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1/49. Intraspinal epidermoid cyst occurring 15 years after lipomyelomeningocele repair. Case report.

    The authors report the case of a spinal epidermoid cyst that developed in a patient who had undergone surgery for lipomyelomeningocele repair 15 years earlier. The patient presented with symptoms of retethering. magnetic resonance imaging revealed a cystic intraspinal mass that extended from L-2 to L-5. The mass proved to be an epidermoid cyst. Spinal epidermoid cysts can cause retethering after a repair of lipomyelomeningocele, and the risk of this development can be present for decades.
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keywords = myelomeningocele
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2/49. A case of cerebrospinal fluid eosinophilia associated with shunt malfunction.

    A 3-month-old female patient presented with a meningomyelocele at the lumber region associated with congenital hydrocephalus. She underwent ventriculoperitoneal (V-P) shunt surgery using the Sophy system. The shunt system was replaced due to a malformation. Following replacement, the patient presented with cerebrospinal fluid (CSF) eosinophilia at the age of 8 months. The eosinophilic granulocytosis of the CSF improved dramatically following systemic prednisolone administration. CSF eosinophilia without accompanying inflammation or pyrexia in the present case may have resulted from an allergic response to a foreign material such as the silicone tube pressure valve of the Sophy system or the sutures rather than bacterial or fungal infection. Based on our results, we believe that some patients may experience CSF eosinophilia following postoperative V-P shunt due to an allergic reaction to the shunt equipment. Prompt steroid treatment can produce spontaneous regression in such cases.
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ranking = 0.06078427968072
keywords = meningomyelocele
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3/49. Migration of the abdominal catheter of a ventriculoperitoneal shunt into the scrotum--case report.

    A 3-day-old male neonate presented with migration of the ventriculoperitoneal (VP) shunt tip through the patent processus vaginalis resulting in scrotal hydrocele. The association of myelomeningocele with hydrocephalus may have been a predisposing factor in this rare complication. Development of scrotal swelling or hydrocele in a child with VP shunt should be recognized as a possible shunt complication.
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ranking = 0.16666666666667
keywords = myelomeningocele
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4/49. Symptomatic calcified subdural hematomas.

    Two unique cases of chronic calcified subdural hematomas are reported in children as a long-term complication of a ventriculoperitoneal shunt. Both the patients had undergone shunt procedures in infancy for congenital hydrocephalus. In one patient, the cause of the hydrocephalus was aqueduct stenosis, while in the second patient, a lumbar meningomyelocele was associated with hydrocephalus. In both these patients, a ventriculoperitoneal shunt was done in infancy. In one of them, following the shunt surgery, a bilateral subdural collection was noticed which required burr hole evacuation. Both the patients remained asymptomatic for 9 years, when they presented to our center with acute raised intracranial pressure and contralateral hemiparesis. Both the patients had a relatively short history and had altered sensorium at admission. Surprisingly, in both the patients, the CT scan showed significant mass effect producing calcified subdural hematomas. The shunt systems were found to be working well at surgery. craniotomy and excision of the calcified subdural hematomas was undertaken. Postoperatively, the patients showed satisfactory recovery, and at discharge the patients were doing well. At the follow-up at the outpatient clinic, the patients were asymptomatic.
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ranking = 0.06078427968072
keywords = meningomyelocele
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5/49. Membranous occlusion of the foramen of Monro following ventriculoperitoneal shunt insertion: a role for endoscopic foraminoplasty.

    We report two cases of lateral ventricle dilatation due to membranous occlusion of the foramen of Monro following ventriculoperitoneal shunt insertion. Both cases were treated successfully by endoscopic foraminoplasty of the obstructed foramen of Monro and III ventriculostomy. One child had meningomyelocele and hydrocephalus. She had CSF infection after repair of the back lesion. Isolated left lateral ventricle occurred after insertion of a right ventriculoperitoneal shunt for hydrocephalus when the girl was 2 months old. A right ventriculoperitoneal shunt was then inserted. Chronic shunt infection with abdominal pseudocyst was found 8 years later. The shunts were exteriorized. Membranous obstruction of the foramen of Monro was found endoscopically. Fenestration of the membranous obstruction along with a III ventriculostomy was performed. After the endoscopic procedure, the exteriorized ventriculoperitoneal shunt was removed 2 weeks later. The patient was still symptom free without shunting 14 months after the operation. The other child had hydrocephalus after a premature birth and hemorrhage. Repeated ventriculoperitoneal shunt infections contributed to membranous obstruction of bilateral foramen of Monro. After the shunt infection was treated this patient's shunting procedure was simplified by endoscopic foraminoplasty of the left and right foramen of Monro along with a III ventriculostomy. He was symptom free with a new ventriculoperitoneal shunt 9 months after the operation.
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ranking = 0.06078427968072
keywords = meningomyelocele
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6/49. Prenatal sonographic diagnosis of uterine rupture following open fetal surgery.

