Development and application of a diagnostic and severity scale to grade post-operative pediatric cerebellar mutism syndrome.

The post-operative pediatric cerebellar mutism syndrome (CMS) affects about one-third of children and adolescents following surgical removal of a posterior fossa tumor (PFT). According to the Posterior Fossa Society consensus working definition, CMS is characterized by delayed-onset mutism/reduced speech and emotional lability after cerebellar or 4th ventricle tumor surgery in children, and is frequently accompanied by additional features such as hypotonia and oropharyngeal dysfunction/dysphagia. The main objective of this work was to develop a diagnostic scale to grade CMS duration and severity. Thirty consecutively referred subjects, aged 1-17 years (median 8 years, IQR 3-10), were evaluated with the proposed Post-Operative Pediatric CMS Survey after surgical resection of a PFT and, in case of CMS, for 30 days after the onset (T0) or until symptom remission. At day 30 (T1), CMS was classified into mild, moderate, or severe according to the proposed scale. CMS occurred in 13 patients (43%, 95% C.I.: 25.5-62.6%), with mild severity in 4 cases (31%), moderate in 4 (31%), and severe in 5 (38%). At T1, longer symptom persistence was associated with greater severity (p = 0.01). Greater severity at T0 predicted greater severity at T1 (p = 0.0001). Children with a midline tumor location and those aged under 5 years at diagnosis were at higher risk of CMS (p = 0.025 and p = 0.008, respectively). In conclusion, the proposed scale is a simple and applicable tool for estimating the severity of CMS at its onset, monitoring its course over time, and providing an early prognostic stratification to guide treatment decisions.

The role of Ommaya reservoir and endoscopic third ventriculostomy in the management of post-hemorrhagic hydrocephalus of prematurity.

OBJECTIVE: The aim of this study is to retrospectively evaluate a series of consecutive patients affected by post-hemorrhagic hydrocephalus in prematurity, treated with an implant of an Ommaya reservoir followed by ventriculo-peritoneal (VP) shunt and/or endoscopic third ventriculostomy (ETV) to evaluate the safety and efficacy of these treatment options in the management of the condition. METHODS: Between 2002 and 2005, 18 consecutive premature patients affected by intra-ventricular haemorrhage (IVH) grades II to IV, presenting with progressive ventricular dilatation, were operated for implant of an intra-ventricular catheter connected to a sub-cutaneous Ommaya reservoir. Cerebrospinal fluid was intermittently aspirated percutaneously by the reservoir according with the clinical requirements and the echographic follow-up. The patients who presented a progression of the ventricular dilatation were finally operated for VP shunt implant or ETV according with the MRI findings. RESULTS: One patient had grade II, 5 had grade III, and 12 had grade IV IVH. The mean age at IVH diagnosis was 5.2 days; the mean age at reservoir implant was 17.3 days. The Ommaya reservoir was punctured on an average basis of 11.4 times per patient (range 2-25), and the mean interval between aspirations was 2.7 days. The mean CSF volume per tap was 20 ml. One patient died for pulmonary complications during the study period. Out of the 17 survivors, 3 did not develop progressive ventricular dilatation, and their reservoir was removed; 14 developed progressive hydrocephalus, 5 of whom were implanted with a VP shunt and 9 received an ETV. Amongst the five shunted patients, two were re-admitted for shunt malfunction and had their shunt removed after ETV after 6.1 and 20.5 months, respectively. Amongst the nine patients who received an ETV, five had to be re-operated for VP shunt implant at an average interval of 2.17 months (range 9-172 days) because of increasing ventricular dilatation. Two of them had a redo third ventriculostomy with shunt removal at 11 and 25.1 months, respectively, after insertion. The first was reimplanted with a VP shunt 4 days later; the second remains shunt free. Therefore, at the end of the follow-up period, 10 out of 17 children affected by post-hemorrhagic hydrocephalus in prematurity were shunt free (59%). CONCLUSIONS: The combination of Ommaya reservoir, VP shunt, and the aggressive use of ETV as a primary treatment or as an alternative to shunt revision allowed for a significant reduction of shunt dependency in a traditionally shunt-dependent population. Further studies are warranted to optimise the algorithm of treatment in these patients.

Osteogenesis induced by autologous bone marrow cells transplant in the pediatric skull.

BACKGROUND AND PURPOSE: The ability of cranial bone to repair defects of continuity is limited and it is mostly dependent on the age of the patient. In infancy and in early pediatric age, the scarce thickness of the calvarial bones and the need for a harmonic development of the child's skull limit the application of most of the surgical procedures usually utilized in older patients. We tested the ability of mononucleated cells, derived from the patient's bone marrow and transplanted on the site of the cranial bone defect, to increase the rate of mineralization of the autologous osteogenesis to obtain the complete restoration of the skull continuity. METHOD: Four children, aged 26, 28, 37, and 79 months, respectively, affected by a stabilized and persistent cranial bone defect of posttraumatic or postsurgical origin, were treated. A sandwich-shaped shell, made of extrused absorbable polylactic copolymers material, was used to hold in place a freeze-dried mineralized collagen matrix associated with a nonceramic hydroxyapatite scaffold, where autologous bone marrow mononucleated cells were inseminated. RESULTS: In all patients, a rapid autologous bone osteogenesis was observed with a clear dimensional reduction of the bone defect few months after the autologous bone marrow cells seeding. CONCLUSIONS: The preliminary results of this research suggest the use of autologous bone marrow cells to increase the autologous osteogenesis in early pediatric age in cases in which correction of skull bone defects is best realized with autologous bone.

Dendritic architecture of corticothalamic neurons in a rat model of microgyria.

MATERIALS AND METHODS: Freezing lesions of the sensorimotor cortex resembling focal human microgyria were carried out in newborn rats on first postnatal day (P1). The fine architecture of dendritic trees of corticothalamic neurons was studied in microgyric animals on P90. RESULTS AND DISCUSSION: Compared with controls, layer 5 corticothalamic neurons located in the cortex surrounding the experimental lesion display longer basal dendrites. This finding is discussed with reference to earlier results on dendrites of layer 3 associative neurons, which, in contrast to those of corticothalamic neurons, appear to be markedly simplified.