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Posterior fossa syndrome

Anna Maria Pinto, Valentina Imperatore, Laura Bianciardi, Margherita Baldassarri, Paolo Galluzzi, Simone Furini, Giovanni Centini, Alessandra Renieri, Francesca Mari
Orofacial clefts are the most common congenital craniofacial anomalies and can occur as an isolated defect or be associated with other anomalies such as posterior fossa anomalies as a part of several genetic syndromes. We report two consecutive voluntary pregnancy interruptions in a nonconsanguineous couple following the fetal ultrasound finding of cleft lip and palate and posterior fossa anomalies confirmed by means of post-termination examination on the second fetus. The quantitative fluorescent PCR, the karyotype, and the comparative genomic hybridization-array analysis after amniocentesis were normal...
October 4, 2016: Clinical Dysmorphology
Yi-Ju Ho, Hung-Chi Chen, Shirley H L Chang, Lung-Kung Yeh, David Hui-Kang Ma
BACKGROUND: Sclerocornea, a congenital corneal pathology characterized by bilateral scleralization of the cornea, which can be found in few cases with posterior fossa malformationshemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe (PHACES) syndrome. Presence of vascularization in peripheral cornea and smaller diameter of recipient cornea correlate to poor outcome of penetrating keratoplasty (PKP) in sclerocornea. Here we report a method to preserve limbus during PKP for small, irregular, and scleralized cornea...
October 2016: Medicine (Baltimore)
T Struffert
Many important structures are located in the confined space within the posterior cranial fossa. This article describes the main aspects of the anatomy. As a uniform classification of malformations of the posterior cranial fossa does not exist the main syndromes, such as Chiari malformations, zerebellar hypoplasia and dysplasia are discussed separately.
October 13, 2016: Der Radiologe
Sean D McEvoy, Amy Lee, Andrew Poliakov, Seth Friedman, Dennis Shaw, Samuel R Browd, Richard G Ellenbogen, Jeffrey G Ojemann, Christine L Mac Donald
Posterior fossa syndrome is a severe transient loss of language that frequently complicates resection of tumors of the cerebellum. The associated pathophysiology and relevant anatomy to this language deficit remains controversial. We performed a retrospective analysis of all cerebellar tumor resections at Seattle Children's Hospital from 2010 to 2015. Diffusion tensor imaging was performed on each of the patients as part of their clinical scan. Patients included in the study were divided into groups based on language functioning following resection: intact (N = 19), mild deficit (N = 19), and posterior fossa syndrome (N = 9)...
2016: NeuroImage: Clinical
Manoel Sarno, Marcelo Aquino, Kleber Pimentel, Renata Cabral, Gisela Costa, Fernanda Bastos, Carlos Brites
OBJECTIVE: Our aim was to describe the pattern and progression of Central Nervous System (CNS) lesions of microcephalic fetuses with suspected Zika Virus (ZIKV) infection. METHODS: We analyzed fetuses diagnosed with microcephaly after a routine primary care ultrasound scan suspect a fetal microcephaly in Salvador, Brazil, from July/2015 to February/2016 and these pregnancies were followed until delivery. Microcephaly was diagnosed when the head circumference was below two or more standard deviation for gestational age and its relationship with ZIKV infection was defined according to World Health Organization criteria...
September 19, 2016: Ultrasound in Obstetrics & Gynecology
Jing Liu, Jiong Qin
Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological entity affecting the posterior brain, i.e. occipital and parietal lobes. The syndrome are characterized by headaches, altered mental status, seizures, and visual disturbances. Although the pathogenesis remains unclear, endothelial dysfunction may be a key factor. The basic disease may play a crucial role in the incidence of PRES. In most cases, PRES resolves spontaneously and patients show both clinical and radiological improvements...
August 2016: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Mario Manto, Christophe Habas
Cerebellar disorders, also called cerebellar ataxias, comprise a large group of sporadic and genetic diseases. Their core clinical features include impaired control of coordination and gait, as well as cognitive/behavioral deficits usually not detectable by a standard neurologic examination and therefore often overlooked. Two forms of cognitive/behavioral syndromes are now well identified: (1) the cerebellar cognitive affective syndrome, which combines an impairment of executive functions, including planning and working memory, deficits in visuospatial skills, linguistic deficiencies such as agrammatism, and inappropriate behavior; and (2) the posterior fossa syndrome, a very acute form of cerebellar cognitive affective syndrome occurring essentially in children...
