CONGENITAL PORTOSYSTEMIC SHUNT: COMPLICATIONS AND OUTCOME AFTER CLOSURE: ABOUT 19 PEDIATRIC CASES
Stéphanie Franchi-Abella, Guillaume Thouvenin, Jean-Yves Riou,
Sophie Branchereau, Danièle Pariente
CHU de Bicêtre, Le Kremlin-Bicêtre (France)
Purpose - Objective: Congenital Porto-Systemic Shunts (CPSS) are rare vascular malformations that can lead to severe cardiopulmonary complications, hepatic tumors and hepatic encephalopathy. Feasibility of their closure is debated and its effects on these complications are not well known. We report a large paediatric series of CPSS after closure.
Material and methods: Between 1999 and 2009, 19 patients (range: neonate to 15 y.o., medium age 9.5 y) had a closure of CPSS. CPSS consisted of 3 patent ductus venosus, 3 porto-hepatic shunts, 10 side-to-side and 3 end-to-side communications between the portal vein and the IVC. Patients were explored by US, MDCT, abdominal and/or cerebral MRI and angiography with shunt occlusion test. Complications, technique of closure and outcome are described.
Results: The complications associated with the CPSS were hepatic tumors (11), pulmonary arterio-venous shunts (2), pulmonary hypertension (3) and hepatic encephalopathy (3). Closure of the shunt was performed surgically in 11, with interventional radiology in 6 and combined techniques in 2. None developed significant long-term portal hypertension. Benign hepatic tumors disappeared in 8 and decreased in 2. Pulmonary shunts disappeared in 1; pulmonary hypertension remained stable in 3. Encephalopathy resolved in all.
Discussion and conclusion: CPSS can be closed whatever its location. Complications secondary to the shunt may resolve or improve after closure except pulmonary hypertension that may only stabilize. Preventive closure of CPSS should be discussed.
PRIMARY ANEURYSMAL BONE CYSTS IN CHILDREN: PERCUTANEOUS ETHANOL INJECTION AND PROPOSAL OF A VASCULAR CLASSIFICATION
Karen Lambot-Juhan, Zagorka Péjin, Stéphanie Pannier, David Grévent, Laureline Berteloot, Sophie Emond-Gonsard, Nathalie Boddaert, Phalla Ou, Christophe Glorion, Francis Brunelle
Hôpital Necker Enfants Malades, Paris (France)
Purpose - Objective: Percutaneous sclerotherapy is an effective treatment for biopsy-proven aneurismal bone cyst (ABC). We report our experience about 29 children treated with Ethanol and propose a vascular classification of the ABC based on aspect of aspirated liquid and drainage per-procedure.
Material and methods: From January 1995 to November 2009, we reviewed 29 patients from 2 to 16 years (mean 9.6 years) who underwent percutaneous Ethanol injection for ABC. Upper extremity was involved in 19 patients, lower extremity in 5, pelvic bone in 3 and spine in 2. Procedure was under general anaesthesia and fluoroscopy. Appearance of the cyst fluid was clear, partially bloody, or bloody. Drainage was absent or venous. Treatment response was considered good when no clinical complain and X-ray significative ossification, fair when one of those conditions was not present and failure when none was.
Results: Cyst fluid and drainage were available in 21 patients. No drainage in 11 among 14 clear and partially bloody liquid cysts, classified as lymphatic. Drainage in the 7 bloody liquid cysts, classified as venous. The mean follow up was 2 years 6 months (3 months-7.5 years). Treatment response was good in 20 cases, fair in 4, failure in 2.
Discussion and conclusion: ABC can be classified as lymphatic or venous and be considered as bone vascular malformation, treated as of soft tissue malformations. Ethanol injection is a safe and effective treatment of ABC.
UNILATERAL SENSORINEURAL HEARING LOSS IN CHILDREN: MRI OR CT FIRST?
Bogdana Tilea, Marianne Alison, Robin Azoulay, Guy Sebag, Monique Elmaleh-Bergès
Hôpital Robert Debré, AP-HP, Paris VII University, Paris (France)
Purpose - Objective: To evaluate the incidence of inner ear and CNS abnormalities in children with unilateral sensorineural hearing loss (USNHL) identified with CT and/or MRI.
Material and methods: Retrospective case review of children referred for USNHL in a tertiary care children’s hospital between 1999 and 2009.
Results: From a database of 3078 children consecutively investigated by CT and /or MRI for ear disorders, 234 had a USNHL. 143 children underwent CT only (61%), 40 MRI only (17%) and 51 (22%) both CT and MRI. 111 examinations were normal (47.4%). 44 (19%) labyrinthine malformations were identified (33 unilateral et 11 bilateral). CT depicted cochlear nerve canal (CNC) and/or internal auditory meatus (IAM) stenosis in 26 cases (11%). MRI confirmed aplasia/ hypoplasia of cochlear nerve in 10 of these cases and demonstrated 11 (4.7%) more cases. Dysmorphic, non stenotic, IAM was found in 10 cases (4.2%). Labyrinthine anomalies due to infection or traumatism were identified on CT in 16 cases (7%) and an abnormal cochlear signal was found in 4 cases (1,7%). Associated or isolated cerebral anomalies were found with MRI in 19 children (8.1%).
Discussion and conclusion: This study confirms the prevalence of inner ear malformations among UNSHL aetiologies in children; cochlear nerve aplasia/hypoplasia is nearly as common as labyrinthine malformations, and only assessed by MRI as well as brain abnormalities.
DIFFUSION TENSOR IMAGING IN ATTENTION DEFICITS IN CHILDREN TREATED FOR POSTERIOR FOSSA TUMOURS: PRELIMINARY RESULTS
Muriel Brun1, Marina Walker2, Olivier Périot3, Sandrine Delord2,
Anne Notz4, Geneviève Lyard4, Michèle Allard1
Imagerie CHU Bordeaux1, Laboratoire de Psychologie2, UMR-CNRS 52313, Université Bordeaux 2, Réseau de Cancérologie d’Aquitaine pédiatrique4, Bordeaux (France)
Purpose - Objective: Among surviving children treated for posterior-fossa tumours, deficit in cognitive development, involving mainly attention and working memory processes, have been observed.. Radiation therapy seems to play a predominant role in anatomical lesions supporting these deficits. As white matter is recognized to be the most vulnerable element to radiation injury in the brain, our purpose is to evaluate brain white matter, in children treated for posterior fossa tumours, using diffusion tensor imaging (DTI).
Material and methods: Between 2000 and 2006, 20 consecutives posterior-fossa tumours treated-patients survived in our institution. Among these patients, eight (aged from 7y-11m to 15y-3m) were enrolled in a retrospective study. The design of this study consisted in a neuropsychological evaluation, a 3D and a 16 directions DTI acquisitions. One patient was secondarily excluded due to artefact that not allowed correct images analyses. A group of 7 age-matched control subjects was also enrolled.
Results: Patients exhibited a diffuse decreased mean diffusion (MD) compared to controls. This MD decrease was related to both parallel diffusion (L1) and radial diffusion (L2) decreases.
Discussion and conclusion: Even if these results appeared counterintuitive, they could reflect different processes, i.e. remylination for radial diffusion decrease and reduced axonal diameter for parallel diffusion decrease. Studies are in progress to evaluate the cognitive efficiency of these morphological processes during the post-treatment chronic phase.