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Clinical predictors of developmental outcome in patients with cephaloceles

Clinical article

Benjamin W. Y. Lo, Abhaya V. Kulkarni, James T. Rutka, Andrew Jea, James M. Drake, Maria Lamberti-Pasculli, Peter B. Dirks, and Lehana Thabane

neural and surface ectoderm after neurulation, paraaxial mesodermal insufficiency is thought to affect formation of the occipital bone, neurocranium, and dura mater. 8 In a third theory, formation of cephaloceles is thought to be influenced by genes controlling neural tube development, including the ventralizing sonic hedgehog pathway and the dorsalizing bone morphogenetic protein . 6 The purpose of our study was to investigate the potential risk factors for the occurrence of developmental delay in patients with cephaloceles, by using both univariate analysis and

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Endovascular treatment of vein of Galen aneurysmal malformation: management strategy and 21-year experience in Toronto

Clinical article

Ai-Hsien Li, Derek Armstrong, and Karel G. terBrugge

enlarging head circumstance in infants, or developmental delay that mostly manifests in childhood but may also occur at any age. 4 , 14 , 24 For the past 20 years, we have been using a consistent strategy in the management of VGAM that was developed in accordance with guidelines proposed by the team at the Hôpital de Bicêtre in France. 14 In neonates presenting with CHF, aggressive medical therapy is given under the supervision of an experienced team of pediatric cardiologists and interventionists. If the medical stabilizing attempt fails to control the worsening CHF, a

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Fetal ventriculomegaly: prognosis in cases in which prenatal neurosurgical consultation was sought

Chang Sub Lee, Seok Ho Hong, Kyu-Chang Wang, Seung-Ki Kim, Joong Shin Park, Jong-Kwan Jun, Bo Hyun Yoon, Young-Ho Lee, Son Moon Shin, Yeon Kyung Lee, and Byung-Kyu Cho

, such as the presence of accompanying anomalies, are analyzed to determine whether there are correlations with developmental delay and/or outcome. Clinical Material and Methods The medical records of 44 cases of FVM managed at the Division of Pediatric Neurosurgery of Seoul National University Children’s Hospital between 1995 and 2002 were retrospectively reviewed. In all cases, FVM was diagnosed in utero using prenatal ultrasonography (HDI 5000 unit, Advanced Technology Laboratories, Bothell, WA; Aloka SSD 650/SSD 680, Aloka, Japan; Sonoace 8000 SE, Medison

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Behavioral, developmental, and educational problems in children with nonsyndromic trigonocephaly

Michael O. Kelleher, Dylan J. Murray, Anne McGillivary, Mahmoud H. Kamel, David Allcutt, and Michael J. Earley

correction. The median age at surgery was 12 months (range 4–144 months). Speech and/or language delay was reported in 34% of the children. Thirty-three percent of the parents reported a developmental delay in their child. Thirty-seven percent of parents expressed concerns about their child’s behavior, reporting attention deficit disorder, autism, and hyperactivity. Of the 63 children, 42 were attending school at the time of this study, and thus only this number was available for assessment of school progress. The majority (90%) attended mainstream schools; the

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Filamin A–negative hyaline astrocytic inclusions in pediatric patients with intractable epilepsy: report of 2 cases

Grant M. Fischer, Elmira Vaziri Fard, Manish N. Shah, Rajan P. Patel, Gretchen Von Allmen, Leomar Y. Ballester, and Meenakshi B. Bhattacharjee

intractable epilepsy but otherwise preserved neurological status. 6 To date, 27 cases of these inclusions have been reported in children and young adults ranging from those with intractable epilepsy and mild developmental delays to those with Aicardi syndrome ( Table 1 ). 3–14 Some patients have concomitant brain anomalies related to migrational problems (e.g., periventricular heterotopia, polymicrogyria, and pachygyria). Interestingly, these inclusions have also been observed in a patient with arteriopathic dementia. 15 TABLE 1. Literature review of cases reporting

