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.     

Successful treatment for advanced small cell carcinoma of the ovary

Small cell carcinoma of the ovary is a rare and aggressive malignant tumour with a poor prognosis. The authors describe two females, 12 and 13 years old, who presented with advanced stage disease. They were treated with surgical resection, multiagent chemotherapy and high-dose chemotherapy followed by autologous bone marrow transplantation. They remain free of disease more than 9.5 and 14 years since the diagnosis.     

Oophorectomy in children. Who and why: 13-year experience in a single centre

Aim: Oophorectomy performed in children is extremely uncommon. We aimed to investigate the disease pattern and the association between the underlying pathology and the clinical presentation among those patients who had their ovaries removed in their childhood. Methods: A retrospective study was performed on 41 consecutive children who underwent oophorectomy in a tertiary referral centre in the period between June 1995 and May 2008. Results: The median age was 11 years, ranged from 11 weeks to 15 years at the time of surgery. The primary presentations were acute lower abdominal pain (n= 20), progressive abdominal distension or abdominal mass (n= 13), chronic abdominal pain (n= 3), irregular menses (n= 1), antenatal diagnosis (n= 3) and incidental finding (n= 1). Ultrasound examination was performed in 31 patients and positive findings of ovarian pathology were found in all but one examination. Twenty cases of ovarian torsion were confirmed intra‐operatively. Patients presenting with acute abdominal pain were more likely to have torsion than other presentations (P < 0.01). Non‐neoplastic conditions and ovarian neoplasms were found in 11 and 30 patients, respectively. The most common neoplasm was mature teratoma (52%). Malignant neoplasms included immature teratoma (n= 3), dysgerminoma (n= 1), mixed dysgerminoma + yolk sac tumour (n= 2), yolk sac tumour (n= 2) and juvenile granulose cell tumour (n= 1). Malignant neoplasms were found to have more chronic presentation and less torsion than benign pathologies (P < 0.05). Conclusion: Although ovarian pathology is uncommon in children, a girl presenting with acute lower abdominal pain or progressive abdominal distension should raise the suspicion and prompt immediate investigation to rule out ovarian torsion or ovarian neoplasms.     

OVARIAN TISSUE CRYOSTORAGE AND GRAFTING: An Option to Preserve Fertility in Pediatric Patients with Malignancies

Fertility preservation in childhood cancer has become an important area of investigation due to increasing survival rates after cancer therapy. For these patients with an increased risk of infertility and premature ovarian failure, cryopreservation of ovarian tissue is a promising tool to preserve at least part of the reproductive potential. In recent years significant improvements have been achieved in this area, and 2 live births after autografting of frozen–thawed ovarian tissue have been reported. However, further research is needed to assess the clinical effectiveness of ovarian cryopreservation, to optimize the technique, and to limit the risk of reintroducing cancer cells in the patient with the graft.     

Ovarian tissue cryostorage and grafting: an option to preserve fertility in pediatric patients with malignancies

Fertility preservation in childhood cancer has become an important area of investigation due to increasing survival rates after cancer therapy. For these patients with an increased risk of infertility and premature ovarian failure, cryopreservation of ovarian tissue is a promising tool to preserve at least part of the reproductive potential. In recent years significant improvements have been achieved in this area, and 2 live births after autografting of frozen-thawed ovarian tissue have been reported. However, further research is needed to assess the clinical effectiveness of ovarian cryopreservation, to optimize the technique, and to limit the risk of reintroducing cancer cells in the patient with the graft.     

Orthotopic ovarian transplant--review and three surgical techniques

Transplantation of organs is a rapidly expanding faculty of medicine. While the solid organ transplantation has grown by leaps and bounds, ovarian transplantation is still in its infancy. Although recent interest has been generated for preservation of fertility in cancer therapy patients, other indications have emerged. Ovarian dysgenesis with missing normal ovarian complement and premature ovarian failure has come in the forefront. Three cases of orthotopic ovarian transplant with different surgical techniques have been described along with a brief overview.     

