Intradural capillary hemangioma of the cauda equina

Hemangiomas, which are usually found in the skin, are extremely rare in an intradural location. An unusual case of capillary hemangioma intimate to the cauda equina is discussed. This entity has not previously been reported in the pediatric or adolescent population to the best of our knowledge, although it has been reported in adults.     

Oncocytic paraganglioma of the cauda equina in a child. Case report and review of the literature

The authors report a case of oncocytic paraganglioma of the cauda equina in a 12-year-old girl who presented with lower back and leg pain on the right side of 6 months' duration. Magnetic resonance imaging revealed an ellipsoidal, intradural, extramedullary mass causing cord compression at the level of L1. Total laminectomy was performed on T12 and L1, and the tumor was excised completely without difficulty despite adherence of the tumor to the spinal cord. Postoperatively, the leg pain and motor weakness were much improved. The use of electron microscopy, and the immunohistochemical demonstration of synaptophysin in this tumor, allowed a confident diagnosis of an oncocytic paraganglioma to be made. To the authors' knowledge, this patient represents the first definite case of an oncocytic paraganglioma of the cauda equina in a child.     

Perinatal listeriosis. A report of a case with a hydrocephalus outcome]

Perinatal listeriosis is a disease whose incidence has increased constantly in recent years. Like neonatal infection determined by B hemolyticus streptococcus, the disease appears in two forms: the first is the septicaemic form with early onset, the second form is meningoencephalitic with late onset. We describe a case of listeriosis with immediate onset in which, in spite of the prompt therapy and the normality of rachiocentesis, developed a purulent ventriculitis with outcome in hydrocephalus.     

Endoscopic third ventriculostomy in prepontine-suprasellar tuberculoma with tuberculous meningitis hydrocephalus: a case report

We present a case of prepontine-suprasellar tuberculoma involving the premamillary region of the third ventricular floor, a site suitable to perform an endoscopic third ventriculostomy (ETV) stoma, managed endoscopically, and discuss our findings. A 5-year-old male child was admitted in an unconscious state with a history of gradually progressive symptoms of raised intracranial pressure and low-grade fever for the last 3 months. Head computed tomography showed thick enhancing basal exudates, a prepontine-suprasellar ring-enhancing lesion with consequent obstructive hydrocephalus. The child was subjected to urgent endoscopy which revealed multiple ependymal tubercles along with prepontine-suprasellar tuberculoma involving the premamillary region of the third ventricular floor. The tuberculoma was decompressed using a 5-french catheter, and ETV was performed. Postoperatively, the child improved clinicoradiologically on antitubercular chemotherapy and needed no further cerebrospinal fluid diversion surgery; he is under regular follow-up. We conclude that ETV may be attempted even in the presence of thick basal exudates and/or prepontine-suprasellar tuberculoma.     

Metastasis of testicular yolk sac tumor to cauda equina

Primary germ cell tumors may spread to the lung, liver, brain, and bone hematogenously. However, spinal metastasis is extremely rare. A case with yolk sac tumor (YST) and cauda equina metastasis was presented in a 2-year-old boy. Two months prior to admisssion, he underwent left radical orchiectomy for testicular YST. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed spinal and cauda equina involvement. This is the first report of metastasis of testicular YST to cauda equina in a child.     

以脑积水起病的甲基丙二酸尿症合并同型半胱氨酸血症1例报道

甲基丙二酸尿症合并同型半胱氨酸血症极少以脑积水起病。本文报道1例以脑积水起病的该病病例,并进行文献复习,探讨该类有机酸代谢病与脑积水的关系。该患儿以抽搐、脑积水起病,存在大细胞性贫血,发育评估落后,视听功能损伤,脑电图高峰失律,头颅磁共振及超声证实脑积水。血同型半胱氨酸升高,为143.06μmol/L,尿代谢筛查甲基丙二酸浓度为正常值的1483倍,基因检测确诊甲基丙二酸尿症合并同型半胱氨酸血症,为位于1p34.1的c.609G>A纯合突变,属于CblC型维生素B12合成酶缺陷。确诊后加用静脉维生素B12、口服叶酸、甜菜碱后,患儿抽搐缓解,脑室回缩,发育较前进步。因此,对于没有明确病因以脑积水作为主诉就诊的患儿,应注意排查代谢性疾病。     

