Literature DB >> 34909321

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

Satish Mahajan1, Dhruv Talwar1, Sunil Kumar1, Sourya Acharya1, Sandeep Iratwar2, Akhilesh Annadatha1.   

Abstract

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.
Copyright © 2021, Mahajan et al.

Entities:  

Keywords:  atlantooccipital assimilation; basilar invagination; craniocervical abnormalities; decompression surgery; marfanoid features

Year:  2021        PMID: 34909321      PMCID: PMC8653923          DOI: 10.7759/cureus.19365

Source DB:  PubMed          Journal:  Cureus        ISSN: 2168-8184


  10 in total

1.  A morphometric analysis of the foramen magnum region as it relates to the transcondylar approach.

Authors:  N Muthukumar; R Swaminathan; G Venkatesh; S P Bhanumathy
Journal:  Acta Neurochir (Wien)       Date:  2005-06-09       Impact factor: 2.216

2.  Total occipitalization of the atlas.

Authors:  Mohamed Akram Al-Motabagani; Manjappa Surendra
Journal:  Anat Sci Int       Date:  2006-09       Impact factor: 1.741

Review 3.  Marfan's syndrome: an overview.

Authors:  Shi-Min Yuan; Hua Jing
Journal:  Sao Paulo Med J       Date:  2010-12       Impact factor: 1.044

4.  Cranio-vertebral junction anomaly: atlanto-occipital assimilation.

Authors:  - Pooja Jain; - Khursheed Raza; - Chiman Kumari; - Manisha Hansda; - Sb Ray
Journal:  Clin Ter       Date:  2016 May-Jun

Review 5.  Marfan syndrome revisited: From genetics to the clinic.

Authors:  Sónia Gomes Coelho; Ana G Almeida
Journal:  Rev Port Cardiol (Engl Ed)       Date:  2020-05-18

6.  The cervical spine in Marfan syndrome.

Authors:  W R Hobbs; P D Sponseller; A P Weiss; R E Pyeritz
Journal:  Spine (Phila Pa 1976)       Date:  1997-05-01       Impact factor: 3.468

7.  Embryogenesis and prenatal development of congenital vertebral anomalies and their classification.

Authors:  P M Tsou; A Yau; A R Hodgson
Journal:  Clin Orthop Relat Res       Date:  1980-10       Impact factor: 4.176

Review 8.  Rescue Nuss procedure for inferior vena cava compression syndrome following posterior scoliosis surgery in Marfan syndrome.

Authors:  M Löhnhardt; A Hättich; A Andresen; M Stangenberg; T S Mir; K Reinshagen; M Dreimann
Journal:  Eur Spine J       Date:  2018-10-05       Impact factor: 3.134

9.  Cervical Myelopathy Secondary to Atlanto-occipital Assimilation: The Usefulness of the Simple Decompressive Surgery.

Authors:  Kang Rae Kim; Young Min Lee; Young Zoon Kim; Yong Woon Cho; Joon Soo Kim; Kyu Hong Kim; In Chang Lee
Journal:  Korean J Spine       Date:  2013-09-30

10.  Complete Atlantooccipital Assimilation with Basilar Invagination and Atlantoaxial Subluxation Treated Non-Surgically: A Case Report.

Authors:  Ali J Electricwala; Amita Harsule; Vishwajeet Chavan; Jaffer T Electricwala
Journal:  Cureus       Date:  2017-06-09
  10 in total

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