    BACKGROUND: Reported cases of uterine rupture diagnosed by ultrasound have shown fetal membranes ballooning through uterine rupture sites, or adjacent areas of hemorrhage. CASE: A 27-year-old gravida 3, para 2 had open fetal surgery to repair a fetal myelomeningocele at 28 weeks' gestation. Her postoperative course was complicated by threatened preterm labor and anhydramnios. At 33 weeks' gestation, with maternal symptoms of bowel obstruction, ultrasound showed a fetal leg and section of umbilical cord protruding through the uterine wall. CONCLUSION: Even in the presence of anhydramnios, uterine wall rupture was identified, because ultrasound evaluation of the uterine wall showed prolapsed fetal parts and umbilical cord. Persistent anhydramnios after open fetal surgery should prompt a search for uterine rupture.
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ranking = 0.16666666666667
keywords = myelomeningocele
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7/49. Apophyseolysis of the fourth lumbar vertebra: an early postoperative complication following kyphectomy in myelomeningocele.

    This is a report of a young girl with congenital kyphosis at the thoracolumbar spine in association with myelomeningocele. Kyphectomy and posterior stabilisation extending from the eighth thoracic to the fourth lumbar vertebra was done. Apophyseolysis occurred as an early postoperative complication at the level of the L4-L5 disc. This failure mode was treated by extending the fusion to the pelvis.
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ranking = 0.83333333333333
keywords = myelomeningocele
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8/49. Clinical features in patients requiring reoperation after failed endoscopic procedures for hydrocephalus.

    The aim of this study was to clarify the clinical features of patients at risk of secondary obstruction following endoscopic fenestration. Clinical notes and endoscopic findings for 15 patients treated with endoscopic procedures were retrospectively reviewed. Endoscopic third ventriculostomy (ETV) was performed as initial treatment in 4 patients with non-communicating hydrocephalus, including a neonate with myelomeningocele, and as an alternative to shunt revision in 4 patients. Two patients with non-communicating hydrocephalus caused by tumor or arachnoid cyst were also managed with third ventriculostomy. Four patients with loculated hydrocephalus underwent endoscopic septostomy. A child with an isolated fourth ventricle was treated with endoscopic aqueductoplasty. Of the 15 patients undergoing endoscopic procedure, 4 required reoperation. Of the 10 patients treated with ETV, only the neonate with myelomeningocele required a ventriculoperitoneal shunt because of failure of the initial procedure. Of the 4 patients treated with endoscopic septostomy, 2 children with loculated hydrocephalus following intraventricular hemorrhage (IVH) underwent a second septostomy. In a patient with an isolated fourth ventricle following posthemorrhagic hydrocephalus, recurrence was noted 8 months after the initial procedure. He underwent a second procedure using a stent implanted into the aqueduct to maintain CSF circulation. Sufficient stomal size or implantation of a stent may be required in the under-2-year age group with hydrocephalus accompanied by IVH and associated with myelomeningocele, in whom the risk of secondary obstruction may be high.
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ranking = 0.5
keywords = myelomeningocele
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9/49. Chiari I malformation: clinical presentation and management.

    Chiari malformations are cerebellar anomalies. The four types of Chiari malformations, as described more than 100 years ago by Dr. Hans Chiari, have neither anatomic nor embryologic correlation. Their only commonality is that they all involve the cerebellum. Chiari I malformation consists of herniation of the cerebellar tonsils into the foramen magnum, thus crowding the craniocervical junction. Chiari II malformation is almost exclusively associated with myelomeningocele and hydrocephalus. It consists of herniation of not only the tonsils but also all the contents of the posterior fossa into the foramen magnum. This herniation involves the brainstem, fourth ventricle, and cerebellar vermis. Chiari III and IV malformations are rare. Chiari III represents an encephalocele (external sac containing brainstem and posterior fossa contents); thus, the cerebellum and brainstem are descending not only into the spine, but also into an external sac. Chiari IV consists of cerebellar hypoplasia. The Chiari I malformation has the latest mean age of clinical presentation. A Chiari type I anomaly presenting in adulthood is the focus of this article. Surgery is indicated with neurological dysfunction, symptomatic syrinx, or hydrocephalus. Of all Chiari I patients, 15%-20% will have hydrocephalus. For some of them, the hydrocephalus will resolve with ventriculoperitoneal shunting, alleviating the need for a Chiari decompression. Long-term prognosis for patients with symptomatic Chiari type I malformations who undergo surgical treatment is variable, based on the patients' presenting symptoms and spinal cord cyst response.
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ranking = 0.16666666666667
keywords = myelomeningocele
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10/49. Lethal pulmonary hypoplasia after in-utero myelomeningocele repair.

    BACKGROUND: In-utero surgical repair of fetal myelomeningocele has been performed as a means to improve the postnatal condition of affected infants. CASE: A nulliparous woman underwent in-utero surgical repair of a fetal lumbosacral myelomeningocele at 24 weeks' gestation. Her postoperative convalescence was complicated by pulmonary edema, abdominal pain, chronic oligohydramnios, and preterm labor. The infant was delivered by cesarean at 33 weeks' gestation, but expired from respiratory distress caused by pulmonary hypoplasia at 9 hours of age. CONCLUSION: Until the benefits of in-utero repair of fetal myelomeningoceles are determined by well-controlled clinical trials, this technique remains investigational. physicians and their patients who are considering this procedure must be fully aware of the potential risks that can occur.
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ranking = 1.1666666666667
keywords = myelomeningocele
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