Cavum septum pellucidum and obstructive hydrocephalus.

Silbert, Peter L.; Gubbay, S S; Vaughan, Richard J.

doi:10.1136/jnnp.56.7.820

Cited by 43 publications

(33 citation statements)

References 12 publications

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“…In contrast, the less frequent noncommunicating cava, originally asymptomatic [1, 29,31] may enlarge, obliterate the foramen of Monro and/or the aqueduct of Sylvius and cause symptoms of internal hydrocephalus [9,31]. These symptomatic cava are exceptional [5,8,12,31,32], usually called septum pellucidum or cavum Vergae cysts [4, 5, 7-9, 31, 32] and justify surgery in most cases. In the present study, we describe the morphological, histological and histo-immunological characteristics of an additional case of septum pellucidum-cavum Vergae cyst communicating with the leptomeningeal space of the anterior interhemispheric fissure.…”

Section: Introductionmentioning

confidence: 89%

Morphology and immunohistochemistry of a symptomatic septum pellucidum cavum Vergae cyst in man

1997

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The cavum septi pellucidi (CSP) and cavum Vergae (CV) are persistent, primitive, or acquired, midline structures of adult human brain. It is customary to distinguish between the non-communicating and the communicating cava, depending on whether the cavum communicates with the cerebral ventricular system or not. Only a few cases of symptomatic non-communicating cava, called septum pellucidum and cavum Vergae cysts, have been described in the literature. In this study, the authors describe the morphological, histological and histo-immunological characteristics of an additional case of septum pellucidum-cavum Vergae cyst in a forty-year-old man who died the day following a ventriculo-peritoneal shunt. We have found a communication between the CSP and the leptomeningeal space of the anterior interhemispheric fissure, in the absence of subarachnoid haemorrhage. The authors discuss the origin of the intracystic fluid and the classification of the CSP.

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Section: Introductionmentioning

confidence: 89%

Morphology and immunohistochemistry of a symptomatic septum pellucidum cavum Vergae cyst in man

1997

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“…Cysts of the cavum septi pellucidi or cavum vergae have been associated with mild to moderate ventriculomegaly and, in older individuals, with various neurologic signs and symptoms, including epilepsy-like seizures, loss of appetite, and daily episodes of vomiting. [7][8][9][10][11][12][13][14][15][16] The association of a cavum vergae cyst with hydrocephalus has been reported in several children and adults, 17 and the literature reflects various theories about that association. Shaw and Alvord 7 suggested that obstruction of the foramina of Monro or rupture of the thin leaves of the cavum may induce the development of hydrocephalus.…”

Section: Discussionmentioning

confidence: 98%

Prenatal sonographic diagnosis of dilated cavum vergae

Şahinoğlu

Uludoǧan

Delikara

2002

J of Clinical Ultrasound

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Several cases of enlarged cavum vergae have been reported, but prenatal diagnosis of this condition is very rare. We report 3 cases of dilated cavum vergae diagnosed prenatally using sonography. In 1 of the 3 fetuses, ventriculomegaly and lumbar meningomyelocele were additional sonographic findings. In 1 of the 3 infants, a stereotactic cyst-peritoneal shunt was placed at 6 months of age to relieve intracranial hypertension due to progressive enlargement of the cavum vergae. The infant who had a meningomyelocele required surgical repair of this defect shortly after birth; in the third infant, the dilated cavum vergae remained asymptomatic, and no surgery was necessary. When interhemispheric cystic lesions are identified prenatally, physicians must distinguish them from pathologic cysts and determine whether associated malformations are present. Sonography is useful for both the differential diagnosis and identification of associated anomalies.

