Search

Background: Growing skull fracture (GSF)/pseudomeningocele is a rare complication of head injury in children. Post-traumatic intradiploic arachnoid cyst (PTIAC) is a variant but it is extremely rare. PTIACs are usually asymptomatic or with mild symptoms like headache. The rupture of PTIAC due to re-trauma leading to pseudomeningocele formation has not been seen or reported before., Patient and Method: We present a case of occipital PTIAC where pseudomeningocele developed after re-trauma, and discuss the pathogenesis and management., Result: En masse removal of the ruptured PTIAC was performed along with dural-defect repair. The post-operative recovery was good., Conclusion: PTIAC is an extremely rare occurrence as a variant of GSF. It is usually asymptomatic, but it may rupture with trivial trauma due to a thinned-out outer table and then converts into pseudomengocele. En masse excision with dural-defect repair gives good results in cases of ruptured PTIAC due to re-trauma.

Objective: Terminal myelocystocele is a rare form of spinal dysraphism. We report on the clinical and radiological features, surgical procedures and outcome of myelocystocele., Methods: Thirty patients, with an age range of 1 month to 15 years, which included 16 (53.3%) male children, had undergone surgery for terminal myelocystocele between 2000 and 2010. We had retrospectively analyzed their data. All patients had a swelling in the lumbosacral region which had a healthy skin cover. Twenty (66.7%) patients had presented with weakness, while 10 (33.7%) patients had no deficits at all. Bladder bowel involvement was evident in 12 (40%) cases. All patients had undergone excision of the meningocele sacs, the tethering bands were lysed, and filum was detethered. Ventriculoperitoneal shunt was done in 3 (10%) patients. Complications like pseudomeningocele were seen in 6 (20%) patients, cerebrospinal fluid leak in 4 (13.3%) patients, and meningitis and surgical site infection in 1 (3.3%) patient each. The mean follow-up period was 15.2 (1-60) months. The status of all patients with no preoperative deficits remained unchanged. Out of 20 patients with motor weakness, 12 (60%) had improvement; worsening was observed in 1 patient., Conclusions: Though rare, the possibility of myelocystocele should be kept in mind when treating lumbosacral region masses as they have a better prognosis when compared with other masses in the region due to spinal dysraphism. We recommend early surgery in all diagnosed cases., (Copyright © 2013 S. Karger AG, Basel.)

Encephaloceles arise from developmental defects in neural tube formation. These lesions contain brain and meninges which herniate through a defect in the skull. These may present as isolated malformations or rarely be associated with brain tumors. We hereby discuss a case of an unusual association of an occipital encephalocele with papillary intralymphatic angioendothelioma or Dabska tumor arising from the sac itself. The patient underwent resection of the herniated brain tissue with repair and closure of the dural defect. Histopathological examination revealed evidence of Dabska tumor from the sac. This is the first case report of the association of an occipital encephalocele with a rare vascular tumor, i.e. papillary intralymphatic angioendothelioma., (Copyright © 2012 S. Karger AG, Basel.)

Objective: Cervical spinal dysraphism is a rare congenital spinal pathology. The results obtained from our series are compared with the results obtained from other series of studies in the literature., Methods: Seven patients with cervical myelomeningocele and meningocele who underwent surgery between January 1996 and March 2009 at the YYU Faculty of Medicine in the Department of Neurosurgery were retrospectively studied., Results: The referral ages of the patients (6 females and 1 male) varied between 4 days and 4 months (median 1 month). A stalk lesion covered with a dysplastic skin formed as a sac and located at the cervical midline was demonstrated in all of the patients. Cervical myelomeningocele was present in 4 patients, while cervical meningocele was present in 3 patients; however, Chiari type II malformation and hydrocephaly were present in 3 patients with myelomeningoceles. Diastematomyelia and a filum terminal lipoma were present in 1 of the patients., Conclusion: In this series, in contrast to the literature, we noted that the number of girls with spinal dysraphism with a cervical myelomeningocele and meningocele was greater than the number of boys. Chiari type II malformation, hydrocephaly and motor weakness in patients with cervical spinal dysraphism are less frequent when compared to patients with caudal spinal dysraphism. The structure of the sac is also more durable and, accordingly, a cerebrospinal fluid leakage is uncommon., (Copyright © 2011 S. Karger AG, Basel.)

