Volkan Etus

Success of endoscopic third ventriculostomy in children less than 2 years of age

Current literature reveals different opinions about the effectiveness of endoscopic third ventriculostomy in the treatment of hydrocephalus in children less than 2 years of age. Performing a retrospective evaluation of our own experience in this age group, we aimed to contribute to the growing data on the controversial issues related to this procedure in children. In a series of 97 endoscopic third ventriculostomy procedures, 25 were performed in children less than 2 years of age as an initial treatment for hydrocephalus. A retrospective analysis of our data revealed that the overall success rate of endoscopic third ventriculostomy in this age group was 56%. However, analysis of the results in subgroups with different etiologies of hydrocephalus showed that the success rate of the procedure was 83% in patients with defined anatomic obstruction, 66.6% in post-hemorrhagic hydrocephalus, 50% in infection related hydrocephalus and 41.6% in hydrocephalus accompanied by myelomeningocele. This article considers our data and the features of endoscopic third ventriculostomy procedure in this age group, with a detailed review of the literature. In our experience, the success of endoscopic third ventriculostomy is etiology related rather than age-dependent. We suggest that there are no grounds for denying children younger than 2 years this chance for a shunt-free life.

Topical application of mitomycin C prevents epineural scar formation in rats

The role of topically applied mitomycin C in preventing postoperative perineural fibrosis was examined by gross anatomical dissection and histological analysis in rats. The sciatic nerve was exposed bilaterally in 24 Wistar adult male rats, and an abrasion injury was produced on the exposed surface of the biceps femoris muscle in all animals. In the experimental group, cotton pads soaked with mitomycin C (0.5 mg/ml) were placed around the nerves for 5 min, whereas cotton pads soaked with saline were applied to the control group. Four weeks after surgery, the neurolysis sites were evaluated by blinded surgical dissection. Perineural adhesions were graded using a numerical grading scheme. The scar tissue formation index was also calculated, and a grading was made according to the number of fibroblasts/fibrocytes counted around the epineurium in histological evaluation. Mitomycin C-treated nerves showed significantly less perineural adhesions than controls. Quantification of the dense connective tissue surrounding the nerves revealed a statistically significant reduction around nerves treated with mitomycin C, and the number of fibroblast/fibrocytes was also significantly reduced. Application of topical mitomycin C might be effective in preventing epineural scar formation after neurolysis of peripheral nerves.

Risk of strabismus and ambylopia in children with hydrocephalus.

ObjectivesThe present study was undertaken to determine the risk of strabismus and ambylopia in children who underwent operation for hydrocephalus and to compare our results with those in previous studies.MethodsFull orthoptic and ophthalmological examinations, including cycloplegic refraction, were performed in all subjects.ResultsTen of 25 patients (40%) were found to have manifest squint. Four of these had esotropia and six had exotropia. No paretic squint or alphabetic pattern strabismus was determined. Refraction measurements revealed amblyogenic refractive errors (significant refractive errors that cause amblyopia) in five of the 25 (20%) patients in this study. Strabismus and the risk of amblyopia were found to be significantly higher in patients who experienced shunt revision than those who had not (P<0.05).ConclusionAmblyopia, strabismus, and other acquired defects in the visual system related to hydrocephalus should be closely monitored and treated when indicated. Regular ophthalmic supervision will provide and help to maintain the best possible standard of vision in children with hydrocephalus.

Association of spondylocostal dysostosis and type I split cord malformation

Abstract.In reports on children with congenital segmental costovertebral malformations who showed neural tube defects, cases with type I split cord malformation are quite rare. Up to now such association has been reported only in two cases with Jarcho-Levin syndrome. Here, a 7-year-old girl presenting with spondylocostal dysostosis and type I split cord malformation is reported. To the best of our knowledge, this is the first case documented in the literature. The association of segmental costovertebral malformations and neural tube defects is discussed. Genetic and embryological studies are also briefly reviewed.

Role of Interpeduncular and Prepontine Cistern Cerebrospinal Fluid Flow Measurements in Prediction of Endoscopic Third Ventriculostomy Success in Pediatric Triventricular Hydrocephalus

Aim: The aim of the present study was to evaluate the correlation of the clinical success of the endoscopic third ventriculostomy (ETV) procedure with the measurements of cerebrospinal fluid (CSF) flow through the interpeduncular and prepontine cisterns in pediatric triventricular hydrocephalus. Methods: 51 children (age range: 25–201 months; mean: 55.3 months) with primary aqueductal stenosis who have been treated with ETV were included in the study. All patients were analyzed by conventional and cine magnetic resonance imaging (MRI) before and after endoscopic treatment. Both the stroke volume and the flow velocity of CSF in the interpeduncular and prepontine cisterns were evaluated. The MRI findings were correlated with clinical outcome. Simple descriptive and independent t tests were used for statistical analyses, and receiver operating characteristic curve analysis was used for the cutoff value of the ‘flow index’ (FI). Results: Among the 51 patients, 37 patients (72.5%) responded to ETV clinically during long-term follow-up. A further analysis was made for the prediction of who would respond to ETV, using the formula (stroke volume of prepontine cistern/stroke volume of interpeduncular cistern) ×100, which was defined as the FI. Early postoperative FI values of over 40% were significantly correlated with ETV success. Conclusion: Our results suggest that ETV success can be predicted by the ratio of early CSF stroke volume values of the interpeduncular and prepontine cisterns.

