining risk of post‐operative graft thrombosis. Thrombophilia testing may be considered in a select population with a history of pretransplant thrombotic event.     

Successful adult‐to‐child renal transplantation utilizing the ovarian vein in children with inferior vena cava/iliac vein thrombosis

Tao R, Shapiro R. Successful adult‐to‐child renal transplantation utilizing the ovarian vein in children with inferior vena cava/iliac vein thrombosis.
Pediatr Transplantation 2010: 14:E70–E74. © 2009 John Wiley & Sons A/S. Abstract: IVC/iliac vein thrombosis has previously been considered to be a contraindication to renal transplantation because of the technical difficulties and the increased risk of graft thrombosis. We report two successful cases of adult‐to‐child kidney transplantation in which we anastomosed the graft renal vein to the recipient ovarian vein in the presence of IVC and/or iliac vein thrombosis, with no short or long term vascular complications. Our experience, which adds to the successful reports from several other centers, suggests that the inability to use the iliocaval axis should no longer be considered a contraindication to pediatric renal transplantation.     

IVC angioplasty using an autologous vascular graft for IVC stenosis due to metallic stent in a pediatric liver transplant

A 12‐year‐old girl underwent LDLT using a left lobe graft for hepatic dysfunction associated with citrin deficiency. A continuous anastomosis suture technique was performed between the recipient's IVC and the donor's left hepatic vein. At age 14, the patient developed intractable ascites. Venography of the IVC and hepatic vein showed twisted‐shape stenosis of the hepatic vein‐IVC anastomosis with intravascular pressure gradient, probably due to the enlarged transplanted liver, for which a metallic stent was placed. The ascites disappeared, and the patient was making satisfactory progress eight months after surgery. However, nine months after surgery, the ascites appeared again with edema in the lower extremities. Since the stent that had been inserted was suspected of hampering the outflow of the graft liver and IVC, it was decided to conduct stent removal and IVC angioplasty. After intravascular exploration, the stent was removed. Angioplasty was performed. An autologous vascular graft patch was designed to be wedge‐shaped to fit the incised part of the IVC, and it was sutured with 5‐0 non‐absorbable surgical sutures using a continuous suture technique. No postoperative complications or perioperative graft dysfunction were observed. The ascites decreased markedly, and the edema in the lower extremities disappeared. Thus, we were able to successfully perform IVC angioplasty using an autologous vascular graft patch in a patient who developed IVC stenosis after stenting. This procedure is one of the most effective treatment options, especially for pediatric patients requiring long‐term vascular patency.     

Imaging the Rex vein preoperatively using wedged hepatic venous portography

Background  

Mesentericoportal bypass, the Rex shunt, restores the physiological hepatic portal flow and reduces the clinical sequelae of portal hypertension in children with extrahepatic portal vein obstruction (EHPVO). The preoperative evaluation includes an accurate assessment of the portal venous inflow and outflow. The former is readily assessed by ultrasound and MRI, while the outflow intrahepatic portal vein is harder to assess.     

Chronic diarrhea, ascites, and protein-losing enteropathy in an infant with hepatic venous outflow obstruction after liver transplantation

Hourigan SK, Anders RA, Mitchell SE, Schwarz KB, Lau H, Karnsakul W. Chronic diarrhea, ascites, and protein‐losing enteropathy in an infant with hepatic venous outflow obstruction after liver transplantation. Abstract: An 18‐month‐old female status post‐orthotopic liver transplant for biliary atresia presented nine months after transplant with severe diarrhea and intolerance of feeds. She was found to have a PLE as evidenced by a low serum albumin and a persistent elevation of fecal A1AT. Investigation eventually revealed that the cause of the PLE was a stricture at the anastomosis site between the hepatic vein and inferior cava, supported by resolution of the PLE after venoplasty of the stricture. The patient has subsequently required several repeat venoplasties for recurrence of her symptoms correlating with recurrence of the stricture. This is a very rare presentation of hepatic venous outflow obstruction. Moreover, normal duplex ultrasound imaging of liver vasculature and her unusual presentation led to a delay in her diagnosis highlighting the need for an increased index of suspicion.     

Spontaneous thrombosis of vein of Galen aneurysmal malformation.

