Hemifacial Hyperplasia With Strabismus

Last update 01 Nov 2024

Basic Info

Synonyms

BENCZE SYNDROME, Bencze Syndrome, Bencze syndrome

+ [6]

Introduction

A malformation syndrome involving the abnormal growth of the facial skeleton as well as its soft tissue structure and organs. The syndrome has characteristics of mild facial asymmetry with unaffected neurocranium and eyeballs, along with esotropia, amblyopia and/or convergent strabismus and occasionally submucous cleft palate. Transmission is autosomal dominant. There have been no further descriptions in the literature since 1979.

100 Clinical Results associated with Hemifacial Hyperplasia With Strabismus

100 Translational Medicine associated with Hemifacial Hyperplasia With Strabismus

0 Patents (Medical) associated with Hemifacial Hyperplasia With Strabismus

Literatures (Medical) associated with Hemifacial Hyperplasia With Strabismus

01 Apr 1995·International Journal of Oral and Maxillofacial SurgeryQ3 · MEDICINE

Perspectives on craniofacial asymmetry. IV. Hemi-asymmetries

Q3 · MEDICINE

Review

Author: Cohen, M M

Hemi-asymmetries discussed here include: 1) hemihyperplasia which may affect either one tissue such as bone with secondary regional consequences or multiple tissues on a primary basis such as "hemihypertrophy"; 2) hemihypoplasia which may also affect one tissue such as bone with secondary regional consequences or multiple tissues on a primary basis such as in hemifacial microsomia; 3) hemiatrophy, the quintessential craniofacial disorder being Romberg syndrome; and 4) miscellaneous conditions such as Bencze syndrome and hemimaxillofacial dysplasia.

01 Nov 1979·Clinical GeneticsQ2 · MEDICINE

An autosomal dominantly inherited syndrome of facial asymmetry, esotropia, amblyopia, and submucous cleft palate (Bencze syndrome)

Q2 · MEDICINE

Article

Author: David B. Shurtleff ; Judith G. Hall ; M. Michael Cohen Jr. ; David Kurnit

This is the second report of a dominantly inherited syndrome of facial asymmetry, esotropia, and amblyopia (Bencze syndrome). The phenotypic spectrum is expanded to include submucous cleft palate. The observation for the first time of male‐to‐male transmission seems to confirm an autosomal dominant mode of inheritance. The facial asymmetry in this family was mild and did not require surgical intervention. With the exception of one patient who had other abnormalities, intelligence was normal.

Kathmandu University Medical Journal

The role of the injection Botulinum Toxin A in cases of Essential Blepharospasm Syndrome, Hemifacial Spasm and Meige’s Syndrome

Article

Author: D N Shah ; M Chaudhary ; J P Agrawal ; P Bastola

Background Benign Essential Blepharospasm Symdrome is a neuropathologic disorder. The cause of blepharospasm is multifactorial. It is unlikely that a single defect in this elusive control centre is the primary cause of this disease. Objectives To evaluate the role of botulinum toxin A in treating cases of Essential Blepharospasm Symdrome, Hemifacial Spasm and Meige’s Syndrome, and to assess orbicularis oculi muscle post treatment, in the patients who attended neuro-ophthalmology clinic and the general outpatient department of BP Koirala Lions Centre for Ophthalmic studies. Methods A prospective, interventional study was carried out on all the patients of Essential Blepharospasm Symdrome, Hemifacial Spasm and Meige’s syndrome who underwent treatment with botulinum toxin A in BP Koirala Lions Centre for Ophthalmic studies during a study period of one and half years. Pre- treatment grading of the spasm was done with Jankovic spasm grading and post treatment response was seen with assessment of orbicularis oculi muscle and improvement in functional impairment scale. Relevant findings were noted. Results A total of 40 cases were enrolled in the study. The mean Jankovic spasm grading in cases of essential blepharospasm, hemifacial spasm and Meige’s syndrome was 3.61 (±Standard deviation 0.50, range 3-4), 3.21 (±Standard deviation 0.63, range 2-4) and 3.67 (+- Standard deviation 0.57, range 3-4) respectively. The mean value for reappearance of significant spasms (in months) in cases of essential blepharospasm, hemifacial spasm and Meige’s syndrome was 4.3 (± Standard deviation 1.6, range 2.0-6.5), 5.8 (±Standard deviation 1.4, range 3-8) and 4.5 (± Std.deviation 2.8, range 2.5-6.5) respectively. Blepharoptosis was the commonest complication accounting for 66.6% of the complications.Conclusions The movement disorders like Essential Blepharospasm, Hemifacial Hpasm and Meige’s syndrome are treated by different modalities. An acceptable and effective treatment modality has been a long felt need in these cases. Our study has shown that injection of botulinum toxin A has been a safe and effective method of treating these cases in Nepal.DOI: http://dx.doi.org/10.3126/kumj.v8i3.6217 Kathmandu Univ Med J 2010;8(3):305-10

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Hemifacial Hyperplasia With Strabismus

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