2016: Handbook of Clinical Neurology
Stylianos Pikis, José E Cohen, Emil Margolin
We report a rare case of a 45-year-old female with an unruptured basilar artery dissecting aneurysm presenting with locked-in syndrome due to brainstem ischemia eleven months following resection of a giant cerebellopontine angle epidermoid cyst and three months after insertion of ventriculo peritoneal shunt due to hydrocephalus. The etiology of basilar artery dissection and the effect of hydrocephalus and ventricular cerebrospinal fluid drainage on disease progression in this patient are unclear. Our report suggests a possible effect of hydrocephalus and ventricular cerebrospinal fluid drainage on intracranial arterial dissection progression...
October 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Cheryl L Brosig, Dawn H Siegel, Anita N Haggstrom, Ilona J Frieden, Beth A Drolet
BACKGROUND: Practitioners who work with children with posterior fossa, facial hemangiomas, arterial anomalies, cardiovascular anomalies, and abnormalities of the eye (PHACE) syndrome need information about neurodevelopmental outcomes to provide appropriate anticipatory guidance and education for parents. This study aimed to determine the neurodevelopmental outcomes in children with PHACE syndrome and identify which children may be at greatest risk for delays. METHODS: Children with a diagnosis of PHACE syndrome (ages 4-18 yrs) were recruited from the PHACE Syndrome International Clinical Registry and Genetic Repository...
July 2016: Pediatric Dermatology
Michael P Sherman, David H Adamkin, Victoria Niklas, Paula Radmacher, Jan Sherman, Fiona Wertheimer, Karel Petrak
OBJECTIVE: To evaluate the safety and explore the efficacy of recombinant human lactoferrin (talactoferrin [TLf]) to reduce infection. STUDY DESIGN: We conducted a randomized, double blind, placebo-controlled trial in infants with birth weight of 750-1500 g. Infants received enteral TLf (n = 60) or placebo (n = 60) on days 1 through 28 of life; the TLf dose was 150 mg/kg every 12 hours. Primary outcomes were bacteremia, pneumonia, urinary tract infection, meningitis, and necrotizing enterocolitis (NEC)...
August 2016: Journal of Pediatrics
Preeti Patil-Chhablani, Sowmya Murthy, Meenakshi Swaminathan
BACKGROUND: Facial nerve palsy (FNP) occurs less frequently in children as compared to adults but most cases are secondary to an identifiable cause. These children may have a variety of ocular and systemic features associated with the palsy and need detailed ophthalmic and systemic evaluation. METHODS: This was a retrospective chart review of all the cases of FNP below the age of 16 years, presenting to a tertiary ophthalmic hospital over the period of 9 years, from January 2000 to December 2008...
December 2015: Eye Science
Amandeep Kumar, Mohit Agrawal, Surya Prakash, Shambanduram Somorendra, Pankaj Kumar Singh, Ajay Garg, Manmohan Singh, Bhawani Shanker Sharma
BACKGROUND: Type I Chiari malformation (CMI) is a rare complication of lumbar cerebrospinal fluid (CSF) drainage that is usually reported after lumbar drain or lumboperitoneal shunt placement. It usually remains asymptomatic; however, even if it becomes symptomatic, symptoms are usually mild. There are only a few reports of acute foramen magnum syndrome following continuous lumbar CSF drainage, and acute foramen magnum syndrome after a single diagnostic lumbar puncture (LP) has not been previously reported...
July 2016: World Neurosurgery
Aintzane Urbizu, Alex Ferré, Maria-Antonia Poca, Alex Rovira, Juan Sahuquillo, Bryn A Martin, Alfons Macaya
OBJECTIVE Traditionally, Chiari malformation Type I has been related to downward herniation of the cerebellar tonsils as a consequence of an underdeveloped posterior cranial fossa. Although the common symptoms of Chiari malformation Type I are occipital headaches, cervical pain, dizziness, paresthesia, and sensory loss, patients often report symptoms related to pharyngeal dysfunction such as choking, regurgitation, dysphagia, aspiration, chronic cough, and sleep disorders. In addition, tracheal intubation is often difficult in these patients...