Desmoplastic non-infantile astrocytic tumor with BRAF V600E mutation

Desmoplastic infantile astrocytomas (DIA) are rare neoplasms of infancy which are defined by a combination of distinctive clinicopathologic features. DIA was originally defined in 1982 by Taratuto et al. as meningocerebral astrocytoma attached to dura with desmoplastic reaction. In 1993, it was included in the WHO classification under the term ‘desmoplastic cerebral astrocytoma of infancy’ [1]. DIA accounts for 1.25 % of pediatric brain tumors [2]. The large majority of cases presents within the first 24 months of life [1, 3]. Non-infantile cases are rarely encountered with only eight cases reported before [3–10]. However, in two of these cases, clinical symptoms ensued within the first year of life [4, 5]. Less is known about the molecular etiology of DIA [1, 11]. Recent studies suggest that certain types of mostly low grade and pediatric brain tumors may have higher rates of BRAF alterations. BRAF V600E mutations have been detected in small series of pediatric gangliogliomas, pleomorphic xanthoastrocytomas, desmoplastic infantile gangliogliomas and atypical teratoid/ rhabdoid tumors as well as pediatric glioblastomas, anaplastic astrocytomas and diffuse astrocytomas [11–15].

Double myelomeningocele in a neonate: Case report and review of the literature

The presence of myelomeningocele at multiple levels along the spinal column is extremely rare. There are only a few cases of double myelomeningocele reported in the literature. We report the case of a newborn male who had two myelomeningoceles with one at the cervical level and the other at the lumbar level. This unusual case report as well as a review of the literature is presented.

Ventriculoperitoneal shunt catheter protrusion through the anus: case report of an uncommon complication and literature review

In this report, Glatstein et al. have presented an 11-year-old girl with rectal protrusion of the ventriculoperitoneal (VP) shunt tube, and they have summarized the 23 cases of anal protrusion of VP shunt reported in the literature. The most frequent (45%) finding in delayed bowel perforation by VP shunt catheters is protrusion of the distal catheter from the anus [1]. In my opinion, this protrusion seems like a chance for the patient as it helps the early diagnosis of perforation as in the case presented herein. The authors have also discussed the pathogenesis of delayed bowel perforation by VP shunt catheter. As the authors have indicated, children with myelomeningocele were reported to be more susceptible to bowel perforation, possibly due to their weaker bowel musculature [2]. In the literature, there are also several other explanations, which should have been emphasized in the report. One of those possible mechanisms was proposed by Brownlee et al. [3], and they suggested that allergic reaction to silicone might lead to adherence of shunt tubing to the intestinal wall with subsequent erosion into the lumen. In another report, Miserocchi et al. pointed out that the pathogenesis was most likely to be related with local infective adhesions of the bowel [4]. Among the reasons for protrusion of the VP catheter from the anus, trauma during the operation should also be taken into consideration. A minor trauma to the bowel may cause a local inflammation which may lead the bowel wall to become more susceptible to the mechanical irritation by the catheter tip. Today, the pathogenesis of delayed bowel perforation by VP shunt catheter is not clear yet. However, we may assume that there is more than one possible mechanism for delayed bowel perforation by VP shunt catheter.

Surgical technique and outcome in cervical and thoracic myelomeningocoele surgery.

Cervical and thoracic myelomeningocoeles differ from common lumbosacral myelomeningocoeles in many respects. We review the surgical technique and outcome achieved for a series of six infants who underwent surgery for cervical or thoracic myelomeningocoele. Five patients, who had intradural exploration and microsurgical untethering of the spinal cord, were neurologically stable on follow-up. The other patient, who had a simple subcutaneous resection of the sac without release of the intradural tethering bands, was re-operated on 16 months later, with progressive neurological symptoms due to cord tethering. Following re-exploration and microsurgical untethering of the spinal cord, the neurological deficits significantly improved. We suggest that the surgical technique in these lesions should include careful intradural exploration and microsurgical release of the spinal cord by meticulous resection of all tethering bands. This enables postoperative neurological improvement and possible prevention of future neurological deficits due to cord tethering.

Multiple sclerosis and coexisting intradural extramedullary spinal cord tumour: a case report.

Abstract. We report the coexistence of multiple sclerosis (MS) and an intradural extramedullary spinal cord tumour in a 46-year-old woman with a 2-year history of MS. The patient presented with right hemitrunk and lower extremity paraesthesias, urinary incontinence, and intermittent lower right back and abdominal pain, which did not respond to pulse steroid therapy. A spinal magnetic resonance imaging (MRI) study revealed an intradural extramedullary spinal cord tumour in the lower thoracic spine, later diagnosed as schwannoma. We call attention to this rare association of MS and a spinal cord tumour, and emphasize the need for scrutiny of new and uncommon symptoms during the follow-up of MS patients.