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Isolated cervical spinal canal stenosis at C-1 in the pediatric population and in Williams syndrome

Clinical article

Sohum K. Desai, Sudhakar Vadivelu, Akash J. Patel, Alison Brayton, and Andrew Jea

congenital abnormality. Normal sagittal diameter measurements in boys < 3 years have been reported as 15.2–23.8 mm; as 15.7–24.3 mm in 8-year-old boys; 16.4–25 mm in 13-year-old boys; and 17–25.6 mm in 18-year-old boys. 6 Among girls, sagittal diameter was reported as 12.9–20.7 mm in 3-year-olds; 13.9–21.7 mm in 8-year-olds; 14.9–22.7 mm in 13-year-olds, and 15.8–23.6 mm in 18-year-olds. 5 , 6 When the spinal canal is < 14 mm, spinal cord compression may be suspected. 4 A sagittal diameter of < 10 mm in the spinal canal at the level of the atlas indicates a high risk of

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Cauda equina syndrome secondary to an absent inferior vena cava managed with surgical decompression

Case report

Mayshan Ghiassi, Mahan Ghiassi, Elyne Kahn, Luke Tomycz, Michael Ayad, and Oran Aaronson

The authors report on the case of a 24-year-old man who presented with back pain and radiculopathy due to epidural venous engorgement in the setting of a congenitally absent inferior vena cava. Despite initial improvement after steroid administration, the patient's health ultimately declined over a period of weeks, and signs and symptoms of cauda equina syndrome manifested. Lumbar decompression was performed and involved coagulation and resection of the compressive epidural veins. No complications occurred, and the patient made a full neurological recovery.

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Effect of molding helmet on head shape in nonsurgically treated sagittal craniosynostosis

Clinical article

Sandeep Sood, Arlene Rozzelle, Blerina Shaqiri, Natasha Sood, and Steven D. Ham


Sagittal craniosynostosis is traditionally considered to be a surgical condition. Poor results of simple suturectomy follow from early reclosure of the suture. A wider craniectomy or use of interposing materials has not improved the outcome. However, endoscopic suturectomy supplemented with postoperative use of a molding helmet has shown good results. Because suturectomy reunites within 8–12 weeks of surgery, the authors questioned if the improved outcome was primarily related to use of the helmet.


In 4 patients whose families opted for calvarial reconstruction when the infant was 4–6 months old, instead of endoscopic suturectomy, a molding helmet was used to minimize compensatory changes in the interim. Patients underwent 3D CT scanning to confirm craniosynostosis. Follow-up visits were made at intervals of 4 weeks for adjustment of the helmet, head circumference measurements, clinical photographs, and cranial index measurement.


There was significant improvement in the head shape within 6 weeks of use of the molding helmet. The cranial index score improved from a mean (± SD) of 67% ± 3% to 75% ± 2%.


These cases demonstrate that molding helmets improve head shape even without a suturectomy in patients with sagittal craniosynostosis, challenging the traditional view.

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Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele

Stephanie L. Da Silva, Yasser Jeelani, Ha Dang, Mark D. Krieger, and J. Gordon McComb


There is a known association of hydrocephalus with encephaloceles. Risk factors for hydrocephalus and neurological deficit were ascertained in a series of patients born with an encephalocele.


A retrospective analysis was undertaken of patients treated for encephaloceles at Children's Hospital Los Angeles between 1994 and 2012. The following factors were evaluated for their prognostic value: age at presentation, sex, location of encephalocele, size, contents, microcephaly, presence of hydrocephalus, CSF leak, associated cranial anomalies, and neurological outcome.


Seventy children were identified, including 38 girls and 32 boys. The median age at presentation was 2 months. The mean follow-up duration was 3.7 years. Encephalocele location was classified as anterior (n = 14) or posterior (n = 56) to the coronal suture. The average maximum encephalocele diameter was 4 cm (range 0.5–23 cm). Forty-seven encephaloceles contained neural tissue. Eight infants presented at birth with CSF leaking from the encephalocele, with 1 being infected. Six patients presented with hydrocephalus, while 11 developed progressive hydrocephalus postoperatively. On univariate analysis, the presence of neural tissue, cranial anomalies, encephalocele size of at least 2 cm, seizure disorder, and microcephaly were each positively associated with hydrocephalus. On multivariate logistic regression modeling, the single prognostic factor for hydrocephalus of borderline statistical significance was the presence of neural tissue (odds ratio [OR] = 5.8, 95% confidence interval [CI] = 0.8–74.0). Fourteen patients had severe developmental delay, 28 had mild/moderate delay, and 28 were neurologically normal. On univariate analysis, the presence of cranial anomalies, larger size of encephalocele, hydrocephalus, and microcephaly were positively associated with neurological deficit. In the multivariable model, the only statistically significant prognostic factor for neurological deficit was presence of hydrocephalus (OR 17.2, 95% CI 1.7–infinity).