Two cases of aneurysmal malformations of the vein of Galen (AVG) with spontaneous thrombosis are reported. Angiogram and MRI before thrombosis demonstrated AVGs with slow arteriovenous shunts and associated stagnation of contrast in the venous sac secondary to severe outflow restriction. Based on these findings, one patient was managed conservatively, and the other underwent placement of a ventriculoperitoneal shunt. Surveillance of the lesions with subsequent MRIs revealed spontaneous thrombosis of the AVGs with excellent clinical outcomes. Proposed mechanisms of spontaneous thrombosis include slow flow shunts, obstruction of the venous outflow or obstruction of the feeding artery. Similar cases in the literature are reviewed with special emphasis on diagnostic tests, symptomatology, mechanisms of thrombosis and therapeutic options.     

“Inverted” positioning of renal allograft during kidney transplantation in children and adolescents: A single‐institution comparative analysis

Renal transplantation is the treatment of choice in children with end‐stage renal failure. Limitations in patient anatomy or a short donor renal vein may necessitate intraoperative inversion of the kidney. There is little evidence to support the use of this surgical technique, and no evidence in the pediatric population. This study identifies the perioperative and post‐operative outcomes of inverted renal transplants in pediatric patients. We reviewed all patients having a renal transplant between January 2012 and December 2016 and collected short‐ and long‐term outcomes of patients who received an inverted allograft. Early graft function was defined as the time to reach creatinine nadir. During this time, our hospital performed 81 transplants, and 50 (62%) were from deceased donors, including the 6 (12%) patients who received inverted renal grafts. Half (3/6) were female, 5/6 (83%) were dialysis‐dependent, and the median age at surgery was 13 years (range 9‐16 years). There was no significant difference in mean creatinine nadir values (P = 0.518) and the time to creatinine nadir mean values (P = 0.190) between the upright and inverted renal transplant groups. There were also no significant differences in rates of post‐operative complications between the upright and inverted allograft recipients. Inversion of renal allografts in pediatric patients is a viable surgical technique to compensate for shortcomings in patient anatomy or in special cases of renal transplantation involving a short donor renal vein. Future research should focus on outcomes of a larger group of pediatric inverted renal transplant patients.     

The use of vascular homografts in pediatric small bowel transplantation: Single‐center experience over a decade

Intestinal transplantation in children has evolved with more isolated small intestine transplants being performed compared to combined liver‐intestine transplants. Consequently, surgical techniques have changed, frequently requiring the use of vascular homografts of small caliber to revascularize the isolated small intestine, the impact of which on outcomes is unknown. Among 106 pediatric intestine and multivisceral transplants performed at our center since 2003, 33 recipients of an isolated small intestine graft were included in this study. Outcome parameters were thrombotic complications, graft, and patient survival. A total of 29 of 33 (87.9%) patients required arterial and/or venous homografts from the same donor, mainly iliac or carotid artery and iliac or innominate vein, respectively (donor's median age 1.1 years [2 months to 23 years], median weight 10 kg [14.7‐48.5]). Post‐transplant, there were three acute arterial homograft thromboses and one venous thrombosis resulting in two peri‐operative graft salvages and two graft losses. Three of four thromboses occurred in patients with primary hypercoagulable state, including the two graft losses. Overall, at a median of 4.1 years (1‐10.2) from transplant, 29 of 33 (88%) patients are alive with 26 of 33 (79%) functioning grafts. The procurement of intact, size‐matched donor vessels and the management of effective post‐transplant anticoagulation are critical.     

Meso‐Rex bypass as an alternative technique for portal vein reconstruction at or after liver transplantation in children: Review and perspectives

de Ville de Goyet J, Lo Zupone C, Grimaldi C, D’Ambrosio G, Candusso M, Torre G, Monti L. Meso‐Rex bypass as an alternative technique for portal vein reconstruction at or after liver transplantation in children: Review and perspectives. Abstract: Direct portal revascularization can be achieved by interposing a vascular graft between the SMV and the Rex recessus (left portal vein system): the MRB. To review indications and results of the procedure in the setting of pediatric liver transplantation, reports were selected from the English literature. Previously reported series were updated to analyze long‐term outcome. A new series was added and analyzed as a complementary set of cases. A total of 51 cases were analyzed. With a 96% overall patient survival rate and a 100% long‐term patency rate when the IJV is used for the bypass, MRB achieves a very successful physiologic cure of chronic portal hypertension and restores the portal flow into and through the liver graft. It also has been used successfully for primary revascularization of liver grafts, as well as for managing early acute portal vein thrombosis episodes. The use of this procedure in conjunction with other strategies and techniques might be of interest for transplant surgeons, particularly those caring for children.