Chiari III Malformation on Prenatal and Postnatal Imaging Complicated by Syndrome of Inappropriate Secretion of Anti-diuretic Hormone (SIADH) and Serratia marcescens Meningitis

Chiari III malformation in a neonate

“…Twenty-one of these cases were either lost to follow-up or their outcome was not mentioned in the literature. Of the rest, 11 cases had a good outcome with minimal or no neurological deficits, 12 cases showed mental retardation with or without global developmental delay, 3 developed infantile spasms or seizures, 2 required continued ventilatory support, 1 developed CNS infection, 1 was stillborn, 1 developed abnormal movements and 7 patients passed away, either due to immediate postoperative complications or later in life due to pneumonia 1 5 12 13 19 20. Given this data, about 18% of the total cases had good outcomes that we know of.…”

“…These are classified based on location, with occipital being the most common type, followed by sincipital and then basal. An isolated occipital encephalocele in contrast to CMIII is typically skin covered and not associated with the crowding of the posterior fossa and foramen magnum and herniation of contents into the cervical spine 3–5. The exact aetiology for CMIII is unknown, but one proposed theory is incorrect neurulation during the ventricle expansion causing an imperfect formation of the occipital area leading to a prolapse of the cerebellum and brainstem.…”

“…The differential diagnosis for occipital encephalocele includes dermoid cysts, haemangiomas, vascular malformations of the scalp and cystic hygroma 3 4. The differential for CMIII includes isolated occipital encephalocele and various syndromic causes, such as Meckel-Gruber syndrome, Mullerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia and Walker-Warburg syndrome 5. In our patient, there were no features suggestive of a syndromic diagnosis, and the patient’s clinical presentation and radiological findings pointed towards CMIII with occipital encephalocele.…”

Neonatal Ventricular Reservoir Implantation for Hydrocephalus Management in Chiari III Malformations: A Case Report

“…Our patient exhibited neither, displaying normal skin and no respiratory issues, thus not requiring emergency surgery [ 2 ]. The diagnosis of Chiari malformation is typically established through fetal MRI or ultrasound [ 16 ]. In our patient, the diagnosis had already been confirmed via ultrasound during pregnancy.…”