In multivariate models, the presence of neural tissue was borderline significantly associated with hydrocephalus and the presence of hydrocephalus was significantly associated with neurological deficit. The location of the encephalocele did not have a statistically significant association with incidence of hydrocephalus or neurological deficit. In contrast to modestly good/fair neurological outcome in children with an encephalocele without hydrocephalus, the presence of hydrocephalus resulted in a far worse neurological outcome.

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Intraspinal lipomas with spina bifida

Prognosis and treatment in 73 cases

Alain Pierre-Kahn, Jacques Lacombe, J. Pichon, Yves Giudicelli, Dominique Renier, Christian Sainte-Rose, M. Perrigot, and Jean-François Hirsch

✓ The authors present a series of 73 cases of intraspinal lipomas in the lumbosacral region. Sixty-four patients were operated on, 43 of these under intraoperative monitoring of neural function. The results of this series and of major series published in the past 10 years demonstrate both the potential severity of these lesions (which are responsible for progressive neurological deficits in 56% of affected cases) and the benignity of their surgical treatment. The authors emphasize the usefulness of systematic early surgical treatment of these lesions.

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Congenital intraorbital optic nerve cyst

Case report

Kyoung Mee Kim, Seok Jin Kang, Dal Soo Kim, Je G. Chi, and Sun Moo Kim

✓ Congenital cystic lesions of the optic nerve are exceedingly rare; only one case is reported in the world literature. The authors describe a case of congenital simple glial cyst in the intraorbital portion of the optic nerve with a brief review of the literature and comment on its histogenesis.

A 45-day-old male infant was admitted to the hospital because of progressive proptosis and hypotropia in the left eye, which had been present since birth. Magnetic resonance imaging of the left orbit revealed an ovoid, well-demarcated, homogeneous cystic mass in the intraconal retrobulbar area. The mass compressed the left eyeball with downward and lateral displacement. The wall of the cystic mass was very thin, and a needle puncture of the cyst released clear, colorless, watery fluid. The cystic wall was lined by loose astroglial nerve fibers with some scattered glial cells.

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Iniencephaly: neuroradiological and surgical features

Case report and review of the literature

Pamir Erdinçler, Mehmet Y. Kaynar, Bülent Canbaz, Naci Koçer, Cengiz Kuday, and Nejat Çiplak

Iniencephaly is a rare congenital anomaly characterized by spina bifida of the cervical vertebrae, fixed retroflexion of the head on the cervical spine, and occipital bone defect. There are only five reports of surviving patients with iniencephaly. The authors report the case of a newborn who presented with iniencephaly and an encephalocele that were surgically treated in our service. Neurological examination of the patient yielded normal results except for a moderate psychomotor retardation. The neuroradiological and surgical findings of the case suggested that the trigger of the anomaly was the occipital bone defect and rachischisis of the posterior vertebral arches.

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Myelocystocele with cerebellar heterotopia

Case report

Anders Suneson and Hannu Kalimo

✓ The authors describe a case with well differentiated cerebellar tissue contained in a hydromyelic dilation of the spinal cord, that is, myelocystocele, in connection with a cervicothoracic meningocele. The embryology is discussed.

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Intracranial extracerebral brain heterotopia

Case report

Tohru Marubayashi and Yasuhiko Matsukado

✓ The authors describe a case of intracranial heterotopic brain located on the medial one-third of the left sphenoid ridge, and enveloped in a huge cystic cavity. The heterotopic brain was resected successfully with satisfactory clinical results. The resected brain was 8 × 4 × 3 cm in diameter, of 7 or 8 months gestational age, and consisted of cerebrum, brain stem, and cerebellum. Anatomical and cytoarchitectural findings of the heterotopic brain are presented.

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Surgical removal of a symptomatic paracondylar process

Case report

Mukesch J. Shah, Jan Kaminsky, and Vassilios I. Vougioukas

C ongenital anomalies of the craniovertebral region constitute a group of heterogenic osseous abnormalities, mostly of embryological origin. 2 , 4 , 7 The paracondylar process is a rare congenital abnormality of the craniocervical junction with a prevalence of 0.077–0.64% that has been identified as a causative agent for severe headache, neck pain, and restricted head movement. 1–3 Conservative treatment is usually sufficient. 1 We report the case of a symptomatic paracondylar process in a young patient who required surgical intervention. Case