Atretic meningoceles are infrequent congenital malformations resulting from the herniation of meninges through a skull defect. All prior reported cases have had a subcutaneous component with or without an underlying osseous defect. We report the unusual case of a completely intraosseous atretic meningocele., (2009 S. Karger AG, Basel)

We report a rare case of congenital type III cystic teratoma that may be falsely diagnosed as an anterior sacral meningocele. A female newborn baby presented with a long, white-colored 4 x 1 cm coccygeal cyst posterior to the anus. Magnetic resonance imaging (MRI) demonstrated that a 10 x 4 cm elliptical intrapelvic and -abdominal mass anterior to the sacrum and coccyx extended inferiorly to the retrorectal space. We intraoperatively found that the cyst consisted of a mostly thin, white wall, but also with small posterior narrow nodules. We suggest that all cysts in the sacrococcygeal area should be excluded from teratoma carefully, with MRI.

Associated cranial abnormalities with spinal dysraphism are not uncommon. We came across an unusual case of a 1-year-old male child with spinal dysraphism having lumbar meningomyelocele, who also had split cord malformation (hemicord with intervening bony spur) with lipoma of one of the hemicord and filum terminale. The patient also had communicating hydrocephalus without Chiari malformation and also near-total frontal bone agenesis. Single photon emission computed tomography scanning of brain revealed normal perfusion. In the first stage of repair, the patient had postoperative CSF leak for which ventriculo-peritoneal shunt was performed. This constitutes a rare anomaly associated with spinal dysraphism., (Copyright 2006 S. Karger AG, Basel)

We report a typical case of nasal chondromesenchymal hamartoma (NCMH) of infancy, which extended to the orbit and intracranium through the skull base. The nasal mass was incidentally found in a 5-month-old otherwise healthy boy who had recently developed ptosis of the left eye. On neuroimaging, there were defects in the left ethmoid bone of the anterior cranial fossa and medial orbital wall. A round heterogeneous mass was found in the left nasal cavity. Solid portions were strongly enhanced. The preoperative diagnosis was nasal glioma or encephalocele. After frontal craniotomy, the mass was separated from the cranial basal dura. Through the transnasal approach, the mass was dissected from the left orbital content and removed. NCMH is a rare benign lesion and histopathological analogue of the mesenchymal hamartoma of the chest wall, having unique pathology: islands of hyaline cartilage, somewhat calcified or ossified hyalinized collagenous trabeculae and abundant myxoid stroma. Its occurrence in the nasal cavity was identified in 1998. The recognition of this entity is necessary to avoid misdiagnosis of the frozen section and for appropriate surgery.

Spina bifida cystica is a closing disorder of the neural tube which infrequently occurs in the thoracic region. A rare lesion called myelocystocele is a variant of spina bifida cystica and is associated with syringomyelia, Chiari type 2 malformation and hydrocephalus. Usually the patient has no neurological deficit, but future deterioration can occur due to posterior tethering of the spinal cord by adhesions. The prenatal diagnosis by ultrasound study can be misleading and in order to attain the correct diagnosis, especially if abortion is considered, a prenatal MRI scan should be done before the parents are counselled, and should be repeated prior to operative treatment. Surgical correction of myelocystocele is not only for cosmetic reasons, but also to untether the spinal cord prophylactically to prevent future neurological deterioration. In this case report, we present a child born with a thoracic myelocystocele, the diagnostic difficulties, consequent implications and surgical treatment.

