Cervical myelomeningocele with CSF leakage: a case-based review

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CASE-BASED REVIEW

Cervical myelomeningocele with CSF leakage: a case-based review Kevin Armas-Melián 1,2 & Sara Iglesias 1 & Bienvenido Ros 1 & María Isabel Martínez-León 3 & Miguel Ángel Arráez 1 Received: 21 April 2020 / Accepted: 11 June 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020

Abstract Background The cystic spinal dysraphism of the cervical and upper thoracic region (CDCT) accounts for only 3.9 to 8% of spina bifida cystica lesions. The presence of external cerebrospinal fluid (CSF) leakage is infrequent and very few authors have reported about surgical complications. Case report We present the case of a new born diagnosed of CDCT C1-C3 with a stalk of fibrovascular tissue, CSF leakage, hydrocephalus, and type Chiari II malformation, discuss about the chosen surgical technique and the associated complications, and make a review of the literature focusing on the main aspects of CDCT. Conclusions The CDCT with a stalk of neuroglial and/or fibrovascular tissue originates from the dorsal surface of the spinal cord and penetrates into a cervical cystic sac. The presence of CSF leakage, hydrocephalus, and/or type Chiari II malformation can influence to decide the most appropriate surgical technique. Keywords Spinal dysraphism . Cervical myelomeningocele . Cervical meningocele . Spina bifida cystica . CSF leakage

Introduction Posterior midline cutaneous mass lesions of the cervical and upper thoracic region have been described under different terms: cervical meningoceles [1, 2], cervical myelomeningoceles (cMMC) [3], cervical spine atretic myelomeningocele [4], limited dorsal myeloschisis [5], and myelocystocele [1, 4, 6–9]. Recently, Salomão et al. [2] defined all lesions as cystic spinal dysraphism of the cervical and upper thoracic region (CDCT). Regarding etiology, there has been described genetic and environmental factors [10, 11]. One of them is maternal folic acid deficiency, although supplemental folic acid is capable of preventing only one half of the cases of cervical spinal dysraphism [12].

* Kevin Armas-Melián [email protected] 1

Neurosurgery Department, Hospital Regional Universitario de Málaga, Málaga, Spain

2

Department of Neurosurgery, Hospital Insular de Las Palmas, Plaza Doctor Pasteur s/n, 35016 Las Palmas de Gran Canaria, Spain

3

Radiology Department, Hospital Regional Universitario de Málaga, Málaga, Spain

External cerebrospinal fluid (CSF) leakage is infrequent in CDCT, and some authors have reported that its presence implies worse control of hydrocephalus and eventual Chiari II malformation [13–15].

Exemplary case description A newborn Moroccan girl, born after an induced labor due to suspected hydrocephalus, was admitted to our hospital in November 2019 because of a congenital posterior cervical mass with CSF leakage. Her mother reported poor pregnancy control due to the socio-sanitary conditions of her country of origin and she did not ingest folic acid. Clinical examination revealed a normal neurological status and a protruding sac located in the