Normal ovarian function and assessment of ovarian reserve in the survivor of childhood cancer

Increasingly young people survive cancer in childhood and as a result complications of its treatment are becoming more common and important. Premature ovarian failure is recognized as a complication of radiotherapy to a field that includes the pelvis and alkylating‐agent‐based chemotherapy. Young pre‐pubertal girls are not protected from the effects of gonadal toxic therapy. A young woman, successfully treated for cancer during childhood, may experience regular periods in the presence of a significantly reduced ovarian reserve. There is, however, no reliable measure of ovarian reserve available for the individual woman. Assessment of ovarian function relies on the use of surrogate markers such as follicle stimulating hormone, inhibin‐B, and anti‐mullerian hormone as well as ultrasound assessment of ovarian volume and antral follicle count. We discuss the physiology of normal ovarian function, the effects of cancer treatments on ovarian function and the techniques for evaluation of ovarian reserve in survivors of childhood cancer. Pediatr Blood Cancer 2009;53:296–302. © 2009 Wiley‐Liss, Inc.     

Indications for combined liver and kidney transplantation in children

Abstract:  A significant number of patients awaiting liver transplantation have associated renal failure and renal dysfunction is associated with increased morbidity and mortality after LT. There has been a recent increase in the number of CLKT in adults. The common indications for CLKT in children are different from those of adults and include metabolic diseases affecting the kidney with or without liver dysfunction and congenital developmental abnormalities affecting both organs. The results are generally encouraging among these groups of patients. Early evaluation and listing of patients before they become severely ill or have major systemic manifestations of their metabolic problem are important.     

Impact of ovarian transposition before pelvic irradiation on ovarian function among long‐term survivors of childhood Hodgkin lymphoma: A report from the St. Jude Lifetime Cohort Study

1 Background

We reviewed the effect of ovarian transposition (OT) on ovarian function among long‐term survivors of childhood Hodgkin lymphoma (HL) treated with pelvic radiotherapy.

2 Procedure

Female participants (age 18+ years) with HL in the St. Jude Lifetime Cohort Study (SJLIFE) were clinically evaluated for premature ovarian insufficiency (POI) 10 or more years after pelvic radiotherapy. Reproductive history including age at menopause and pregnancy/live births was available on all patients.

3 Results

Of 127 eligible females with HL, 90 (80%) participated in SJLIFE, including 49 who underwent OT before pelvic radiotherapy. Median age at STLIFE evaluation was 38 years (range 25–60). In a multiple regression adjusted for age at diagnosis, pelvic radiotherapy doses > 1,500 cGy (hazard ratio [HR] = 25.2, 95% confidence interval [CI] = 3.1–207.3; P = 0.0027) and cumulative cyclophosphamide equivalent doses of alkylating agents > 12,000 mg/m² (HR = 11.2, 95% CI = 3.4–36.8; P < 0.0001) were significantly associated with POI. There was no significant association between OT and occurrence of POI (HR = 0.6, 95% CI = 0.2–1.9; P = 0.41).

4 Conclusions

OT did not appear to modify risk of POI in this historic cohort of long‐term survivors of HL treated with gonadotoxic therapy. Modern fertility preservation modalities, such as mature oocyte cryopreservation, should be offered to at‐risk patients whenever feasible.     

Successful arterial thrombolysis and percutaneous transluminal angioplasty for early hepatic artery thrombosis after split liver transplantation in a four-month-old baby

Abstract:  Early HAT is the most frequent and severe vascular complication following liver transplantation. It is one of the major causes of graft failure and mortality. Endovascular thrombolytic treatment in patients with thrombotic complications after liver transplantation is an attractive alternative to open surgery as lower morbidity and mortality rates are reported for it. PTA following transcatheter thrombolysis has been successfully used to treat HAT in adults. To the best of our knowledge, there have not been any reports of a successful transcatheter thrombolysis using interventional radiological techniques in a patient only four months old. The present report describes the successful endovascular emergency treatment of a HAT three days after DD split liver transplantation.     