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“…Regarding the cystic CSP, some reports in patients without MPPH have described intermittent hydrocephalus from its enlargement, with improvement or resolution of symptoms following treatment. 6 In MPPH syndrome, a cystic CSP has been described in all except 1 patient, a higher occurrence rate than that in the general infant population of 10%-40%. 2,5,7,8 Hence, the cystic CSP in our patient could have related to chronic low-level hydrocephalus, with a decreased size from extraventricular decompression outside the ventricles, because the subarachnoid spaces along the convexities were more prominent on follow-up MR imaging.…”

Section: Case Reportmentioning

confidence: 99%

“…We also report an elongated pituitary infundibulum along with a regressing cystic cavum septum pellucidum (CSP); cystic CSP has been described previously in MPPH, but without reports of regression. 2,5,6 We discuss how to distinguish MPPH from other syndromes presenting with polymicrogyria or megalencephaly. …”

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confidence: 99%

Syndrome of Megalencephaly, Polydactyly, and Polymicrogyria Lacking Frank Hydrocephalus, with Associated MR Imaging Findings: Fig 1.

Töre¹,

McKinney²,

Nagar

et al. 2009

AJNR Am J Neuroradiol

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SUMMARY:Megalencephaly, polymicrogyria, polydactyly, and hydrocephalus (MPPH) syndrome has been recently recognized and is very rare. Each case reported so far has demonstrated hydrocephalus to varying degrees. We report an infant with MPPH syndrome, but lacking frank hydrocephalus. The additional finding of an abnormally elongated pituitary infundibulum has not been described in this syndrome and, along with the presence of a regressing cystic cavum septum pellucidum, suggests that chronic underlying hydrocephalus may have been present. Megalencephaly, polymicrogyria, polydactyly, and hydrocephalus (MPPH) syndrome was first described in 2004, with only 9 cases reported so far; hence, the prevalence has not yet been determined.1,2 Hydrocephalus has been considered a mandatory component of this syndrome. [1][2][3][4] We report a patient with macrocephaly, polymicrogyria, and polydactyly, but without overt hydrocephalus. We also report an elongated pituitary infundibulum along with a regressing cystic cavum septum pellucidum (CSP); cystic CSP has been described previously in MPPH, but without reports of regression. 2,5,6 We discuss how to distinguish MPPH from other syndromes presenting with polymicrogyria or megalencephaly. Case ReportA 1-day-old boy was born to a healthy nonconsanguineous family at 39-weeks' gestation. Findings of prenatal sonography had suggested mildly dilated lateral and third ventricles, but with an otherwise unremarkable prenatal follow-up. After initially low Apgar scores but subsequent improvement with bag-mask ventilation, the patient was admitted to the neonatal intensive care unit with normal breathing. On physical examination, he was macrocephalic (head circumference Ͼ3.5 SDs above normal) with normal body length and weight, but with bilateral sixth digits along the ulnar aspects of the hands on plain films ( Fig 1A). He had low-set ears, a high arching palate without cleft or cyst, and esotropia. No hypotonia/hypertonia was present, and deep tendon reflexes were normal. Results of blood biochemistry, cultures, and investigations for metabolic disease were unremarkable. Findings of cytogenetic analysis were unrevealing. Kidney and cardiac ultrasound findings were unremarkable.Cranial sonography was then performed, which suggested subtle lateral ventricular dilation. Thereafter, MR imaging demonstrated diffuse polymicrogyria bilaterally, favoring the posterior frontal lobes and perisylvian regions (Fig 1B, -C). Additional findings included an elongated pituitary infundibulum without other sellar abnormalities.A cystic CSP was present, measuring 1.5-cm maximum transverse diameter (Fig 1C). The myelination pattern appeared normal. There was mild colpocephaly of the lateral ventricles, but without enlargement of the anterior recesses of the third ventricle or of the temporal horns of the lateral ventricles (Fig 1D). The midbrain and corpus callosum appeared normal.At 7 months, the patient developed seizures, which were controlled by medication. An electroencephalogram indicated seizure ac...

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Cavum septum pellucidum and obstructive hydrocephalus.

Cited by 43 publications

References 12 publications

Morphology and immunohistochemistry of a symptomatic septum pellucidum cavum Vergae cyst in man

Morphology and immunohistochemistry of a symptomatic septum pellucidum cavum Vergae cyst in man

Prenatal sonographic diagnosis of dilated cavum vergae

Syndrome of Megalencephaly, Polydactyly, and Polymicrogyria Lacking Frank Hydrocephalus, with Associated MR Imaging Findings: Fig 1.

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