The Journal of Contemporary Dental Practice

Register      Login

SEARCH WITHIN CONTENT

FIND ARTICLE

Volume / Issue

Online First

Archive
Related articles

VOLUME 18 , ISSUE 8 ( August, 2017 ) > List of Articles

CASE REPORT

Lingual Frenectomy in Joubert Syndrome

Vivek Gurjar, Minal Gurjar, Channaveer Pattanshetti, Banashree Sankeshwari

Citation Information : Gurjar V, Gurjar M, Pattanshetti C, Sankeshwari B. Lingual Frenectomy in Joubert Syndrome. J Contemp Dent Pract 2017; 18 (8):728-731.

DOI: 10.5005/jp-journals-10024-2115

Published Online: 01-08-2017

Copyright Statement:  Copyright © 2017; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Aim

Our aim is to present a case of a rare disorder, such as Joubert syndrome (JS) which required oral surgical intervention under general anesthesia at a very young age to help in achieving normal developmental milestones.

Background

Ciliopathies are an emerging class of diseases of which JS is a significant member. There have been very few cases of JS with its distinguishing features which have been reported in recent literature.

Case report

We herewith present a case of JS who reported to our unit with a complaint of speech abnormality. Intraoral examination revealed a tongue-tie which was planned for surgical release. As any drugs that interfere with respiratory depression have to be used with utmost care, adequate precautions were taken in this case and the patient was treated under general anesthesia. The tongue-tie was released and associated hamartomatous outgrowths from the floor of the mouth were also excised. Appropriate postoperative care was taken and the patient was discharged.

Conclusion

The patient treated by us did not reveal any significant intraoperative or postoperative complications. Treatment of cases of JS required a team effort of multiple dental and medical specialists. Long-term follow-up of such patients is required so that they achieve normal chronological development.

Clinical significance

Joubert syndrome being a rare disorder requires special anesthetic care during any surgical procedure, especially that involving the oral cavity. The need and timing of surgical intervention in such cases should be carefully assessed and appropriate precautions should be taken.

How to cite this article

Gurjar V, Gurjar M, Pattanshetti C, Sankeshwari B. Lingual Frenectomy in Joubert Syndrome. J Contemp Dent Pract 2017;18(8):728-731.


PDF Share
  1. Joubert syndrome and related disorders. Orphanet J Rare Dis 2010 Jul;5:20.
  2. Clinical features and revised diagnostic criteria in Joubert syndrome. J Child Neurol 1999 Sep;14(9):583-590.
  3. Cerebellar involvement in metabolic disorders: a pattern-recognition approach. Neuroradiology 1998 Jun;40(6):347-354.
  4. Joubert syndrome: a review. Am J Med Genet 1992 Jul;43(4):726-731.
  5. Follow-up in children with Joubert syndrome. Neuropediatrics 1997 Aug;28(4):204-211.
  6. Familial agenesis of the cerebellar vermis. A syndrome of episodic hyperpnea, abnormal eye movements, ataxia, and retardation. Neurology 1969 Sep;19(9):813-825.
  7. Joubert syndrome: insights into brain development, cilium biology, and complex disease. Semin Pediatr Neurol 2009 Sep;16(3):143-154.
  8. Joubert syndrome: long-term follow-up. Dev Med Child Neurol 2004 Oct;46(10):694-699.
  9. Molar tooth sign of the midbrain-hindbrain junction: occurrence in multiple distinct syndromes. Am J Med Genet A 2004 Mar;125A(2):125-134.
  10. Joubert syndrome and related disorders: spectrum of neuroimaging findings in 75 patients. AJNR Am J Neuroradiol 2011 Sep;32(8):1459-1463.
  11. Renal disease in Arima syndrome is nephronophthisis as in other Joubert-related cerebello-oculo-renal syndromes. Am J Med Genet A 2004 Nov;131(1):71-76.
  12. Case 25: Joubert syndrome. Radiology 2000 Aug;216(2):379-382.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.