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Parietal cephalocele: clinical importance of its atretic form and associated malformations

Akira Yokota, Hidehiko Kajiwara, Masato Kohchi, Isao Fuwa, and Hidetaka Wada

S ince the report of McLaurin in 1964, 23 parietal cephaloceles have become well known for their morphological diversity and frequent association with midline brain anomalies. However, only a few authors 22, 23, 27 have mentioned the clinical features and prognosis of patients with a parietal cephalocele, probably because of its rare occurrence. An extremely high correlation has been identified between the presence of some cerebral malformations and a cephalocele or a scalp defect developing in the parietal midline, and it is suggested that these cutaneous

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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

C ephaloceles represent congenital malformations of the central nervous system with protrusion of cranial contents beyond the normal confines of the skull. These malformations range from meningoceles, with protrusion of meninges and CSF, to encephaloceles, with involvement of brain tissue and meninges. 12 Cephaloceles can be classified according to their location, including anterior (basal and sincipital), parietal (vertex), temporal, occipital, and occipitocervical. 9 They account for 10–20% of all craniospinal dysraphisms, with an estimated prevalence

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Transpalatal approach for the extracranial surgical repair of transsphenoidal cephaloceles in children

Erin M. Kennedy, David P. Gruber, David A. Billmire, and Kerry R. Crone

C ephaloceles represent congenital herniations of intracranial contents through a bone defect. They are described as encephaloceles when the sac contains neural elements and meningoceles when no recognizable brain tissue is present within the sac. 18 Cephaloceles are classified according to the site of bone defect and herniation. Suwanwela and Suwanwela 29 introduced the currently used classification system, which includes sincipital (frontal), parietal, occipital, and basal defects. Basal cephaloceles have been further subclassified by Gisselsson, 12 and

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Endoscopic treatment of an oral cephalocele

Case report

Christian Raftopoulos, Philippe David, Serge Allard, Brigitte Ickx, and Danielle Balériaux

A cephalocele consists of a protrusion of intracranial contents through a defect in the skull. When brain tissue is present in the herniated sac, it is called an “encephalocele.” A cephalocele is found in one per 8333 births in the United States 4 and in one per 12,500 births in Australia. 16 The mortality rate can be as high as 44%, whereas intellectual impairment is described in 40% to 91% of cases. 13 The etiology remains unknown. 3, 7 Cephaloceles are classified according to the anatomical site of the skull defect through which the malformation

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Atretic cephalocele: the tip of the iceberg

Juan F. Martinez-Lage, Joaquin Sola, Carlos Casas, M´ximo Poza, Maria J. Almagro, and Diego G. Girona

“rudimentary cephalocele,” 2, 6, 7, 16 referring to a skin-covered subscalp lesion that consists of meninges and rests of glial and/or CNS tissues. The gross appearance of this malformation is that of an unimportant and benign lesion. McLaurin 10 and Yokota, et al. , 16 have stressed the high frequency of associated cerebral malformations with atretic encephaloceles. Recently, Drapkin 6 described a more favorable prognosis for this anomaly, distinct from the true encephalocele. We report our experience with 16 cases of this “inoffensive” congenital defect. To our

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Natural history of posterior fetal cephaloceles and incidence of progressive cephalocele herniation

Nisha Gadgil, Samuel G. McClugage III, Guillermo Aldave, David F. Bauer, Howard L. Weiner, Thierry A. G. M. Huisman, Magdalena Sanz-Cortes, Michael A. Belfort, Lisa Emrick, Gary Clark, Luc Joyeux, and William E. Whitehead

F etal cephaloceles are rare congenital malformations of the central nervous system characterized by the protrusion of cranial contents—meninges only (meningocele) or brain tissue and meninges (encephalocele)—beyond the normal confines of the skull. 1 , 2 They occur in 0.08 to 0.5 per 1000 live births 3 – 6 and can be diagnosed prenatally by ultrasound screening in the 1st trimester. 7 Approximately 50% of affected fetuses die prior to birth. 8 – 10 One-third of those born with encephalocele die within the 1st year of life. 9 Outcomes vary, but

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Anatomical and embryological considerations in the repair of a large vertex cephalocele

Case report

Eelco Hoving, Susan Blaser, Edmond Kelly, and James T. Rutka

A cephalocele is defined as a protrusion of cranial contents beyond the normal confines of the skull. 1, 2, 11, 16 In this definition, the term cephalocele includes meningocele (herniation of meninges and cerebrospinal fluid [CSF]), encephalomeningocele (herniation of brain tissue and meninges), and hydroencephalomeningocele (herniation of a portion of the ventricle, brain tissue, and meninges). Most classifications of cephaloceles are based on the location of the defects in the cranium. 17 A subdivision into six main groups has been proposed: 11 1) occipital

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Cavum trigeminale cephalocele associated with intracranial hypertension in an 18-month-old child: illustrative case

Giovanni Miccoli, Domenico Cicala, Pietro Spennato, Alessia Imperato, Claudio Ruggiero, and Giuseppe Cinalli

Cavum trigeminale cephaloceles (CTCs) are rare lesions that are characterized radiologically by their cystic appearance and extension of the posterolateral aspect of Meckel’s cave into the superomedial portion of the petrous apex. 1 , 2 Although termed “cephaloceles,” these lesions usually do not contain brain tissue as is true for other cephaloceles of the skull base. A differential diagnosis should be done and should include cholesteatoma, trapped fluid, petrous apicitis, mucocele, cholesterol granuloma, and Meckel’s cave schwannoma. 3 , 4 Although

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Sphenoethmoidal cephalocele with cleft palate: transpalatal versus transcranial repair

Report of two cases

Michael L. Lewin

A mong the cerebral cephaloceles, the sincipital type (frontal, occipital, or orbital), which forms external tumefactions, is the most common. Basal encephaloceles are estimated to occur in one of every 35,000 live births. 19 Nasal cephaloceles, which prolapse through the cribiform plate, are relatively frequent and have been successfully operated on through a transcranial approach. However, the sphenoethmoidal cephaloceles, which exit from the cranium through huge defects in the sphenoid and ethmoid bones (including the sella), often contain vital cerebral

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Cerebrospinal fluid leak with recurrent meningitis following tonsillectomy

Case report

Shawn L. Hervey-Jumper, Ahmer K. Ghori, Douglas J. Quint, Lawrence J. Marentette, and Cormac O. Maher

B acterial meningitis is a rare and potentially lifethreatening condition. Among other causes, anatomical, immunological, congenital, and oncological conditions can result in a CSF fistula that links the intradural compartment to the external environment, thus predisposing the patient to recurrent bouts of meningitis. Petrous apex cephaloceles represent a protrusion of meninges and CSF from the Meckel cave into the petrous apex. 6 These lesions are often asymptomatic incidental findings discovered on imaging performed for a variety of clinical concerns. In