A Case Series Outlining the Relationship between Dolichoectasia and Ectrodactyly-Ectodermal Dysplasia-Clefting Syndrome

Ectrodactyly-Ectodermal Dysplasia-Clefting Syndrome (EEC) is a rare heritable condition marked by ectodermal dysplasia with nails, teeth, sweat glands, and hair, eye and lacrimal duct malformations, midfacial hypoplasia, ectrodactyly (loss of fingers or toes), syndactyly, clinodactyly, auricular anomalies, short height, orofacial cleft palates, genitourinary anomalies, malformations within the central nervous system (CNS) leading to mental impairment or hearing loss, nevocellular nevi, flat noses, and hypopigmentation (National Foundation for Ectodermal Dysplasias 2021 and Cyriac & Lashpa 1980). The connection between dolichoectasia and EEC has not yet been outlined in a formal literature review or even case series, so this article aims to explore a series of case reports to better highlight the various etiologies, symptoms, and presentations of EEC as they relate to dolichoectasia. The dilation and elongation of arteries in the cranial cavity is called cerebral arterial dolichoectasia. To date, the correlation between dolichoectasia and EEC has not been studied thoroughly, though one case report specifically mentioned the possibility of those two conditions being frequently paired without delving into the literature to verify this claim. EEC, specifically lobster claw, occurs both in a familial inheritance pattern as well as sporadically.