Patient-specific biodegradable implant in pediatric craniofacial surgery.

Surgical correction of premature fusion of calvarial sutures involving the fronto-orbital region can be challenging due to the demanding three-dimensional (3D) anatomy. If fronto-orbital advancement (FOA) is necessary, surgery is typically performed using resorbable plates and screws that are bent manually intraoperatively. A new approach using individually manufactured resorbable implants (KLS Martin Group, Tuttlingen, Germany) is presented in the current paper. Preoperative CT scan data were processed in iPlan (ver. 3.0.5; Brainlab, Feldkirchen, Germany) to generate a 3D reconstruction. Virtual osteotomies and simulation of the ideal outer contour with reassembled bony segments were performed. Digital planning was transferred with a cutting guide, and an individually manufactured resorbable implant was used for rigid fixation. A resorbable patient-specific implant (Resorb X-PSI) allows precise surgery for FOA in craniosynostosis using a complete digital workflow and should be considered superior to manually bent resorbable plates.

Decompressive craniectomy in severe cerebral venous and dural sinus thrombosis.

OBJECTIVE: To evaluate the outcome of patients with most severe cerebral venous and dural sinus thrombosis (CVT) after decompressive craniectomy. Indications and techniques for decompressive craniectomy and intensive care regimen are discussed. METHODS: Between 2000 and 2004 15 patients with CVT and intracerebral hemorrhage were treated at the Department of Neurosurgery, University Hospital Zurich. Among them, four patients with the most severe illness course were treated with decompressive craniectomy. Indications for decompressive craniectomy were deterioration of level of consciousness with CT signs of space occupying brain edema, venous infarction and congestional bleeding with mass effect, midline shift and obliteration of the basal cisterns. RESULTS: Among 15 patients with CVT and intraparenchymatous hemorrhage four patients were treated with decompressive craniectomy. Glasgow Coma Scale (GCS) immediately before the operation was in mean 10.2 (range 6 to 13). No patient showed signs of unilateral or bilateral third nerve palsy before surgery. No surgical complications were observed. All four patients who underwent decompressive craniectomy recovered with favourable functional outcome (Glasgow Outcome Scale; GOS 4 and 5). Anticoagulation therapy with heparin was reconvened 12 hours postoperatively with half dosage and 12 hours later with full dosage. No enlargement of existing intraparenchymatous hematoma or other intracranial bleeding complications occurred. CONCLUSIONS: Favorable functional outcome in selected patients with most severe courses of CVT can be achieved after decompressive craniectomy. Postoperative anticoagulation therapy with full dose heparin 24 hours after craniotomy seems to be safe. Precise indications and techniques for combined surgical decompression and thrombectomy deserve to be evaluated in future studies.

Thoracolumbar intradural extramedullary bronchiogenic cyst.

Intradural extramedullary bronchiogenic cysts are rare findings. All five reported cases were located cervically or upper thoracically. To our knowledge, we describe the first case of an intraspinal bronchiogenic cyst in a thoracolumbar location. We present the case of a 41-year-old patient with a known spina bifida occulta who suffered from a continuous, sharp, and therapy-refractory pain in the left leg. Magnetic resonance imaging of the thoracic and lumbar vertebra revealed an intradural extramedullar mass at T12 to L1 level. After laminectomy T-12 through L-1/L-2 and longitudinal opening of the dura mater, the cystic mass was shown to be attached to the conus medullaris and the cauda equina, and therefore could be removed only partially. Histopathological examination revealed the diagnosis of bronchiogenic cyst. We therefore conclude that intradural extramedullary bronchiogenic cysts may appear also at thoracolumbar levels. Surgical resection can be achieved with good outcome.

Obstetrical brachial plexus palsy: an analysis of 105 cases.

Obstetrical brachial plexus palsy (OBPP) remains a dramatic consequence after complicated childbirth. An increasing number of methods are being developed for the physical therapy and the early repair of the nerve lesions in OBPP, including neuroma excision and nerve grafting, neurolysis and neurotization. Secondary deformities of the shoulder, forearm, and hand can be reconstructed using soft tissue and skeletal procedures. In this article we analyze our approach to 105 patients to obtain optimal functional outcome in patients with OBPP.

