Cases reported "Spina Bifida Occulta"

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1/53. The use of Gore-Tex membrane for adhesion prevention in tethered spinal cord surgery: technical case reports.

    OBJECTIVE: The incidence of retethering caused by postoperative adhesions at the repair site after initial tethered spinal cord surgery is not uncommon. To assess the effectiveness of a Gore-Tex membrane in preventing these adhesions, only clinical radiological and experimental animal evaluation has been reported. In this report, we describe two cases in which Gore-Tex membrane was implanted at the initial untethering surgery and in which we were able to confirm the real effectiveness of the Gore-Tex membrane during a second operation. methods: In the first patient, Gore-Tex membrane was used for dural repair in the untethering surgery of the split spinal cord malformation. Because of the suspicion of a thickened filum terminale, repeated surgery was indicated 10 months after the initial procedure. In the second patient, Gore-Tex membrane was implanted during the initial untethering surgery for a lipomyeloschisis and a dermal sinus. Because of a persistent fistula of the dermal sinus, a second operation was necessary 1 year after the first operation. RESULTS: During the repeated surgery, a thorough inspection of the implanted Gore-Tex membrane revealed no adhesions between the Gore-Tex membrane and the intradural content in both cases. CONCLUSION: We support the use of Gore-Tex membrane in the prevention of postoperative dural adhesions in the repair of spinal dysraphism.
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2/53. Thoracic neurenteric cyst in an adult: case report.

    OBJECTIVE AND IMPORTANCE: Neurenteric cysts are very rare (particularly in the adult age group) congenital intraspinal cysts of endodermal origin. The patient described was a 48-year-old man who was diagnosed as having an isolated thoracic neurenteric cyst at the T5-T6 level, without a mediastinal enterogenous cyst. Radiological studies demonstrated multiple anterior vertebral column abnormalities. CLINICAL PRESENTATION: The patient presented with a long history of mild midback pain. During the 2 years preceding admission, his midback pain worsened and he experienced right intercostal pain in the midthoracic area. Several months before admission, he noticed right lower extremity weakness, which led him to undergo neurological evaluation and radiological studies. INTERVENTION: Thoracic spine x-rays revealed marked thoracic scoliosis and multiple vertebral abnormalities. magnetic resonance imaging and myelography revealed an intradural extramedullary cystic mass at the T5-T6 level, severely compressing the spinal cord from the ventral side. Thoracic laminectomy was performed and the cystic lesion was completely removed, with disconnection of the ventral tract. CONCLUSION: This is an unusual presentation, at this age, of a congenital intraspinal cyst in the thoracic area. Such cysts must investigated for early diagnosis and treatment. A neurenteric cyst should be suspected particularly if the cyst is associated with anterior vertebral column abnormalities.
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3/53. Temporal dermoid cyst with a partial dermal sinus tract.

    BACKGROUND: Cranial dermal sinuses are rare and occur most frequently in the posterior fossa and along the midline. Likewise, supratentorial dermoid cysts are very uncommon. METHOD: We report a unique case of an adult female patient with both a supratentorial dermoid cyst and an incomplete dermal sinus tract. RESULTS: The patient is a 31-year-old female, who presented with a new onset complex partial seizure. neuroimaging and surgery revealed a right superior temporal dermoid cyst with an associated dermal sinus tract. Furthermore, the dermal sinus tract was incomplete and had no cutaneous manifestations. CONCLUSION: We present a rare patient with an off midline supratentorial dermoid cyst associated with a uniquely incomplete cranial dermal sinus tract. The dermal sinus tract involved the bone, dura and intradural compartment, without involving the overlying skin. This represents a novel variant in the spectrum of cranial dermal sinus abnormalities.
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4/53. A congenital dermal sinus presenting the muscle fasciculation and hypertrophy.

    OBJECTIVE: To report unique and unknown clinical features of muscle fasciculation and muscle hypertrophy in a case of congenital dermal sinus. patients: A 16-year-old girl presented with continuous fasciculation, often cramp, and hypertrophy of the left calf muscle. The radiography showed spina bifida of L4, L5 and S1. MRI revealed dermal sinus tract from the skin dimple of the back to the dura mater, and connected to the intradural inclusion tumor. At surgery the inclusion tumor contained many short hairs, and the cauda equina were severely adherent. microdissection of the tumor and the adhesion was performed. At 2 years after surgery fasciculation decreased but continued; however, painful cramps of the calf muscle do not occur. CONCLUSIONS: Short hairs of dermoid and the adherence might be irritative to the cauda equina. The hyperactivity of the stimulated motor neuron may cause the muscle fasciculation leading to hypertrophy of the calf muscle.
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5/53. Cervical dermal sinus associated with dermoid cyst.

