Urodynamic score in children with lipomyelomeningocele: a prospective study.

PURPOSE: We assessed the correlation between urodynamic score and presence of symptoms in children with lipomyelomeningocele, and the usefulness of this measure in monitoring these children. MATERIALS AND METHODS: We prospectively evaluated 29 patients 1 month to 13 years old (mean 1.9 years) with lipomyelomeningocele from 1995 to 2010. A 4-parameter urodynamic score ranging from 0 to 17 was assigned to each patient at diagnosis and followup. Indications for surgery were presence of symptoms and signs or abnormal urodynamic score. Children were divided into 2 groups, symptomatic and asymptomatic. The latter group was further divided into operated and conservatively managed cases. Average followup was 6.7 years (range 2 to 16). RESULTS: The symptomatic group contained 11 children (38%) operated on at a mean age of 2.9 years. Mean followup was 6.7 years (range 2 to 12). All patients presented with an abnormal urodynamic score. Postoperative urodynamic score improved in 6 patients (55%), remained abnormal in 4 (36%) and deteriorated in 1 (9%). The asymptomatic group contained 18 patients, of whom 7 (39%) were operated on at a mean age of 1.4 years. Mean followup was 10 years (range 3 to 16). Postoperative score improved in 6 patients (86%) and remained unchanged in 1 (14%). A total of 11 cases (61%) were managed conservatively, with a mean followup 4.3 years (range 2 to 7). Of these patients 3 (27%) presented with late urodynamic score deterioration at 3, 5 and 6 years of followup while remaining asymptomatic. CONCLUSIONS: Urodynamic score is a useful tool in monitoring children with lipomyelomeningocele because it has a good correlation with the presence of symptoms.

Cervical nerve root synovial sarcoma in a child with chromosomal (X;18) translocation. Case report and review of the literature.

We report on an 11-year-old female with a history of cervicobrachialgia and progressive weakness of the right arm. Cervical spine MRI showed an enhancing heterogeneous intradural mass occupying the right C6-C7 foramen. She underwent a right C6-C7 foraminotomy with a complete macroscopic removal of the lesion. Pathological examination revealed a synovial sarcoma. Treatment was completed by chemotherapy and proton radiotherapy, and the girl remained free of symptoms for 3 years. After appearance of new symptoms, a local recurrence was confirmed, and despite aggressive treatment with salvage chemotherapy and radiotherapy, the disease progressed beyond medical control, and the child died, 6 years after diagnosis. Early recognition of this rare entity compared to its more benign differential diagnosis is crucial, as an aggressive management is needed.

The Geneva and Lausanne (French-speaking Switzerland) experience: in favor of the transsphenoidal approach when feasible.

BACKGROUND: The authors present their current attitude towards management of craniopharyngiomas in children. Radical surgery cannot be performed when one is not sure about its potential danger to the visual pathways and the hypothalamus. METHODS: Most of the surgeries that have been performed in our institution via an intracranial approach were incomplete and followed by radiotherapy, in the last 10 years with stereotactic conformational radiotherapy. DISCUSSION: Considering our past and present experience concerning the transphenoidal approach for treatment of craniopharyngiomas, we make a plea for the consideration of this approach in children whenever feasible (around 25% of the cases in children older than 5 years). Craniopharyngiomas that can be removed by this approach represent a milder form of the disease and the results concerning the visual, hypothalamic functions and quality of life are significantly better than that obtained via an intracranial approach. When the craniopharyngioma cannot be removed by this approach because of tight hypothalamic adherences, the technique of cystosphenoidostomy with a custom-made shunt plus adjuvant stereotactic conformational radiotherapy is an alternative for its total removal.

Introduction of a urodynamic score to detect pre- and postoperative neurological deficits in children with a primary tethered cord.

OBJECT: An increasing number of asymptomatic children are diagnosed with occult spinal dysraphism, raising the question of their optimal management. Urodynamic study (UDS) is the most reliable method of detecting neuro-urological abnormalities in these children. The rate of postoperative retethering ranges from 10 to 20% and is not always immediately clinically significant. The aim of this prospective study was to develop a reliable method that could be used in the preoperative assessment and postoperative follow-up of children with a tethered cord syndrome (TCS). METHODS: From 1989 to 1997, 15 children underwent spinal cord untethering for TCS. Preoperatively, patients were assessed with MRI and UDS. Postoperative UDS were repeated at 6- to 12-month intervals. Four UDS parameters were identified, graded, and added to obtain a UDS score. A group of 38 children without dysraphic condition was used as control and allowed the calculation of a normal score. CONCLUSIONS: There was a statistically significant difference in the preoperative UDS scores between the control group and the study group ( p<0.001). Postoperatively, there was a statistically significant improvement ( p<0.001) in UDS scores. UDS score is a reliable tool for identifying and quantifying neuro-urological disorders in patients with TCS. Postoperatively, this score was useful in the early diagnosis of spinal cord retethering.

[Management of simple cranial injuries and minor cranio-cerebral injuries in children]. / La prise en charge des traumatismes crâniens simples et traumatismes crânio-cérébraux mineurs de l'enfant.

Owing to the frequent occurrence of minor head injuries in their daily practice, pediatricians and primary care physicians have a constant concern about their management. Evaluation of a neurotraumatised children must take account of the epidemiological, anatomical, pathophysiological and clinical specificity of each age class. Recent publications of the American Academy of Pediatric, with evidence based medicine review of the literature and experts consensus have permitted to define precise guidelines for the management of children over and below 2 years of age. Taking account in the initial evaluation of the indirect signs of severity and pediatric mechanisms criteria allows to better define the principles of triage.

What and where in human audition: selective deficits following focal hemispheric lesions.

A sound that we hear in a natural setting allows us to identify the sound source and localize it in space. The two aspects can be disrupted independently as shown in a study of 15 patients with focal right-hemispheric lesions. Four patients were normal in sound recognition but severely impaired in sound localization, whereas three other patients had difficulties in recognizing sounds but localized them well. The lesions involved the inferior parietal and frontal cortices, and the superior temporal gyrus in patients with selective sound localization deficit; and the temporal pole and anterior part of the fusiform, inferior and middle temporal gyri in patients with selective recognition deficit. These results suggest separate cortical processing pathways for auditory recognition and localization.