[The biosafety of the HSV-TK/GCV gene therapy in five cases of recurrent glioblastoma multiforme].

Herpes simplex virus-thymidine kinase/ganciclovir (HSV-TK/GCV) therapy was performed in five cases of recurrent glioblastoma multiforme. In the last study, the authors demonstrated response of the HSV-TK/GCV therapy against tumor progression (Adachi N, et al.: No Shinkei Geka). The aim of this study is to estimate the biosafety of in vivo HSV-TK gene transfer and GCV administration in five cases. Six parameters were analyzed sequentially up to the 6th month after the vector producer cells (VPCs) inoculation as follows; i) clinical symptom, ii) vital sign, iii) peripheral blood cell count, iv) blood biochemical analysis, v) serological test, vi) molecular biological test in peripheral leukocytes. In addition, ten systemic organs extracted from the two subjects in whom death occurred were also analyzed biologically. One case suffered from transient deterioration of left hemiparesis on the 34th day, which could be considered a probably-related but not adverse event. Serological tests detected anti-VPC antibody at the 1st month in one case and anti-vector antibody at the 1st and 4th month in another. The other examinations revealed no abnormal findings at all. These data indicate that the HSV-TK/GCV therapy might be a satisfactorily safe approach against glioblastoma multiforme.

[The HSV-TK/GCV gene therapy in five cases of recurrent glioblastoma multiforme].

Herpes simplex virus-thymidine kinase/ganciclovir (HSV-TK/GCV) gene therapy was performed in five cases of recurrent glioblastoma multiforme. The mean age of cases, three males and two females, was 60 +/- 5 years old. All of the tumors were confirmed pathologically as glioblastoma multiforme and recurred after the initial treatments (surgery and irradiation). A total number of 1 x 10(9) vector producer cells (VPCs), which produce retroviral vectors containing the HSV-TK gene, was inoculated into the tumor-bed spaces after removal of the recurrent tumors. From the following 14th day to the 27th day, GCV was transfused 5 mg/kg i.v. twice a day. The effect of the HSV-TK/GCV gene therapy was evaluated by the Karnofsky performance scale and MRI findings sequentially, before the therapy and in the 1st, 2nd, 4th and 6th month after the VPCs inoculation. During the follow-up period of 12 months, two cases died (survival period; 8.4 and 9.9 months), whereas the other three are still alive for over 12 months (1-year survival; 60%). Karnofsky performance scale showed the maximum at the 2nd and 4th month in all cases; the mean performance rating of living cases was 80% and that of dead cases was 70%. MRI revealed progression in none of the cases until the 2nd month. These results obtained in five cases suggest that the HSV-TK/GCV gene therapy may promise a feasible approach against glioblastoma multiforme.

Neonatal Escherichia coli meningitis: spinal adhesions as a late complication.

We describe two boys who had severe spinal complications in adolescence after a favorable initial recovery from neonatal Escherichia coli meningitis. Due to spinal granulomatous adhesions, one boy died after an attempted scoliosis operation (high cord lesion). The other showed severe progressive neurological deterioration with spinal and cerebellar symptoms. Conclusion The severe complication of chronic arachnoiditis with spinal adhesion may occur many years after neonatal acute bacterial meningitis.

Anticipation in familial cavernous angioma: ascertainment bias or genetic cause.

OBJECTIVES: Anticipation has been linked to unstable trinucleotide repeats in many neurological disorders. We examined the hypothesis of genetic anticipation in familial cavernous angioma (FCA) of the central nervous system. MATERIAL AND METHODS: The mean ASO of affected individuals was compared between successive generations in 55 families. Intergenerational pair-wise comparisons were employed to avoid several ascertainment biases. Regarding severity of disease both type of manifestation and number of cavernous angiomas were compared between generations. RESULTS: The mean ASO decreased significantly both from the first to the second generation (31.6 vs 17.8 years; P = 0.000) and from the second to the third generation (17.8 vs 6.7 years; P = 0.002). The pair-wise comparisons also showed significantly earlier ASO. No clear evidence for anticipation with regard to severity of disease was found. CONCLUSIONS: Molecular genetic studies will determine whether trinucleotide repeats are the underlying mechanism for our observation of anticipation in FCA.