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Subdural hematoma prevalence and long-term developmental outcomes in patients with benign expansion of the subarachnoid spaces

Katherine G. Holste, Clare M. Wieland, Mohannad Ibrahim, Hemant A. Parmar, Sara Saleh, Hugh J. L. Garton, and Cormac O. Maher

. Patients may or may not have mild ventriculomegaly as well. BESS is frequently associated with mild developmental delay, particularly gross motor and speech/language delay. 3–5 , 8 , 9 In a prior retrospective study of 99 patients with BESS, the majority of motor delays resolved within the 13-month follow-up period. 9 While these delays were thought to resolve in the first few years of life, recent long-term studies have challenged this assumption. One study documented subtle motor deficits on long-term follow-up, while other studies have documented subtle

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Choroid plexus papilloma and Pierpont syndrome

Case report

Sudhakar Vadivelu, Morris Edelman, Steven J. Schneider, and Mark A. Mittler

I n 1998, Mary Ella Pierpont first described 2 unrelated boys (9 and 2.5 years of age) with remarkably similar facial dysmorphism, plantar fat pads on hands and feet, and global developmental delay. 5 The facial features were unique in that both patients displayed midface hypoplasia, anteverted nostrils, a central palatal ridge, and an elevated forehead together with mild microcephaly. Plantar fat pads were described as deep grooves generating the appearance of “pillowing” pads between the grooves. Severe speech delay was demonstrated in both patients

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A novel syndrome of cerebral cavernous malformation and Greig cephalopolysyndactyly

Laboratory investigation

Kaya Bilguvar, Mohamad Bydon, Fatih Bayrakli, A. Gulhan Ercan-Sencicek, Yasar Bayri, Christopher Mason, Michael L. DiLuna, Margretta Seashore, Richard Bronen, Richard P. Lifton, Matthew State, and Murat Gunel

performed following amplification. We used the standard curves with an efficiency between 90 and 100%, which corresponds to a slope between −3.58 and −3.20. Illustrative Case History and Examination This 32-month-old girl presented to Yale University with a history of developmental delay. The mother's pregnancy and delivery were uncomplicated, and the child was born vaginally at a gestational age of 40 weeks (APGAR scores: 8 at 1 minute and 9 at 5 minutes). The family had no history of mental retardation, infant deaths, or multiple spontaneous abortions. The

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Adenosine-induced cardiac standstill for endovascular treatment of pediatric vein of Galen malformations

Nam K. Yoon, Jonathan P. Scoville, and Philipp Taussky

head circumference and at times developmental delay. 6 , 8 Medical management can only temporize symptomatology but can be useful in neonates who are not yet stable enough or lack a mature enough vascular system to undergo open surgical or endovascular intervention. Historically, these malformations were almost universally fatal, and up to 100% mortality with either medical or surgical management has occurred in reported case series. 9 With advances in endovascular treatments, such as coils, glue, and Onyx (Medtronic) liquid embolic agent, this rare disease has

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Bilateral squamosal synostosis: unusual presentation of chromosome 1p12–1p13.3 deletion. Illustrative case

Sarut Chaisrisawadisuk, Nithiwat Vatanavicharn, Verayuth Praphanphoj, Peter J. Anderson, and Mark H. Moore

. Echocardiography showed mild coarctation of the aorta and a hypoplastic aortic arch. She had global developmental delay and failure to thrive, with her height and weight under the third percentile. Three-dimensional computed tomography (3D-CT) showed bilateral squamosal suture fusion with a 4.6 × 4.6–cm anterior fontanelle ( Fig. 2 ). Ventriculomegaly was not observed. FIG. 1. Abnormal head shape, with bilateral bulging of the parietal regions. FIG. 2. 3D-CT scans showing the fusion at both the parietosquamosal and parietomastoid sutures, with bulging of both parietal bones