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David McLone, David Frim, Richard Penn, Charles N. Swisher, Peter Heydemann, Kenneth M. Boyer, A. Gwendolyn Noble, Peter K. Rabiah, Shawn Withers, Kristen Wroblewski, Theodore Karrison, Samuel Hutson, Kelsey Wheeler, William Cohen, Joseph Lykins, and Rima McLeod

T oxoplasma gondii is a widespread protozoan parasite that causes ocular and neurological disease in persons infected congenitally or with compromised immune systems. When a woman acquires T. gondii infection for the first time while pregnant, the parasite may cross the placenta and infect the fetus. 3 , 5 In this circumstance, severe disease may occur because the fetal immune system is immature. The prevalence of hydrocephalus in congenital toxoplasmosis has been estimated to be between 3.8% and 30%, dependent on whether diagnosis included universal

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Lars de Jong, Michiel Verfaillie, Steven Pans, Philippe Lauweryns, Jan Goffin, and Bart Depreitere

subsequent myelopathy. Severe degeneration of the C1–2 joints may rarely produce a similar condition. In this paper, we report the medical history of two 70-year-old monozygotic twin sisters who both suffered from severe atlantoaxial instability and had identical radiographic features. Signs of inflammatory or metabolic disease, clear congenital disorders, or traumatic injuries could not be identified. Had either sister been a solitary case, degenerative atlantoaxial instability would have been the most straightforward diagnosis. In this case, however, the question

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Gurpreet S. Gandhoke, Ezequiel Goldschmidt, Robert Kellogg, and Stephanie Greene

( Fig. 1H ) views demonstrate good retraction of the encephalocele in the posterior fossa. Discussion No prior case of a prenatally diagnosed meningocele becoming an encephalocele by the time of delivery has been reported. Such a phenomenon may occur earlier in the gestation period than can be identified with current imaging modalities, but this case occurred late enough to be readily identifiable. Congenital encephaloceles classically contain gliotic neural tissue on pathological analysis; this tissue is routinely resected without neurological sequelae to close the

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Aneek Patel, Hussam Abou-Al-Shaar, Michael C. Chiang, Hanna N. Algattas, Michael M. McDowell, Jeremy G. Stone, Ellen B. Mitchell, Stephen P. Emery, and Stephanie Greene

H ydrocephalus is the most common reason for pediatric neurosurgical intervention in the United States, with an estimated incidence of 1 per 1000 newborns. 1 , 2 Various etiologies have been associated with neonatal hydrocephalus, including intraventricular hemorrhage (IVH), meningitis, spina bifida, mass lesions, and congenital aqueductal stenosis (CAS). Subgroup analyses of patients with a single etiology of hydrocephalus are lacking in the literature. Although similar in presentation and intervention, these etiologies vary in terms of age at diagnosis

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Kirpal S. Mann, Virender K. Khosla, and Des R. Gulati

T he spontaneous development of a communication between the ventricular system and the subarachnoid space proximal to the site of obstruction in obstructive hydrocephalus, leading to amelioration of symptoms, is a rare phenomenon. To date, only eight well documented cases have been reported. 1, 3, 8, 9, 11, 14–16 No previous case of congenital ventriculocisternostomy has been reported. We are presenting a case in which a communication between the floor of the third ventricle and the interpeduncular cistern was demonstrated by Conray ventriculography

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Pravin Salunke, Manish Sharma, Harsimrat Bir Singh Sodhi, Kanchan K. Mukherjee, and Niranjan K. Khandelwal

. 1 The genesis of congenital RAAD was thought to be different. 2 , 14 The inferior sagittal C-1 facet angles in RAAD patients are slightly acute in comparison with those in control individuals. Therefore, in the presence of lax ligaments, a congenital absence of the transverse ligament, or a short peglike odontoid or os odontoideum, there may be dislocation of the C1–2 facet joint. As the facet angle is relatively flat, it returns to the normal position on extension. Thus, it appears that RAAD and IrAAD may be part of the same spectrum. In healthy individuals

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Mehdi Zeinalizadeh, Seyed Mousa Sadrehosseini, Zohreh Habibi, Farideh Nejat, Harley Brito da Silva, and Harminder Singh

A congenital encephalocele is defined as a herniation of cranial contents through a cranial defect in a patient with a medical history negative for secondary causes. 17 Basal encephaloceles are rare, with an estimated incidence of 1 in every 35,000 live births. They are classified into transethmoidal, sphenoorbital, sphenomaxillary, and transsphenoidal. 19 Transsphenoidal encephaloceles are the rarest of the rare, accounting for 5% of basal herniations, and having an estimated incidence of 1 in 700,000 live births. 7 Transsphenoidal encephaloceles have

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Nicholas Boyko, Melissa Ann Eppinger, Deborah Straka-DeMarco, and Catherine Anne Mazzola

C ongenital torticollis presents with lateral neck flexion and neck rotation to the opposite side, as shown in Fig. 1 . The incidence of torticollis has been reported to be as high as 8.2%–16%, 9 , 15 but it is more commonly reported as between 0.3% and 3.92%. 4 , 5 , 7 , 8 , 14 , 16 The cause of congenital torticollis may be due to an abnormal position of the intrauterine fetus or fetal head descent causing trauma to the sternocleidomastoid muscle or its innervation. 7 , 9 Birth trauma may result in compartment syndrome, which is characterized by ischemia

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Brian J. McHugh, Ryan A. Grant, Alyssa B. Zupon, and Michael L. DiLuna

O s odontoideum is a well-described abnormality of the axis, consisting of an independent bony ossicle with smooth cortical margins separated from a hypoplastic dens. 21 This anomaly was initially described in 1886 8 and has generated significant debate regarding its etiology since its conception. Currently, there are 2 prevailing theories—congenital versus posttraumatic, 3 , 22 with the latter favored; however, some authors argue that elements of both theories represent the actual etiology. 17 To demonstrate this multifactorial etiology, we present a

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Jetan H. Badhiwala, Eric M. Thompson, Armando J. Lorenzo, and Abhaya V. Kulkarni

lack of understanding of the natural history of this condition. We report on two male infants with SLC who experienced spontaneous improvement of lower urinary tract dysfunction, providing further insights into the variable natural history of this congenital anomaly. Case Reports Case 1 History and Examination This male infant presented shortly after birth with a subcutaneous mass and two small overlying cutaneous hemangiomas in the lower lumbar region. The infant appeared to be urinating and defecating without any issues. Neurological examination