[Show abstract][Hide abstract] ABSTRACT: Occipito-condylar hyperplasia is a very rare anomaly of the cranio-vertebral junction that was only reported in two patients before and managed through posterior approach.
A 10-year-old girl with a sudden attack of quadriparesis and respiratory distress was admitted to our center. A detailed work up favored a high cervical myelopathy due to bilateral occipito-condylar hyperplasia and Chiari malformation.
An endoscopic endo-nasal approach under navigation guide was used to drill the compressive lesion.
Our patient is added to the literature as the third one that was approached through a different surgical corridor. Successful decompression with excellent results was gained.
Child s Nervous System 05/2015; 31(7). DOI:10.1007/s00381-015-2717-3 · 1.11 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Endoscopic procedure has been known as the method of choice for treatment of hydrocephalus with 8.5% complication rate. It seems that good dural closure, reconstructing bone defect and perfect pericranium suturing can decrease the wound complications. Here, we describe the method of minicraniotomy instead of the burr hole in the endoscopic procedure.
A case-control study regarding the cranial opening for endoscopic surgery was done in 45 patients of <12 months age; 15 patients in case group for minicraniotomy and 30 infants as control group for burr hole. They were followed at least 1-month for complications including cerebrospinal fluid collection, wound dehiscence, wound infection, and meningitis.
Patients were between 1 and 11 months. Hydrocephalus (73%) was the most common etiology for endoscopic surgery in this series, followed by the arachnoid cyst (20%). Two patients in the case group and eight in control group developed complications. Meningitis was found in one infant in the control group. Despite less complications in the case group the difference between two cohorts was not statistically significant.
Minicraniotomy with providing more space in comparison to burr hole makes dural closure possible. It provides a small bone flap that can be replaced inside the bone defect. In spite of nonsignificant statistical difference between two groups regarding complication rate, we found less wound complications with minicraniotomy. Therefore, we advise this technique for the endoscopic procedure and propose minicraniotomy even in the older population to provide better dural opening, watertight dural closure and reconstructing the bone defect.
Journal of Pediatric Neurosciences 04/2015; 10(1):1-4. DOI:10.4103/1817-1745.154309
[Show abstract][Hide abstract] ABSTRACT: Parasitic fetus is a kind of conjoined twin, in which one of the twins is resorbed and some parts of the body can remain attached to the other twin. In the spinal form of parasitic twin-the so-called parasitic rachipagus-an ectopic or accessory limb is attached to the back of a normal baby or only a mass of viable tissue may remain.
Here, we report two cases of rachipagus that one had a lower extremity on her back and the other had some tissues similar to genitalia and formed bony structures on his back.
Child s Nervous System 02/2015; 31(6). DOI:10.1007/s00381-015-2664-z · 1.11 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Intradural sacrococcygeal teratoma (SCT) is a rare entity that has been reported in only a few cases previously. The authors present the case of a 2-week-old, otherwise healthy neonate with a mass in the buttock. The imaging findings and the high level of serum alpha-fetoprotein were highly suggestive of SCT. On operation the authors found intradural extension of the teratoma. The lesion was managed successfully without any remaining sequelae. The authors briefly review the currently proposed etiology regarding teratoma formation and the intradural extension of SCT.
[Show abstract][Hide abstract] ABSTRACT: Lipomyelomeningocele, a congenital spine defect, is presented as skin-covered lipomatous tissue that attaches to the cord in different ways according to its subtypes. Unlike other types of neural tube defects, the exact cause of this birth defect has not been confirmed yet, but it is proposed to be a multifactorial disease with involvement of both genetic and environmental factors. The authors describe identical twins with lipomyelomeningocele of the same subtype and location without any familial history of similar abnormality. Therefore, the same genetic and/or environmental risk factors could have played a part in their condition.
[Show abstract][Hide abstract] ABSTRACT: Re-synostosis after standard surgical procedures for nonsyndromic craniosynostosis is a rare event, which can occur at the same suture or rarely in adjacent sutures. Here, we report 2 patients with primary metopic craniosynostosis who developed bicoronal synostosis in previously opened sutures several months after surgery.
Journal of Pediatric Neurosciences 09/2014; 9(3):242-5. DOI:10.4103/1817-1745.147578
[Show abstract][Hide abstract] ABSTRACT: Background:
In general, it seems that both genetic and environmental factors play important roles in the induction of neural tube defects. Lipomyelomeningocele (LipoMMC) is a rather common type of closed neural tube defect, but only limited studies have investigated the potential risk factors of this anomaly. Therefore, the purpose of this case-control study was to investigate the risk factors involved in LipoMMC formation.