Adaptive functioning and its correlates after intestine and liver transplantation

In this cross‐sectional study, we compared levels of adaptive functioning and examined potential correlates of adaptive functioning in 18 pediatric intestine (ITX) and 22 liver (LTX) recipients transplanted between June 2003 and March 2009. Family caregivers completed the ABAS‐II scale and provided socio‐demographic information regarding recipients' age at transplantation, gender, ethnicity, time since transplantation, and caregivers' role, ethnicity, education, and family income. Overall adaptive functioning and all three adaptive functioning subdomain scores were significantly lower in ITX patients compared with LTX patients (p ≤ 0.04) and compared with the general population normative mean (p ≤ 0.003). Significant correlates of adaptive functioning after abdominal transplant included type of transplant procedure (r = ?0.4, p = 0.02), gender (r = 0.4, p = 0.01), and educational level of caregiver (r = 0.5, p = 0.003) and together explained 45% of the variance in overall adaptive functioning. Findings provide new information regarding everyday functioning outcomes of ITX patients, add to existing data regarding non‐medical outcomes for LTX patients, and highlight the need for ongoing monitoring and intervention following transplantation to enhance outcomes.     

Subclinical cardiovascular changes in pediatric solid organ transplant recipients: A systematic review and meta‐analysis

CV disease is a major cause of morbidity and mortality following solid organ transplantation in adults. While the prevalence of multiple cardiometabolic risk factors is increased in pediatric solid organ transplant recipients, it is not clear whether they have subclinical CV changes. cIMT, central pWV, and CAC are indicative of subclinical CV disease, and, in adults, predict future CV events. The objective of this systematic review and meta‐analysis was to investigate the prevalence of subclinical CV changes, as measured by cIMT, pWV, and CAC among pediatric solid organ transplant recipients. We searched MEDLINE^® and EMBASE and conducted meta‐analysis for studies that evaluated cIMT, central pWV, and CAC among pediatric solid organ transplant recipients (kidney, lung, intestine and liver). The search identified nine eligible studies that included a total of 259 patients and 685 healthy controls. Eight studies reported on kidney transplant recipients and one study on a combined cohort of kidney and liver transplant recipients. The mean cIMT of transplant recipients was significantly higher than that of healthy controls (mean difference = 0.05 mm, 95% CI 0.02–0.07; p < 0.0001) with an estimated pooled prevalence of elevated cIMT of 56.0% (95% CI 17.0–95.0). The one study that assessed pWV showed increased vascular stiffness in transplant recipients compared to healthy controls. No studies assessing for CAC were found. There were limited data regarding subclinical CV disease following pediatric solid organ transplantation. In conclusion, kidney transplantation in childhood is associated with a higher prevalence of subclinical CV changes compared to healthy children. Longitudinal studies are needed to determine whether children have increased CV morbidity and mortality after transplantation.     

Living-donor liver transplantation for propionic acidemia

Propionic acidemia is a rare autosomal recessive disorder affecting the catabolism of branched-chain amino acids because of a genetic defect in PCC. Despite the improvements in medical treatment with protein restriction, sufficient caloric intake, supplementation of l-carnitine, and metronidazole, patients with the severe form of propionic acidemia have life-threatening metabolic acidosis, hyperammonemia, and cardiomyopathy, which results in serious neurologic sequelae and sometimes death. This study retrospectively reviewed three children with neonatal-onset propionic acidemia who received LDLT. Between November 2005 and December 2010, 148 children underwent LDLT, with an overall patient survival of 90.5%, in our center. Three patients were indicated for transplantation because of propionic acidemia. All recipients achieved a resolution of metabolic derangement and better quality of life with protein restriction and medication, although urine methylcitrate and serum propionylcarnitine levels did not decrease markedly. LT can reduce the magnitude of progressive cardiac/neurologic disability as a result of poor metabolic control. Further evaluation is therefore required to determine the long-term suitability of this treatment modality.     