May 6, 2016: Journal of Neurosurgery
Kimberly A Foster, William J Ares, Zachary J Tempel, Andrew A McCormick, Ashok Panigrahy, Lorelei J Grunwaldt, Stephanie Greene
INTRODUCTION: PHACE syndrome is a neurocutaneous disorder involving large facial hemangiomas in association with posterior fossa abnormalities, cerebral arterial anomalies, cardiac defects, and eye abnormalities. A recent consensus statement has delineated criteria necessary for the diagnosis of PHACE syndrome. Extracutaneous manifestations of PHACE syndrome predominately affect the cerebrovascular system. To date, there are no reports of cerebral cavernous malformations (CCMs) in children with PHACE syndrome...
August 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Takashi Hitosugi, Masanori Tsukamoto, Shigeki Fujiwara, Takeshi Yokoyama
Dandy-Walker syndrome (DWS) is characterized by perfect or partial defect of the cerebellum vermis and cystic dilatation of the posterior fossa communicating with the fourth ventricle. Common clinical signs are mental retardation, cerebellar ataxia, and those of increased intracranial pressure (ICP). Associated congenital anomalies are craniofacial, cardiac, renal, and skeletal abnormalities. We experienced a case of intravenous sedation and six times of "the same day" general anesthesia for a school-aged boy (10-13 years old) with DWS and hypodentinogenesis...
March 2016: Masui. the Japanese Journal of Anesthesiology
Houssein A Darwish, Edward H Oldfield
This report describes the circumstances of a patient with a cauda equina syndrome due to the development of a lumbar subdural CSF collection with ventral displacement of the cauda equina shortly following posterior fossa decompression for Chiari malformation Type I (CM-I). This unusual, but clinically significant, complication was successfully treated with percutaneous drainage of the extraarachnoid CSF collection. Although there are a few cases of intracranial subdural hygroma developing after surgery for CM-I, often attributed to a pinhole opening in the arachnoid, as far as the authors can determine, a spinal subdural hygroma associated with surgery for CM-I has not been recognized...
September 2016: Journal of Neurosurgery. Spine
Ursula G Schulz, Urs Fischer
One in five strokes affects the posterior circulation. Diagnosing posterior circulation stroke can be challenging, as the vascular anatomy can be variable, and because presenting symptoms are often non-specific and fluctuating. Nevertheless, making the correct diagnosis is important, as these strokes have a high chance of recurrence, can be life threatening, and can lead to equally life-threatening complications. Investigation and management largely follow those for stroke in general, although some specific differences exist...
April 12, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
Nisha Gadgil, Daniel Hansen, James Barry, Rocky Chang, Sandi Lam
No abstract text is available yet for this article.
2016: Surgical Neurology International
Vikash S Oza, Dana F Feigenbaum, Cyril Jacquot, Christopher P Hess, Dawn Siegel, Ilona J Frieden
This report describes the clinical, radiologic, and autopsy findings of a newborn with PHACE syndrome (posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects, and eye anomalies) and fetal alcohol spectrum disorder. To our knowledge, the concurrence of these conditions has not been reported in the literature.
April 4, 2016: Pediatric Dermatology
Lipika Nayak, Bhagabat Nayak, Gautam Sinha, Sudarshan Khokhar
An 11-month-old baby girl presented with white reflex in her left eye. On examination, there was a 6.5×5 mm(2)haemangioma present over her face involving on her lower lip. Systemic examinations were within normal limits. The left eye was small, with an axial length of 16.08 mm and had a cataract. Ultrasonography of the left eye was suggestive of the presence of a vascular stalk, persistent hyperplasia of a primary vitreous, or persistent fetal vasculature with vitreous haemorrhage. On MRI, the left eye was small with vitreous haemorrhage...
March 31, 2016: BMJ Case Reports
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