Sabine Irtan

Ovarian transposition in prepubescent and adolescent girls with cancer

Ovarian transposition was the first procedure proposed to preserve fertility in girls with cancer and is indicated for patients with tumours requiring pelvic radiation at doses of 42·0-58·4 Gy, much higher doses than those that can induce loss of ovarian function (4-20 Gy). Ovarian transposition is usually done after neoadjuvant chemotherapy and is completed by minimally invasive surgery or open surgery in case of concomitant resection of the abdominal tumour. According to the type of tumour, the ovaries are moved and placed in the paracolic gutters when the radiation field reaches the midline (for medulloblastoma or urogenital rhabdomyosarcoma), contralaterally to the tumour (for pelvic sarcomas), or in line with the iliac crests (for Hodgkins lymphoma). However, in 10-14% of cases the procedure can fail to protect the ovaries. Although few long-term results in adults are available, normal hormonal function and pregnancies have been reported in a few long-term follow-up studies. In view of the continued development of fertility preservation techniques, ovarian transposition should be discussed at a multidisciplinary meeting at the time of cancer diagnosis.

Renal transplantation in children: Critical analysis of age related surgical complications

Irtan S, Maisin A, Baudouin V, Nivoche Y, Azoulay R, Jacqz‐Aigrain E, El Ghoneimi A, Aigrain Y. Renal transplantation in children: Critical analysis of age related surgical complications.u2028Pediatr Transplantation 2010: 14:512–519.

MYO5B and bile salt export pump contribute to cholestatic liver disorder in microvillous inclusion disease

Microvillous inclusion disease (MVID) is a congenital disorder of the enterocyte related to mutations in the MYO5B gene, leading to intractable diarrhea often necessitating intestinal transplantation (ITx). Among our cohort of 28 MVID patients, 8 developed a cholestatic liver disease akin to progressive familial intrahepatic cholestasis (PFIC). Our aim was to investigate the mechanisms by which MYO5B mutations affect hepatic biliary function and lead to cholestasis in MVID patients. Clinical and biological features and outcome were reviewed. Pretransplant liver biopsies were analyzed by immunostaining and electron microscopy. Cholestasis occurred before (nu2009=u20095) or after (nu2009=u20093) ITx and was characterized by intermittent jaundice, intractable pruritus, increased serum bile acid (BA) levels, and normal gamma‐glutamyl transpeptidase activity. Liver histology showed canalicular cholestasis, mild‐to‐moderate fibrosis, and ultrastructural abnormalities of bile canaliculi. Portal fibrosis progressed in 5 patients. No mutation in ABCB11/BSEP or ATP8B1/FIC1 genes were identified. Immunohistochemical studies demonstrated abnormal cytoplasmic distribution of MYO5B, RAB11A, and BSEP in hepatocytes. Interruption of enterohepatic BA cycling after partial external biliary diversion or graft removal proved the most effective to ensure long‐term remission. Conclusion: MVID patients are at risk of developing a PFIC‐like liver disease that may hamper outcome after ITx. Our results suggest that cholestasis in MVID patients results from (1) impairment of the MYO5B/RAB11A apical recycling endosome pathway in hepatocytes, (2) altered targeting of BSEP to the canalicular membrane, and (3) increased ileal BA absorption. Because cholestasis worsens after ITx, indication of a combined liver ITx should be discussed in MVID patients with severe cholestasis. Future studies will need to address more specifically the effect of MYO5B dysfunction in BA homeostasis. (Hepatology 2014;60:301–310)

Minimally invasive surgery of neuroblastic tumors in children: Indications depend on anatomical location and image‐defined risk factors

Minimally invasive surgery (MIS) is still not a well‐accepted surgical approach to remove neuroblastic tumors. We aimed to assess the indications and limits of MIS in this childhood tumor according to tumor location and image‐defined risk factors (IDRFs).

Image‐defined risk factor assessment of neurogenic tumors after neoadjuvant chemotherapy is useful for predicting intra‐operative risk factors and the completeness of resection

Patients with neuroblastoma are now stratified at diagnosis according to the presence and number of image‐defined risk factors (IDRFs). We examined the added value of IDRF assessment after neoadjuvant chemotherapy for predicting surgical resection.

Stomal prolapse in children with chronic intestinal pseudoobstruction: a frequent complication?

