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IMAGES IN NEUROLOGY
Year : 2016  |  Volume : 19  |  Issue : 1  |  Page : 123-124
 

Asymmetric crying facies in a neonate with congenital hypoplasia of depressor anguli oris muscle (CHDAOM)


1 Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India
2 Department of Neurology, Suraj Neurological and Multispecialty Hospital, Navi Mumbai, Maharashtra, India

Date of Submission22-Jun-2015
Date of Decision08-Sep-2015
Date of Acceptance14-Sep-2015
Date of Web Publication2-Feb-2016

Correspondence Address:
Kaustubh S Chaudhari
Type 5, Bldg. No. 5, Flat No. 20, RCF Colony, Chembur, Mumbai - 400 074, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.168638

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How to cite this article:
Jagtap SA, Chaudhari KS. Asymmetric crying facies in a neonate with congenital hypoplasia of depressor anguli oris muscle (CHDAOM). Ann Indian Acad Neurol 2016;19:123-4

How to cite this URL:
Jagtap SA, Chaudhari KS. Asymmetric crying facies in a neonate with congenital hypoplasia of depressor anguli oris muscle (CHDAOM). Ann Indian Acad Neurol [serial online] 2016 [cited 2017 Mar 27];19:123-4. Available from: http://www.annalsofian.org/text.asp?2016/19/1/123/168638



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A 28-day-old neonate born in an uneventful, unassisted vaginal delivery at term was admitted to the hospital for cyanosis. On examination, the baby had asymmetric frowning, complete eye closure, and normal extraocular movements. No facial asymmetry was observed when the baby was quiet and resting [Figure 1]. However, on crying, while the left corner of the mouth deviated downward and outward, the right corner did not [Figure 2]. The lower lip was thin near its right margin. The frontalis, orbicularis oculi, zygomaticus, and mentis muscles functioned adequately.
Figure 1: Symmetrical face at rest with cyanosis

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Figure 2: On crying, the left corner of the mouth deviated to left and downward

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The child had central cyanosis, with normal S1 and single S2. An ejection systolic murmur was heard at the lower left sternal border. Echocardiography revealed double outlet right ventricle (DORV) with pulmonary atresia (PA), transposition of the great arteries (TGA), and patent ductus arteriosus (PDA).

The child was diagnosed with asymmetric facies due to right-sided congenital hypoplasia of the depressor angularis oris muscle (CHDAOM) with cardiac anomalies.

Asymmetric crying facies is found in 3-8/1,000 births. [1] It occurs secondary to nerve compression or faulty muscle development. CHDAOM is a clinical diagnosis, and electrophysiology is normal. It is differentiated from facial nerve palsy by the presence of normal eye closure, no drooling of saliva, and thinning of the lower lip on the affected side. It is associated with other congenital anomalies-such as cardiac syndromes; "CATCH 22" (DiGeorge) syndrome; and the nonrandom co-occurrence of vertebral anomalies, anal atresia, cardiac defects, tracheoesophageal fistula and/or esophageal atresia, renal and radial anomalies, and limb defects (VACTERL association)-that coexist in patients with 45-70% asymmetric crying facies. [2] Asymmetric crying facies due to CHDAOM in patients merits evaluation and treatment of associated anomalies. Familial occurrence has been reported, and the mode of inheritance has been suggested as autosomal dominant inheritance with variable expressivity. [3] If isolated, it is clinically benign and can be corrected for cosmetic purposes by plastic reconstruction surgery. [4] No difficulty is encountered while feeding or speaking. As the child grows up, the other facial muscles compensate for emotive actions. [3] Detection of CHDAOM, however, warrants a thorough evaluation for other birth defects, especially those cardiovascular anomalies.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Dubnov-Raz G, Merlob P, Geva-Dayan K, Blumenthal D, Finkelstein Y. Increased rate of major birth malformations in infants with neonatal "asymmetric crying face": A hospital-based cohort study. Am J Med Genet A 2007;143:305-10.  Back to cited text no. 1
    
2.
Gupta R, Prasad PL. Congenital hypoplasia of depressor angularis oris muscle. Med J Armed Forces India 2009;65:188-9.  Back to cited text no. 2
    
3.
Sapin SO, Miller AA, Bass HN. Neonatal asymmetric crying facies: A new look at an old problem. Clin Pediatr (Phila) 2005;44:109-19.  Back to cited text no. 3
    
4.
Kumar KJ, Chavan A, Shetty C, Sharma R. Unusual association of congenital hypoplasia of the depressor anguli oris muscle (DAOM). Indian J Pediatr 2013;80:519-20.  Back to cited text no. 4
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