Background:In 1900, Joseph Blake described a transient posterior evagination of the tela choroidea of the fourth ventricle in the normal 130-day old human embryo. He was the first to recognize and fully elucidate on the real nature of the foramen of Magendie as an aperture, which develops within a saccular expansion of the embryonic fourth ventricular cavity. The persistence of this temporary fourth ventricular outpouching into the postnatal period and its significance either as separate entity or as an entity within the Dandy–Walker continuum has over the years been one of the most controversial topics in both neurosurgical and neuroradiological literature.Methods:A search of the medical literature was conducted for publications addressing the historical, embryological, and neuororadiological features as well as the clinical presentation and management of persistent Blake's pouch.Results:The literature on the various features of Blake's pouch cyst has limited areas of consensus between various authors.Conclusion:Blake's pouch cyst is a rare entity that is thought to belong to the Dandy–Walker continuum. It has a variable clinical presentation and when symptomatic can be treated with an endoscopic third ventriculostomy or shunting.
John Edwin Scarff (1898-1978) was one of the pioneers of neuroendoscopy and the head of the Department of Neurological Surgery at Columbia University in New York from 1947 to 1949. In this article, we highlight the pioneering and longstanding efforts of John E. Scarff in support of endoscopic choroid plexus coagulation. These efforts represent an important part of the rich history of neuroendoscopy and a legacy to which the current procedure owes a great credit.
Background:We report a case of a neonate with proximal spinal muscular atrophy (SMA) type 1 (also known as Werdnig-Hoffmann disease or severe infantile acute SMA) associated with a Blake's pouch cyst; a malformation that is currently classified within the spectrum of Dandy-Walker complex. The association of the two conditions has not been previously reported in the English literature. A comprehensive review of the pertinent literature is presented.Case Description:A male neonate was noted to have paucity of movement of the four limbs with difficulty of breathing and poor feeding soon after birth. Respiratory distress with tachypnea, necessitated endotracheal intubation and mechanical ventilation. Pregnancy was uneventful except for decreased fetal movements reported by the mother during the third trimester. Neurological examination revealed generalized hypotonia with decreased muscle power of all limbs, nonelicitable deep tendon jerks, and occasional tongue fasciculations. Molecular genetic evaluation revealed a homozygous deletion of both exons 7 and 8 of the survival motor neuron 1 (SMN1) gene, and exon 5 of the neuronal apoptosis inhibitory protein (NAIP) gene on the long arm of chromosome 5 consistent with Werdnig-Hoffmann disease (SMA type 1). At the age of 5 months, a full anterior fontanelle and abnormal increase of the occipito-frontal circumference were noted. Computed tomographic (CT) scan and magnetic resonance imaging (MRI) of the brain revealed a tetraventricular hydrocephalus and features of Blake's pouch cyst of the fourth ventricle.Conclusions:This case represents a previously unreported association of Blake's pouch cyst and SMA type 1.
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