AIMnThe aim of the study was to evaluate the morbidity rate of stoma in children diagnosed with chronic intestinal pseudoobstruction (CIPO) and try to determine risk factors.nnnMATERIAL AND METHODSnTwenty-two children (65%) of 34 referred to our center between 1988 and 2008 had a stoma. They were compared with 22 other children referred for another pathology necessitating a stoma.nnnRESULTSnThe incidence of stomal prolapse in CIPO children was 45% vs 9% in non-CIPO children (P = .01). Prolapse occurred between the first postoperative day and the 10th postoperative month, with a median of 2 months. Surgical management was required in 60%, with an intestinal necrosis rate of 20% leading to intestinal resection. No mortality was noted. No risk factors favoring prolapse in CIPO children were identified.nnnCONCLUSIONnChildren with CIPO have a high rate of stomal prolapse with an increased risk of intestinal necrosis. Careful management of the stoma is necessary to avoid the risk of intestinal resection, which may aggravate the underlying intestinal disorder.

Pancreaticobiliary Maljunctions in European Patients with Bile Duct Cysts: Results of the Multicenter Study of the French Surgical Association (AFC).

BackgroundPancreaticobiliary maljunctions (PBMs) are congenital anomalies of the junction between pancreatic and bile ducts, frequently associated with bile duct cyst (BDC). BDC is congenital biliary tree diseases that are characterized by distinctive dilatation types of the extra- and/or intrahepatic bile ducts. Todani’s types I and IVa, in which dilatation involves principally the main bile duct, are the most frequent. PBM induces pancreatic juice reflux into the biliary tract that is supposed to be one of the main factors of biliary cancer degeneration, although the diagnostic criteria of PBM that can be either morphological and/or functional are not well defined especially in Western series.ObjectiveThe aim of this study was to assess the relative prevalence of PBM in BDC in a large European multicenter study, to analyze the characteristics of PBM and try to propose diagnostic criteria of PBMs based on morphological and/or functional criteria and define the positive, negative predictive values, sensibility and specificity of either criteria.ResultsFrom 1975 to 2012, 263 patients with BDC were analyzed. Among them, 190 (72.2xa0%) were considered to present PBM. Types I and IVa had a similar rate of PBM association. According to the “AFC classification,” 57.2xa0% had a C–P type, 34.5xa0% a P–C type and 8.3xa0% a complex type (“anse-de-seau”). The median length of the common channel in patients with PBM was 15.8xa0±xa06.8xa0mm (range 5–40xa0mm). The median intrabiliary amylase and lipase levels were 65,249 and 172,104xa0UI/L, respectively. For the diagnostic of PBM, a common channel length of more than 8xa0mm and an intrabiliary amylase level superior to 8000xa0UI/L were associated with a predictive positive value and a specificity of more than 90xa0%. Synchronous biliary cancer had an incidence of 8.7xa0% in all patients with BDC and PBM 11.1xa0% in adults. Compared to type IV, the type I BDC was associated with statistically more cancer patients in the presence of PBM.Conclusions Characteristics of PBM associated with BDC in Western population are quite close to reported Eastern series. The results suggest considering both the intrabiliary value of amylase >8000xa0UI/L and a length of a common channel >8xa0mm as appropriate values for positive diagnosis of PBM.

Technical standardization of laparoscopic repair of Morgagni diaphragmatic hernia in children: results of a multicentric survey on 43 patients

BackgroundThis study aimed to standardize the surgical correction technique of congenital Morgagni diaphragmatic hernia (CMDH), analyzing the results of an international multicentric survey.Methods The medical records of 43 patients (29 boys, 14 girls) who underwent laparoscopic repair of CMDH in 8 pediatric surgery units in a 5-year period were retrospectively reviewed. Their average age was 3.3xa0years. Ten patients (23.2%) presented associated malformations: 9 Down syndrome (20.9%) and 1 palate cleft (2.3%). Thirty-five patients (81.4%) were asymptomatic, whereas 8 patients (18.6%) presented symptoms such as respiratory distress, cough or abdominal pain. As for preoperative work-up, all patients received a chest X-ray (100%), 15/43 (34.8%) a CT scan, 8/43 (18.6%) a barium enema and 4/43 (9.3%) a US.ResultsNo conversion to open surgery was reported. Average operative time was 61.2xa0min (range 45–110xa0min). In 38/43 (88.3%) patients, a trans-parietal stitch was positioned in order to reduce the tension during the repair. In 14/43 cases (32.5%), the sac was resected; in only 1/43 case (2.3%) a dual mesh of goretex was adopted to reinforce the closure. Average hospital stay was 2.8xa0days. The average follow-up was 4.2xa0years, and it consisted in annual clinical controls and chest X-ray. We recorded 2 complications (4.6%): one small pleural opening that required no drain and one recurrence (2.3%), re-operated in laparoscopy, with no further recurrence.ConclusionsTo the best of our knowledge, this is the largest series published in the literature on this topic. Laparoscopic CMDH repair is well standardized: The full-thickness anterior abdominal wall repair using non-resorbable suture with interrupted stitches is the technique of choice. Postoperative outcome was excellent. Recurrence rate was very low, about 2% in our series. We believe that children with CMDH should be always treated in laparoscopy following the technical details reported in this paper.