Material and method:
Various risk factors were evaluated in 35 children between 1 month and 10 years of age with LipoMMC in a hospital-based case-control study. The 2 control arms consisted of 35 children with myelomeningocele (MMC group) and 35 children with congenital anomalies other than central nervous system problems (control group). All groups were matched for age and visited the same hospital. A structured questionnaire was used for the collection of all data, including the mothers' weight and height during pregnancy, education, reproductive history, previous abortions, and socioeconomic status, as well as the parents' consanguinity and family history of the same anomalies.
Univariate analysis of the children with LipoMMC compared to the control group showed that the use of periconceptional folic acid supplementation was significantly lower in the MMC and LipoMMC groups compared to the control group. In addition, comparison of the MMC and control groups revealed statistically significant differences regarding the use of folic acid and maternal obesity. In multivariate analysis, use of folic acid in the periconceptional period and during the first trimester was an independent risk factor for LipoMMC and MMC. Furthermore, maternal obesity was a significantly positive risk factor for MMC.
The probable risk factors for LipoMMC were investigated in this case-control study. Consumption of folic acid in the periconceptional period and during the first trimester is an independent protective factor against LipoMMC. It seems that larger studies are needed to examine other possible risk factors.
[Show abstract][Hide abstract] ABSTRACT: Objective:
Exophytic gliomas of the medulla are rare childhood tumors that mostly are pilocytic astrocytomas. Here we report our experience in 11 -children with this rare tumor.
A retrospective study was performed using the records of children with exophytic gliomas of the medulla at Children's Hospital Medical Center in Tehran, Iran, from 2002 through 2013. The general, clinical, and radiological data and follow-up of all patients were reviewed.
The patients mostly were male aged from 11 months to 7 years. Swallowing problems, failure to thrive and nausea and vomiting were the most common symptoms. The time span between the onset of symptoms and the diagnosis was 2-24 months. Gross total resection of tumor was possible in 8 patients. Most tumors were pilocytic astrocytomas. Patients were followed for 2 months to 11 years (mean = 3.6 years). There was no intraoperative mortality. Recurrence occurred in 1 child with fibrillary astrocytoma.
Gross total resection of symptomatic dorsal exophytic medullary glioma is recommended. Most tumors are pilocytic astrocytomas. The attachment of these tumors to important brainstem structures usually inhibits total resection. Electrophysiological monitoring of sensorimotor pathways and cranial nerves can be helpful to preserve surrounding neural tissue during tumor resection and to minimize complications. Regular follow-up of patients with clinical examination and brain MRI is mandatory. Repeated surgery, radiation therapy and chemotherapy are suggested in cases with tumor recurrence or progression.
[Show abstract][Hide abstract] ABSTRACT: Lipomyelomeningocele, a closed neural tube defect, usually occurs in lumbosacral area as a single lesion but can be associated with other spinal dysraphism. We report an infant with a very rare presentation of tandem lumbosacral lipomyelomeningoceles, thick filum terminale and split cord malformation.
Journal of Pediatric Neurosciences 09/2013; 8(3):204-6. DOI:10.4103/1817-1745.123665
[Show abstract][Hide abstract] ABSTRACT: Myelomeningocele is a complex central nervous system malformation mostly associated with other neural and extraneural anomalies. A rare special feature of the forehead and skull was observed in myelomeningocele. Here, we present several patients with myelomeningocele, severe hydrocephalus, and interfrontal encephalocele accompanied by metopic suture widening and abnormally shaped frontal bones.
Five children with this feature were enrolled in this series. The age, sex, location of myelomeningocele sac, neurological deficits, hydrocephalus and history of shunt surgery, and follow-up period were evaluated.
Patients were aged from 1 to 12 months (mean, 6 months). All patients were male. Most sacs were located in lumbar and lumbosacral areas. Neurological deficits varying from only sphincter problem to paraplegia were found in all patients. Hydrocephalus was found in all children that needed a shunt procedure. Asymptomatic Chiari malformation, interfrontal encephalocele, and corpus callosum agenesis were evident in five, five, and three patients, respectively.
Anterior fontanel anomalies known as interfrontal encephalocele associated with myelomeningocele have been reported before. The patients have an open metopic suture extending widely to the nasal radix producing hypertelorism accompanied by interfrontal herniation of frontal lobes. The associated hydrocephalus exaggerates the anomaly. Treatment of accompanying hydrocephalus is advised to decrease the severity of frontal lobe herniation. Some patients may need frontal bone reconstruction surgery to provide cosmetic correction at the place of the midline frontal bone defect.
Child s Nervous System 08/2013; 29(8):1349-52. DOI:10.1007/s00381-013-2050-7 · 1.11 Impact Factor