Use of pediatric donor en bloc kidneys along with bladder segment in pediatric liver‐kidney and multivisceral‐kidney transplantation

The combination of pediatric multivisceral and kidney transplantation leads to additional recipient risks due to the number of anastomoses and to the small sizes of donor structures. The inclusion of donor kidneys, ureters, and a bladder patch en bloc with multivisceral organs decreases the number and complexity of anastomoses and has not yet been reported. Four patients were transplanted in this fashion; three underwent multivisceral‐kidney and one underwent liver‐kidney transplantation. The first patient was a 3‐year‐old male with polycystic kidney disease and congenital hepatic fibrosis. The second was a 7‐year‐old female with complications from necrotizing enterocolitis. The third was a 12‐month‐old male with megacystis microcolon intestinal hypoperistalsis syndrome and secondary hydronephrosis, and the fourth was a 3‐year‐old male with multiple intestinal resections secondary to incarcerated hernia. The third patient developed a right ureteral stenosis with an intact bladder patch. The fourth child expired from maintained abdominal sepsis. The first 3 patients maintained normal graft function. There were no cases of thrombosis, arterial stenosis, or urinary leakages. These reported cases demonstrate that small pediatric en bloc transplantation of the multivisceral organs and dual kidneys with a bladder patch anastomosis is a feasible and less complex alternative to the standard procedure.     

Feasibility of ovarian tissue cryopreservation for prepubertal females with cancer

BACKGROUND: Loss of fertility is one of the long-term adverse effects of high-dose chemotherapy or total body irradiation for cancer, even in children. Ovarian tissue cryopreservation (OTC) may make it possible for survivors of childhood cancer to have children. We evaluated the feasibility of this technique for prepubertal girls. METHODS: Between September 2000 and February 2005, 49 prepubertal girls were referred to the Reproductive Biology Unit for OTC before sterilizing treatment. RESULTS: One ovary each was collected from 47 patients, by laparoscopy in 24 patients and laporotomy in the others. In 16 cases, the ovary was harvested during laparotomy to resect a residual abdominal tumor. No complications occurred after operations. Ovarian tissue was frozen by a slow-cooling protocol, using DMSO and sucrose as cryoprotectants. An mean of 17.6 +/- 6.5 ovarian tissue fragments was cryopreserved per patient. Follicle concentration was evaluated histologically for 46 patients and a strong correlation was found between age and follicular density. None of the cases had visible ovarian tumor components. Ovarian cryopreservation was not carried out for two patients. CONCLUSION: The cryopreservation of ovarian tissue could be systematically offered even to prepubertal girls at risk of sterility due to gonadotoxic treatment.     

Biliary reconstruction before clamp removal to avoid portal vein thrombosis in pediatric living‐donor liver transplantation using hyper‐reduced left lateral segment grafts: A novel technical strategy

LT has become the treatment of choice for children with end‐stage liver disease. The scarcity of donors and the considerable mortality on waiting lists have propelled the related living‐donor techniques, especially in small children. This population need smaller and good quality grafts and are usually candidates to receive a LLS from a related donor. Many times this grafts are still large and do not fit in the receptor's abdomen, so a further hyper‐reduction may be required. Despite all advances in LT field, vascular complications still occur in a considerable proportion remaining as a significant cause of morbidity, graft loss, and mortality. Technical issues currently play an essential role in its genesis. The widely spread technique for biliary and vascular reconstruction in living donor LT (LDLT) nowadays implies removal of the portal vein (PV) clamp after the venous anastomosis, then the arterial reconstruction is done, followed by the biliary reconstruction. However, due to the posterior location of the LLS bile duct, for its reconstruction, a rotation of the liver is required risking a potential transient PV occlusion leading to thrombosis afterward. We describe a new technique that involves performing biliary reconstruction after the PV anastomosis and before removing the vascular clamp, thus allowing to freely rotate the liver with less risk of PV occlusion and thrombosis.