Urachal rhabdomyosarcoma in childhood: a rare entity with a poor outcome

BACKGROUND/PURPOSEnRhabdomyosarcoma (RMS) of the urachus is rare and gathered in the abdominal and other locations group for oncological treatment purpose, and therefore not well characterized. Our aim was to assess the clinical and prognostic specific features of urachal primary RMS in childhood.nnnMETHODSnWe retrospectively reviewed the charts of 8 patients with an urachal RMS treated between 1984 and 2013 in two Pediatric Oncology Departments. Median follow-up was 42 months (18-100).nnnRESULTSnUrachal RMSs were embryonal in 6, alveolar in 1, and not otherwise specified in 1. Age at diagnosis was 4.4 years (2.6-6). All patients had advanced locoregional extension (IRS IIIIV) and 1 had distant metastasis. All had chemotherapy and surgical resection. Six had external radiotherapy. Four had extensive peritoneal recurrence including 2 with distant metastasis, within a median of 25 months (11-82) after the end of treatment. One had metastatic progression under primary treatment. Four of them died between 18 and 57 months after diagnosis, and 1 is still under treatment for a late recurrence. Only 3 are free of disease after 3.3 to 7.9 years of follow-up.nnnCONCLUSIONSnPediatric urachal cancer is rare and poorly identified. In our series, RMS was exclusive in this location. Locoregional extension was always advanced. Prognosis was poor despite current multimodal therapy. We underline the need for new therapeutical strategies.

Living or Brain-dead Donor Liver Transplantation for Hepatocellular Carcinoma: A Multicenter, Western, Intent-to-treat Cohort Study.

Objective: An intent-to-treat analysis of overall survival (ITT-OS) of cirrhotic patients with hepatocellular carcinoma (HCC) listed for living donor liver transplantation (LDLT) or brain-dead donor liver transplantation (BDLT) across 5 French liver transplant (LT) centers. Background: Comparisons of HCC outcomes after LDLT and BDLT measured from time of transplantation have yielded conflicting results. Methods: Records from 861 cirrhotic patients with HCC consecutively listed for either LDLT (n = 79) or BDLT (n = 782) from 2000 to 2009 were analyzed for ITT-OS using a Cox model; and tumor recurrence using 2 competitive risk models. Results: Tumor staging was similar between groups. In total, 162 patients dropped out (20.7%), all from Group BDLT (P < 0.0001). The postoperative mortality rate and the retransplantation rate were similar between LDLT and BDLT. At 5 years, no statistically significant difference was found in ITT-OS between LDLT and BDLT groups (73.2% vs 66.7%; P = 0.062). LDLT waitlist inclusion (hazard ratio: 0.61 (0.39–0.96); P = 0.034) and a time-of-listing MELD score ≥ 25 (hazard ratio: 1.93 (1.15–3.26); P = 0.014) were independent predictors of ITT-OS. Similar 5-year post-LT OS rates (73.2% and 73.0% for Group LDLT and Group BDLT, respectively; P = 0.407) and HCC recurrence rates (10.9% and 11.2% for Group LDLT and Group BDLT, respectively; P = 0.753) were found. Upon explant analysis, tumors exceeding the Milan criteria, macroscopic vascular invasion, and AFP score>2 were independent predictors of recurrence, whereas LT type was not. Conclusions: LDLT improves ITT-OS, and it is not a risk factor for tumor recurrence. Therefore, LDLT and BDLT should be equally encouraged in countries where both are available.