Ct Findings in an Alobar Holoprosencephaly Associated With Dandy-Walker's Cyst

Hayashi, Takashi; Kuramoto, Shinken; Takagi, Shigeyuki; Kojyo, Nobuto; Nakayama, Kenji

doi:10.2739/kurumemedj.27.139

Cited by 3 publications

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“…Holoprosencephaly associated with Dandy walker cyst is rare. Till date only few cases have been reported [1][2][3][4][5][6]. Five of them had semilobar and two had alobar type of holoprosencephaly.…”

Section: Discussionmentioning

confidence: 99%

Holoprosencephaly and Dandy Walker Malformation: A Rare Association Presenting as Birth Asphyxia

Mohapatra¹

2014

J Neonatal Biol

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Section: Discussionmentioning

confidence: 99%

Holoprosencephaly and Dandy Walker Malformation: A Rare Association Presenting as Birth Asphyxia

Mohapatra¹

2014

J Neonatal Biol

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Holoprosencephaly with Retroprosencephalic Extracerebral Cyst

Rössing¹,

Friede²

1992

Develop Med Child Neuro

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The fusion of the cerebral hemispheres in holoprosencephalic brains is consistently associated with severe hypoplasia of the neocortex; accordingly, afflicted infants are always microencephalic. The holoprosencephalon contains a bulky common ventricle which opens through a large horseshoe-shaped aperture on the occipital face of the holoprosencephalon. The communication between the rim of this aperture and the midbrain is formed by a thin membrane bulging between the holoprosencephalon and the cerebellum. Some authors describe this membrane as a 'dorsal sac' (Probst 1981), 'dorsal cyst' (Yokota ef al. 1984) or as a 'cyst'. In fact, it r e p r e s e n t s t h e d i s t e n d e d roof of t h e residual ventricle (DeMyer ef al. 1964, Kobori el af. 1987 , Friede 1989. It ruptures easily on removal of the brain, exposing the widely open common ventricle.The following report concerns a rare variant of holoprosencephaly, in which the 'cyst' between the holoprosencephalon and the cerebellum was not formed by the bulging ventricular roof. Instead, the region of the superior cistern was occupied by a true, non-communicating cyst compressing and displacing the malformed brain, causing herniations at the foramen magnum and enlargement of the head. The resultant clinical features mimicked hydranencephaly or hydrocephalus. Case reportThe 10-week-old male infant was admitted with dyspnoea and suspected aspiration pneumonia. Hydranencephaly, muscular hypotonus and a median cleft lip and palate had been diagnosed previously. A computed tomogram taken the day after birth showed a fluid-filled, distended cranium with a thin, flat mass of cerebral tissue at the floor of the anterior fossa (Fig. 1). The child died of cardiac arrest soon after admission.At autopsy bodyweight was 7700g. There were signs of an infantile respiratory distress syndrome, with extensive atelectases of the lung. Mild interstitial.and severe intra-alveolar lung oedema was seen, with no pneumonia. No visceral deformities were found. ,The head was enlarged, with a circumference of S2cm and a biparietal width of lScm (Fig. 2). Eyelids were slanted and eyebrows were fused at the midline. The interpupillary distance was reduced, the nose flat and broad, and there was a cleft lip and cleft palate. The skin of the back was intact and no deformities of the spine were evident.Opening the cranium exposed a large cyst which ruptured and collapsed during examination: approximately two litres of clear fluid were collected. There was neither falx nor superior sagittal sinus. Attached to and pressed against the floor of the anterior fossa was a flattened mass of brain tissue. This was covered by the collapsed membranes of the cyst, which were continuous over the entire cranial base, including the exposed contents of the posterior fossa (Fig. 3). The cyst did not communicate with the common ventricle of the holosphere, nor with the fourth ventricle.The cerebral tissue in the anterior fossa measured approximately l5cm in length and 9cm in width, and weighed 135g. I t consiste...

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Review Article: Holoprosencephaly and Related Midline Cerebral Anomalies: A Review

Leech

Shuman

1986

J Child Neurol

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We propose a simple pathogenetic mechanism that reduces a bewildering variety of central nervous system malformations to a manageable group sharing defects of midline prosencephalic growth. It is neither new nor innovative, but attempts to summarize many pathologic entities within a concept that accounts for known embryologic events and the sequence and timing of those events. We propose midline prosencephalic dysgenesis as a category of malformations including aprosencephaly, holoprosencephaly, septo-optic dysplasia, and agenesis of the corpus callosum.

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Ct Findings in an Alobar Holoprosencephaly Associated With Dandy-Walker's Cyst

Abstract: The authors describe a case of alobar holoprosencephaly with a huge cyst in the posterior fossa, possibly representing a Dandy-Walker's cyst, and marked cerebellar hypoplasia in the absence of midline facial dysmorphias that has recently been encountered at our clinic.

Cited by 3 publications

References 2 publications

Holoprosencephaly and Dandy Walker Malformation: A Rare Association Presenting as Birth Asphyxia

Holoprosencephaly and Dandy Walker Malformation: A Rare Association Presenting as Birth Asphyxia

Holoprosencephaly with Retroprosencephalic Extracerebral Cyst

Review Article: Holoprosencephaly and Related Midline Cerebral Anomalies: A Review

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