    We report a rare case of cervical dermal sinus associated with a dermoid cyst in a 10-month-old infant, who presented with a 1-month history of motor weakness of the right upper extremity. magnetic resonance imaging showed an intradural extramedullary dermoid cyst extending from C3 to C4, which was connected with the skin dimple along the sinus tract. Total excision of the dermoid cyst and the sinus tract with C3-5 laminectomy resulted in good functional recovery.
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6/53. Limited dorsal myeloschisis associated with multiple vertebral segmentation disorder.

    A 3-year-old girl was admitted to our department with spina bifida occulta. At birth, thoracic dysplasia with severe respiratory dysfunction and a soft pedunculated mass connecting with an intradural mass were noted. The patient did not start to walk and partial removal of the intradural mass was performed via a laminectomy of the fused vertebrae. There was no boundary between the spinal cord and the mass and the histological diagnosis of this mass was connective tissue. The anomalies in this case were considered to be multiple vertebral segmentation disorder (MVSD) and limited dorsal myeloschisis. The coincidence of these anomalies might suggest the causal genesis of MVSD.
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7/53. Cervical and thoracic dermal sinus tracts. A case series and review of the literature.

    Cervical and thoracic dermal sinus tracts (DSTs) account for 1 and 10%, respectively, of all DSTs. Few case reports describe this diagnosis. To characterize this entity, a 30-year retrospective audit was utilized to identify cases. Nine cases were identified, five of which were cervical and four thoracic. Four cases less than 1 year old presented with skin findings and no neurologic deficit. All five cases greater than 1 year old presented with neurologic findings. Initial examination revealed changes in motor function (n = 5), sensation (n = 4), reflexes (n = 5), gait (n = 4) and altered bowel/bladder function (n = 2). Eight patients had cutaneous findings and 7 had bifid spinous processes overlying tract entry into the dura. Operative findings included 6 buckled tethered cords, opacified arachnoid or frank arachnoiditis in 4 patients, 2 tract CSF leaks, 2 split cord malformations and 2 intradural tumors. Mean follow-up was 36 months. All infants remained neurologically intact. Four of the 5 patients greater than 1 year old demonstrated improvement; 1 continued with a stable deficit. This series of cervical and thoracic DSTs highlights the need for close attention to skin lesions in infants and consideration of retethering or tumor in patients with previous resections who deteriorate. Definitive operation including intradural exploration should be performed with the initial operation in an attempt to obviate future complications.
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8/53. Cerebellar abscesses secondary to occipital dermoid cyst with dermal sinus: case report.

    BACKGROUND: hydrocephalus and cerebellar abscesses as the principal manifestations of posterior fossa dermoid cyst are rare. In addition, extradural dermoid cyst of the posterior fossa has been described in only 9 cases in the literature. We present an unusual case of obstructive hydrocephalus due to cerebellar abscesses induced by an adjacent extradural dermoid cyst with complete occipital dermal sinus. CASE DESCRIPTION: A 14-month-old child presented with acute raised intracranially pressure, seizures, and meningitis. Neuroradiological studies revealed cerebellar cysts with ring enhancement associated with a contiguous occipital cyst, with compression of the adjacent cisterns and the fourth ventricle causing hydrocephalus. The diagnosis of cerebellar abscesses with congenital occipital defect was briefly entertained. The patient was treated by radical excision of the occipital cyst with hair contents, the dermal sinus, and the abscesses through a suboccipital approach, followed by systemic antibiotic therapy with a good outcome. Pathologic examination revealed a dermoid cyst. CONCLUSION: Posterior fossa dermoid cyst should be considered in all children with occipital skin lesions, especially dermal sinus. CT scan and MRI are the methods of choice for further investigation of suspect congenital dermal lesions. Neurosurgical treatment of these malformations should be planned early to prevent the high incidence of infections such as bacterial meningitis and cerebellar abscess. Clinical presentation, diagnostic evaluation, and treatment of these rare lesions are reviewed.
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9/53. Bilateral retro-auricular dermal sinus tracts with intradural extension. Case report.

    The authors describe a previously unreported malformation involving paired, bilaterally symmetrical dermal sinus tracts in the retro-auricular area, both of which passed through the asterion and posterior fossa dura mater to end intracranially. Cranial dermal sinus tracts are congenital lesions that virtually always originate from the midline scalp posteriorly at the external occipital protuberance, anteriorly at the nasion or along the nasal dorsum, or in the posterior parietal midline. A lateral origin is extremely rare, and intracranial extension of a lateral dermal sinus tract has not been reported previously. The authors propose an embryological mechanism to explain the origin of this rare malformation and discuss its management.
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10/53. Intracranial dermoid cysts with nasal dermal sinuses.

    Dermal sinuses penetrating the dura are important in that they may be complicated by C.N.S. infection, and this complication can be prevented by early surgery. Although well recognised over the occiput and lumbar spine, nasal dermal sinuses extending intracranially are much rarer and have received little attention in the neurosurgical literature. Two unique cases are presented, together with a literature review, discussing the anatomy, radiology, and management of the condition.
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