We describe a case of parietal cephalocele. MRI and MR venography were useful for accurate anatomical depiction. We speculate on developmental pathogenesis in the context of the literature available., (Copyright 2000 S. Karger AG, Basel)

Basal meningoencephalocele is frequently associated with midfacial anomaly, optic disc anomaly, brain anomaly, cerebrospinal fluid rhinorrhea, chiasma syndrome, and endocrinologic disturbance. The combination of basal meningoencephalocele and moyamoya disease is extremely rare. A 29-year-old man had basal meningoencephalocele (transsphenoidal type), anomaly of the optic disc (morning glory syndrome), panhypopituitarism and moyamoya disease. The patient was treated by hormone replacement, but surgical intervention was not required. Basal meningoencephalocele and moyamoya disease are a possible combination of the diseases., (Copyright 2000 S. Karger AG, Basel.)

A newborn presented with a skin-covered lumbar mass with a subcutaneous hemangioma and on a magnetic resonance image (MRI) revealed a tethered spinal cord with a local mass. The mass had signal characteristics compatible with a lipoma. An initial diagnosis of a lipomeningocele with tethered cord was made, and the patient underwent surgical exploration and subtotal resection of the mass. A follow-up MRI revealed that the cord was still tethered, but an additional mass was present. The initial mass with signal characteristics of lipomatous tissue was accompanied by a low-signal mass in the lumbosacral canal, ventral to the cord, and bilateral enlargement of the foramina at the lumbosacral level. Because of a concern for an intraspinal tumor, a second operative intervention was performed. Multiple biopsies of the mass inside the spinal cord, the nerve roots and at the level of the foramina revealed angiomas that had similar pathology in all the specimens. A partial resection of the masses and a release of the tethered cord was performed by sectioning the thickened filum terminale. The diagnosis of Cobb's syndrome was made. The unique association of a tethered cord and the Cobb syndrome is reported here.

In this article we report the cases of an 11-year-old girl who presented with an occipital atretic cephalocele and 2 of her siblings who had similar occipital lesions. Neuroimaging studies in these three instances showed a spectrum of posterior fossa cystic malformations. The girl's parents and a further sibling were also investigated by neuroimaging studies that proved to be normal. The familial occurrence of cephaloceles in general, and of atretic cephaloceles in particular, seems to be very rare in the absence of a known syndrome. Current literature on the familial presentation of cephaloceles and of Dandy-Walker complex is briefly reviewed. To the best of our knowledge this is the first description of the familial presentation of atretic cephaloceles not associated with a recognized syndrome.

Currarino's triad is a hereditary condition diagnosed when three abnormalities are noted: (1) an anorectal malformation; (2) an anterior sacral defect, and (3) a presacral mass. This condition often presents with symptoms related to the presacral mass or with an incidental finding of a sacral anomaly on plain radiographs. Presented here are 2 cases of Currarino's triad noted in siblings, both of whom underwent surgery for anorectal malformations in infancy. The importance of early diagnosis and treatment of this condition and the need for genetic counseling are emphasized.

A 2-month-old boy with an anterior basal meningoencephalocele of the transsphenoidal type was successfully treated via the transbasal intra- and extradural approach. Magnetic resonance imaging was useful for obtaining a precise diagnosis, and the transbasal combined approach is suitable for this type of meningoencephalocele.

In the literature, the combination of an abnormal cerebral venous system at conventional cerebral angiography with meningoencephalocele has been described. To our knowledge an abnormal venous system in patients with a meningoencephalocele disclosed by magnetic resonance angiography (MRA) has never been reported. A case is presented and the value of MRA, especially in the pediatric population, is stressed.

A male child with type I (no neck) Klippel-Feil syndrome presented at birth with a transient and partial cord injury. Investigations failed to reveal spinal instability or foramen magnum compression. Subsequent deterioration led to investigations that demonstrated neuroschisis. A cervical meningocoele manque with cord tethering was found at exploration and untethering reversed the deficit. The surgical pathology of the cervical cord is described.