Risk of fracture prevention in spina bifida patients: correlation between bone mineral density, vitamin D, and electrolyte values.

PURPOSE: The aim of our study was to investigate the relationship between bone mineral density (BMD), vitamin D, and electrolyte blood values in patients with spina bifida, to find a possible therapeutic regimen and an intervention to reduce the risk of fractures in this population. METHODS: BMD values were measured in 49 patients (32 females, 17 males; aged 14.1 ± 3.86 years; range 5-20 years) using dual-energy X-ray absorptiometry (DEXA) and were analyzed based on sex, the level of spinal involvement, vitamin D, and electrolyte values, physical activity, body mass index (BMI), and ambulatory status [patients were divided into three subgroups: full-time wheelchair (FTWC), limited ambulator (LA), and full-time ambulator (FTA)]. These data were analyzed considering sex-, age-, and BMD-matched values and compared with those of normal population. RESULTS: BMD was significantly lower in these patients compared with that in the general healthy population (Z-score: -1.2 ± 1.8); in particular, females had Z-score values significantly lower that of the males (Z-score: -2.43 ± 2.02; P < 0.0004). In FTWC subgroup, Z-score was lower than that of the other two subgroups (P < 0.009). Vitamin D values were significantly lower compared with those in the general healthy population (vitamin D spina bifida group: 14.6 ± 8.7 mg/dL; normal subjects: 35 ± 9.8 mg/dL; P < 0.001). Subjects with spina bifida showed hypophosphatemia (<3 mg/dL) because of the lower levels of vitamin D (3.1 ± 0.9 mg/dL; P < 0.001). CONCLUSIONS: Spina bifida patients showed lower BMD, vitamin D, and electrolyte values than the healthy population; hence, they have an increase risk of developing pathological fractures. Vitamin D supplementation for a longer time period could reduce this risk.

Functional hemispherectomy in children with epilepsy and CSWS due to unilateral early brain injury including thalamus: sudden recovery of CSWS.

PURPOSE: To try to prove in patients with refractory symptomatic epilepsy due to early brain injury involving thalamus and complicated by CSWS the effects of the isolation of the injured hemisphere, performed with functional hemisperectomy, on epilepsy, namely on CSWS. METHODS: Full clinical follow-up before and after surgery of two cases with CSWS onset at four years in whom functional hemispherecomy was performed with resection of inter-hemispheric connections. RESULTS: An immediate effectiveness of the surgical treatment was observed on both epileptic evolution (no more seizures) and EEG abnormalities. In particular, CSWS completely disappeared, together with a concurrent progressive improving of the cognitive and behavioural disorders. DISCUSSION: The isolation of the injured hemisphere through the section of inter-hemispheric cortico-cortical connections could prevent the contralateral diffusion of discharges coming from the injured cortex and cortico-thalamic network, favouring a normal function of thalamo-cortico-thalamic circuitries in the healthy hemisphere. That could explain the disappearance of CSWS after surgery in our patients and the consequent improvement of cognitive abilities and behaviour.

Organization of cortico-cortical associative projections in a rat model of microgyria.

Microgyria was experimentally induced by focal freezing lesions of the frontal cortex in newborn rats. Adult microgyric animals received cortical injections of biotinylated dextran amine combined with NMDA, in order to obtain a Golgi-like retrograde labeling of cortico-cortical association neurons. Injections were performed either rostrally or caudally to the microgyric lesion. Results demonstrate that long-range association projections traveling across the zone of the microgyric lesion arise mainly from infragranular layers. In normal animals the same projections originate both from supragranular and infragranular layers. The analysis of single basal dendrites of layer 2/3 in microgyric animals demonstrates a simplified branching pattern, with a number of end points lower than in control animals. Potential implications for microgyria-associated epilepsy are discussed.

Management of recurrent craniopharyngioma.

Although histologically benign, craniopharyngioma can regrow either from macroscopic remnants of the tumour left behind at operation, or even after an apparently gross total removal. Recurrence rates vary significantly in the literature, depending on the efficacy of surgical treatment and also on the growth potential of the tumour itself. The main factor influencing tumour regrowth is obviously the extent of surgical resection, as total removal carries a much lesser risk of recurrence compared to subtotal or partial resections (although in such cases radiation therapy can lower this risk significantly). Other factors involved are the duration of follow-up and patient's age at operation, as children tend to relapse more frequently than adults. Even in the "microsurgery" era, characterized by high percentages of total resections, recurrences remain high and continue to represent a major problem of craniopharyngioma treatment. Twenty-seven children and adolescents were operated on for craniopharyngioma at the Department of Neurosurgery, Section of Pediatric Neurosurgery, Catholic University Medical School, Rome between June 1985 and June 1997. Total tumour resection was achieved in 18 cases, subtotal in 7 and partial in 2 instances. One patient died post-operatively. Post-operative neuroradiological investigations confirmed the operative findings, although 3 children with an apparently gross total removal showed a residual non-enhancing calcium fleck adherent to the hypothalamus (which remained stable at the following examinations). Three of the 9 patients with less than total removal underwent post-operative radiation therapy. Out of the 26 surviving patients 6 presented a recurrence of their craniopharyngioma, 2 after an apparently gross total removal and 4 after a subtotal or partial resection (one of them had received radiation therapy). The diagnosis was merely neuroradiological in 5 cases, as only one child presented a clinical picture suggestive of tumour regrowth. Surgery was the first therapeutic option in all the cases. Total tumour resection was accomplished in 3 cases, subtotal in 2 and partial in the last one. One child died post-operatively. Four of the 5 survivors received radiation therapy. All the patients are presently alive and stable (mean follow-up: 5.6 yrs). The authors conclude that surgery should be the first therapeutic option in case of recurrent craniopharyngioma and that radiation therapy should also be considered but only as adjuvant therapy.

"No allogeneic blood transfusion" protocol for the surgical correction of craniosynostoses. II. Clinical application.

The authors describe the results obtained in 13 consecutive cases of craniosynostosis operated on according to a protocol devised at avoiding allogeneic blood transfusion. The protocol is based on pre- and postoperative treatment with erythropoietin, preoperative autologous blood donation, preoperative normovolemic hemodilution and intraoperative blood salvage. Nine subjects were affected by simple forms of craniosynostosis, whereas the remaining 4 presented with oxycephaly or craniofacial syndromes. Five of the 13 children were under 7 months and a further 3, under 10 months of age at the time of the surgical operation. Seven children weighed less than 10 kg. Allogeneic blood transfusion was avoided in 11 of the 13 children considered. Two failures - defined as the necessity to reinfuse the patient with an allogeneic blood transfusion - were recorded, 1 of them resulting from an unexpected hemorrhage during surgery. The results obtained indicate that this protocol designed to avoid allogeneic blood transfusion can be safely applied in the great majority of children with craniosynostosis, even when the surgical correction is carried out early in life.

"No allogeneic blood transfusion" protocol for the surgical correction of craniosynostoses. I. Rationale.

Improved anesthesiological and surgical care has resulted in a progressively declining need for allogeneic blood transfusion. In infants with craniosynostosis, however, allogeneic blood transfusion is still performed as a routine procedure. In the present paper, the authors describe a protocol they have devised with the aim of limiting or even avoiding allogeneic blood transfusion even in very young patients, consequently avoiding the risks of infective or immunologic reactions associated with the procedure. The protocol is based on stimulation of the hematopoietic system with erythropoietin, selection of an appropriate age for operation when a favorable balance between fetal and adult-type hemoglobin is established (that is after 4-6 months), preoperative preparation of the autologous blood supply, and intraoperative blood salvage.