A model of pulsations in communicating hydrocephalus

The traditional theory of communicating hydrocephalus has implicated the bulk flow component of CSF motion; that is, hydrocephalus is generally understood as an imbalance between CSF formation and absorption. The theory that the cause of communicating hydrocephalus is malabsorption of CSF at the arachnoid villi is not substantiated by experimental evidence or by physical reasoning. Flow-sensitive MRI has shown that nearly all CSF motion is pulsatile, and there is substantial evidence that hyperdynamic choroid plexus pulsations are necessary and sufficient for ventricular dilation in communicating hydrocephalus. We have developed a model of intracranial pulsations based on the analogy between the pulsatile motion of electrons in an electrical circuit and the pulsatile motion of blood and CSF in the cranium. Increased impedance to the flow of CSF pulsations in the subarachnoid space redistributes the flow of pulsations into the ventricular CSF and into the capillary and venous circulation. The salient features of communicating hydrocephalus, such as ventricular dilation, intracranial pressure waves, narrowing of the CSF-venous pressure gradient, diminished cerebral blood flow, elevated resistive index and malabsorption of CSF, emerge naturally from the model. We propose that communicating hydrocephalus is the result of a redistribution of CSF pulsations in the cranium.     

Intra-abdominal extra lobar pulmonary sequestration communicating with gastric duplication -- a case report.

Gastric duplications with associated pulmonary sequestration are very infrequent abnormalities. Although often asymptomatic, they should be considered in the differential diagnosis of intra-abdominal or retro-peritoneal masses. The authors report the case of a ten-year-old boy who had an occasional finding of poorly symptomatic intra-abdominal mass, recognised at intervention as a gastric duplication with associated extralobar pulmonary sequestration. The authors describe the surgical treatment of this case and briefly discuss the current views on the embryological pathogenesis of such malformations.     

Magnetic resonance imaging of the cauda equina in two patients with Guillain-Barré syndrome

The results are presented of magnetic resonance imaging (MRI) of the spine in two cases of childhood Guillain-Barré syndrome. After injection of gadolinium-diethylenetriamine pentaacetic acid, MRI showed enhancement of the cauda equina in these patients. These MRI observations may help confirm the diagnosis of Guillain-Barré syndrome. The nerve root enhancement resolved as the clinical symptoms improved. Serial imaging may be useful in monitoring the response to therapy and assessing new treatment regimens. It may also yield a better understanding of the disease process.     

Anaplastic clear cell ependymoma of the conus medullaris and cauda equina in a 3-year-old child

Only a minority of ependymomas present in the spinal cord. We report on a child with recurrence of a rare anaplastic clear cell type of ependymoma. This was initially misdiagnosed as a World Health Organization grade I myxopapillary ependymoma because this type of ependymoma occurs more commonly and the magnetic resonance imaging features and location at the conus medullaris were considered characteristic.     

Neurocutaneous melanosis with hydrocephalus, intraspinal arachnoid collections and syringomyelia: case report and literature review

Neurocutaneous melanosis (NCM) is a rare nonfamilial syndrome, characterised by large or numerous congenital pigmented nevi and excessive proliferation of melanin-containing cells in the leptomeninges. We report the MR findings in the brain and spine of a child with NCM who underwent neurosurgical treatment and was followed up for 8 years. The findings in this child (small hyperintense collections of melanocytes in both temporal lobes, mild meningeal enhancement along the spine and the development of an extensive subarachnoid CSF accumulation with cord compression and syringomyelia) are believed to be exceptionally rare. Received: 26 January 1999/Accepted: 10 October 1999     

Fucosidosis with hypothyroidism: a case report

Fucosidosis is a rare, autosomal recessive lysosomal storage disorder caused by a severe deficiency of alpha-L-fucosidase. Here we present a 27-month-old male who was referred to us for evaluation of developmental delay, which was first detected at age six months. His past medical history was also remarkable for recurrent pulmonary infections and myoclonic seiures. His family history revealed that he was the first living child from a consanguineous marriage. He had a younger sister who died at five months of age from pneumonia who had facial resemblance to the proband, developmental delay and a congenital heart defect. Physical examination revealed length: 81 cm (25-50p), weight: 10.2 kg (25-50p), and head circumference: 49 cm (50-75p). He had a coarse face, hepatomegaly and generalized spasticity. His initial laboratory examination revealed negative urine screening column chromatography for mucopolysaccharidosis. His X-ray findings were consistent with mild form of dysostosis multiplex. Based on clinical and laboratory features, fucosidosis was suspected. Fucosidase enzyme activity was zero. In addition to fucosidosis, thyroid function tests indicated primary hypothyroidism. This is, to the best of our knowledge, the fourth case of fucosidosis diagnosed in Turkey.