Ventriculoperitoneal Shunt Alone for Spontaneous Cerebrospinal Fluid Rhinorrhea as a Presenting Symptom of Hemi-hydranencephaly.

A 27-year-old female patient presented with chronic spontaneous cerebrospinal fluid (CSF) rhinorrhea. She had deformity and weakness on the left side since childhood. Imaging examinations demonstrated hemi-hydranencephaly with a nearly complete absence of the right cerebral hemisphere, which was replaced with a membranous sac filled with CSF.

A forensic case of hydranencephaly in a preterm neonate fully documented by postmortem imaging techniques.

Unlabelled: The authors present a medico-legal autopsy case of hydranencephaly in a male preterm newborn, fully documented by postmortem unenhanced and enhanced imaging techniques (postmortem computed tomography and postmortem magnetic resonance imaging). Hydranencephaly is a congenital anomaly of the central nervous system, consisting in almost complete absence of the cerebral hemispheres and replacement of the cerebral parenchyma by cerebrospinal fluid, rarely encountered in forensic medical practice. A premature baby was born during the supposed 22nd and 24th week of pregnancy in the context of a denial of pregnancy without any follow-up.

Unveiling what is absent within: illustrating anesthetic considerations in a patient with hydranencephaly - a case report.

Background: Hydranencephaly is a rare and debilitating congenital condition in which most anesthesiologists are unfamiliar. Primary surgical treatment involves CSF diversion, though other palliative procedures requiring anesthesia are often required. With medical advancements and a resulting prolonged life expectancy, caring for these patients is becoming more routine.

Expanding the clinical spectrum of Fowler syndrome: Three siblings with survival into adulthood and systematic review of the literature.

Proliferative vasculopathy and hydranencephaly-hydrocephaly syndrome (PVHH, OMIM 225790), also known as Fowler syndrome, is a rare autosomal recessive disorder of brain angiogenesis. PVHH has long been considered to be prenatally lethal. We evaluated the phenotypes of the first three siblings with survival into adulthood, performed a systematic review of the Fowler syndrome literature and delineated genotype-phenotype correlations using a scoring system to rate the severity of the disease.

The Benefits of a Guideline on Safe Termination of Pregnancy for Legal Indications: An Illustrative Case Report of a Hydranencephaly.

After years of the worsening burden of unsafe abortion and attendant morbidities and mortalities in Nigeria, a National Guideline on the Safe termination of pregnancy for legal indications was enunciated. This report presents and discusses an illustrative case of a hydranencephaly that benefited from it. A 43-year old multipara was informed during routine ultrasonography at booking for antenatal care, at 16 weeks of gestation, of a major defect in her baby and advised to meet her physician.

An unusual presentation of bobble-head doll syndrome in a patient with hydranencephaly and Chiari 3 malformation.

Bobble-head doll syndrome is a rare movement disorder that is usually associated with lesions involving the third ventricle. It is characterised by stereotypical rhythmic up-and-down or side-to-side head movements. The pathophysiology and anatomical basis for this unusual manifestation is still a subject of intense scrutiny.

Phenotypic spectrum of NDE1-related disorders: from microlissencephaly to microhydranencephaly.

Biallelic variants in the NDE1 gene have been shown to occur in extreme microcephaly. Most of the patients displayed microlissencephaly but one with microhydranencephaly. We report on three sibs in which the brain MRI and CT scans demonstrated variable degree of reduced volume of cerebral hemispheres and ventriculomegaly.

Prenatal diagnosis of hydrancephaly and enlarged cerebellum and cisterna magna in a fetus with thanatophoric dysplasia type II and a review of prenatal diagnosis of brain anomalies associated with thanatophoric dysplasia.

Objective: We present prenatal diagnosis of hydrancephaly and enlarged cerebellum and cisterna magna in a fetus with thanatophoric dysplasia type II (TD2) and a review of prenatal diagnosis of brain anomalies associated with TD.

Case Report: A 33-year-old woman was referred for genetic counseling at 25 weeks of gestation because of fetal ultrasound abnormalities. Prenatal ultrasound at 14 weeks of gestation revealed an increased nuchal translucency (NT) and hydrocephalus.