Atlantooccipital Assimilation and Basilar Invagination Treated Successfully in a Young Male With Marfanoid Features: A Stitch in Time.

atlantooccipital assimilation basilar invagination craniocervical abnormalities decompression surgery marfanoid features

Journal

Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737

Informations de publication

Date de publication:
Nov 2021
Historique:
accepted: 08 11 2021
entrez: 15 12 2021
pubmed: 16 12 2021
medline: 16 12 2021
Statut: epublish

Résumé

Marfan syndrome is a spectrum of disorders caused by a genetic defect involving connective tissue and is heritable by the autosomal dominant mode of inheritance. Atlantooccipital assimilation is a partial or complete fusion of the atlas and the occiput base congenitally. Although primarily asymptomatic, some patients with atlantooccipital assimilation may present with neurological issues, including myelopathy. Here, we are discussing a case of an 18-year-old male who presented with bilateral paraesthesia, tingling and neck pain which, upon investigations, turned out to be a case of atlantooccipital assimilation along with basilar invagination with spinal cord compression. The patient also had marfanoid features like tall stature, reduced upper to lower segment ratio, and increased arm span to height with positive wrist and thumb signs. As myelopathy had already developed, the patient was treated surgically rather than with medical management with a favorable outcome.

Identifiants

pubmed: 34909321
doi: 10.7759/cureus.19365
pmc: PMC8653923
doi:

Types de publication

Case Reports

Langues

eng

Pagination

e19365

Informations de copyright

Copyright © 2021, Mahajan et al.

Déclaration de conflit d'intérêts

The authors have declared that no competing interests exist.

Références

Acta Neurochir (Wien). 2005 Aug;147(8):889-95
pubmed: 15924208
Anat Sci Int. 2006 Sep;81(3):173-80
pubmed: 16955668
Spine (Phila Pa 1976). 1997 May 1;22(9):983-9
pubmed: 9152448
Cureus. 2017 Jun 9;9(6):e1327
pubmed: 28690960
Sao Paulo Med J. 2010 Dec;128(6):360-6
pubmed: 21308160
Clin Orthop Relat Res. 1980 Oct;(152):211-31
pubmed: 7438606
Clin Ter. 2016 May-Jun;167(3):77-9
pubmed: 27424507
Rev Port Cardiol (Engl Ed). 2020 Apr;39(4):215-226
pubmed: 32439107
Eur Spine J. 2019 Jun;28(Suppl 2):31-36
pubmed: 30291489
Korean J Spine. 2013 Sep;10(3):189-91
pubmed: 24757486

Auteurs

Satish Mahajan (S)

Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Science (Deemed to be University), Wardha, IND.

Dhruv Talwar (D)

Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Science (Deemed to be University), Wardha, IND.

Sunil Kumar (S)

Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Science (Deemed to be University), Wardha, IND.

Sourya Acharya (S)

Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Science (Deemed to be University), Wardha, IND.

Sandeep Iratwar (S)

Department of Neurosurgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Science (Deemed to be University), Wardha, IND.

Akhilesh Annadatha (A)

Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Science (Deemed to be University), Wardha, IND.

Classifications MeSH