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Decompressive aspiration in myelinoclastic diffuse sclerosis or Schilder disease

Case report

Farideh Nejat and Behzad Eftekhar

✓ This 9-year-old girl with rapidly progressive cerebral demyelinating disease presented with hemiplegia and intracranial hypertension. Brain images revealed four lesions with mass effect in the subcortical white matter of both hemispheres. Demyelination was found on pathological studies of these lesions. The patient experienced some recovery with corticosteroid treatment but improved completely with decompressive aspiration of the largest lesion.

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Spontaneous resorption of a sacral meningocele

Case illustration

Farideh Nejat and Syed Shuja Kazmi

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Editorial. Separation of craniopagus twins

James T. Goodrich

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Possible genetic correlation of an occipital dermal sinus in a mother and son

Case report

Saeed Ansari, Majid Dadmehr, and Farideh Nejat

✓ Occipital dermal sinuses (ODSs) are congenital lesions located in the midline and characterized by a cutaneous pit or dimple. The intracranial extension as well as the associated symptoms are variable. To date, a familial occurrence of these lesions has not been reported. In this paper the authors report on a 2-year-old boy with an ODS and intracranial hypertension. The boy’s mother had a similar lesion but did not have any complaints. Following their experience with this case and a literature review, the authors suggest that there may be a genetic basis in certain instances of ODS.

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Letter to the Editor: Trigonocephaly

Farideh Nejat and Mostafa El Khashab

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Double spinal dysraphism

Report of three cases

Sepideh Keshavarzi, Farideh Nejat, and Hadi Kazemi

✓The simultaneous presence of multiple spinal neural tube defects is unusual. There have been only a few of these cases reported in the literature. The authors report on three cases of double spina bifida cystica. One patient had two myelomeningoceles (MMCs) at the cervical and lumbosacral regions, one was noted to have both thoracolumbar and sacral defects, and the third presented with double MMCs at lumbar and lumbosacral levels. All three neonates in these cases underwent surgical treatment and ventriculoperitoneal (VP) shunt insertion for associated hydrocephalus. One child died at the age of 2 months despite a well-functioning VP shunt. The other two patients had no complications. Current models of neural tube closure do not thoroughly explain the mechanisms of multiple spinal dysraphism, but the multisite closure model provides a better understanding of caudal neural tube closure than other closure-site models.

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Teratoma within an encephalocele: common etiology or coincidence

Case report

Farid Radmanesh, Farideh Nejat, and Maryam Monajemzadeh

✓Encephalocele is a cystic congenital malformation in which central nervous system structures herniate through a defect in the cranium. The coincidence of encephalocele and neoplasm is a very rare condition. The authors present the case of a 5-month-old girl who underwent surgery for encephalocele and whose postoperative histopathological assessment revealed evidence of a mature teratoma inside the lesion. The embryogenesis of such a lesion is discussed.

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Spina bifida occulta

Thomas Pittman

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Letter to the Editor: Changes in the trend of hydrocephalus subtypes in infants

Zohreh Habibi and Farideh Nejat

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Teratoma inside a myelomeningocele

Zohreh Habibi, Farideh Nejat, Parisa Emami Naeini, and Fatemeh Mahjoub

Object

A myelomeningocele (MMC) can be associated with paraplegia, bladder and bowel incontinence, Chiari malformation Type II, and hydrocephalus. The coincidence of an MMC and a neoplasm is rare, and only limited reports on the concurrence of a teratoma within an MMC have been published.

Methods

A retrospective study was performed using the records of 330 children who underwent operations to correct an MMC at the Children's Hospital Medical Center in Tehran between January 2001 and June 2005. The postoperative histopathological assessments in 15 (4.5%) of these patients revealed evidence of a teratoma inside the MMC. The age of these patients at admission ranged from 2 days to 2 years (median 30 days). Neurological findings were normal in all patients except for three with lower-extremity weakness or paralysis. Hydrocephalus was detected in six patients. Physical appearance of the teratoma in all cases included cystic or solid soft-tissue masses in the dorsal midline area, covered with abnormal rudimentary skin. Pedunculated skin tags, a primitive genitalia-like appendage, dermal sinus, a human tail, and cutaneous stigmata such as color changes, hemangioma, dysplastic skin, and tufts of hair around the mass were occasionally observed.

Conclusions

The special feature of a protruding fingerlike appendage or intraoperative detection of a cystic portion of an MMC without direct connection to the spinal canal can be possible signs of teratoma concurrent with an MMC. Teratoma inside an MMC is a benign neoplasm, without any recurrence after standard surgery for an MMC.