Asymmetric crying facies

Shapira M, Borochowitz ZU. Asymmetric crying facies. NeoReviews. 2009 Oct; 10(10): e502-e509.

ACF is a minor facial anomaly that consists of asymmetry during crying, has multifactorial inheritance, and is found in 1 per 160 live births. ACF can be differentiated from true facial paralysis solely on a clinical basis. The first of two primary causes of ACF is nerve compression, which may be observed on physical examination, noting mandibular asymmetry and maxillary-mandibular asynclitism. This form of ACF generally has a good prognosis. The second cause of ACF is DAOM hypoplasia or agenesis, which has the possibility of associated major and minor anomalies and deformations. The most frequently associated anomalies are cardiovascular and cervicofacial. Ultrasonographic and electromyographic testing of the facial muscles can aid in elucidating this cause. The management and evaluation of neonates and infants who have ACF depends on the cause. If the cause is traumatic, no further evaluation is needed. In the case of faulty muscle development, further evaluation depends on the presence of abnormalities on cardiac examination or dysmorphology.

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Sapin SO, Miller AA, Bass HN. Neonatal asymmetric crying facies: a new look at an old problem. Clin Pediatr (Phila). 2005 Mar;44(2):109-19.

Neonatal asymmetric crying facies, described 75 years ago, is a clinical phenotype resembling unilateral partial peripheral facial nerve paralysis, with an incidence of approximately 1 per 160 live births. The cause is either facial nerve compression or faulty facial muscle and/or nerve development. Spontaneous resolution is expected with the former, but not necessarily with the latter etiology. Approximately 10% of the developmental cases have associated major malformations. Mandibular asymmetry and maxillary-mandibular asynclitism (non-parallelism of the gums) are frequently overlooked visual clues to nerve compression. Ultrasound imaging of facial muscles and electrodiagnostic testing may be useful for differential diagnosis and management.

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Caksen H, et al. A review of 35 cases of asymmetric crying facies. Genet Couns. 2004; 15(2):159-65.

A review of 35 cases of asymmetric crying facies: Congenital asymmetric crying facies (ACF) is caused by congenital hypoplasia or agenesis of the depressor anguli oris muscle (DAOM) on one side of the mouth. It is well known that this anomaly is frequently associated with cardiovascular, head and neck, musculoskeletal, respiratory, gastrointestinal, central nervous system, and genitourinary anomalies. In this article we report 35 ACF patients (28 children and 7 adults) and found additional abnormalities in 16 of them (i.e. 45%). The abnormalities were cerebral and cerebellar atrophy, mega-cisterna magna, mental motor retardation, convulsions, corpus callosum dysgenesis, cranial bone defect, dermoid cyst, spina bifida occulta, hypertelorism, micrognatia, retrognatia, hemangioma on the lower lip, short frenulum, cleft palate, low-set ears, preauricular tag, mild facial hypoplasia, sternal cleft, congenital heart defect, renal hypoplasia, vesicoureteral reflux, hypertrophic osteoarthropathy, congenital joint contractures, congenital hip dislocation, polydactyly, and umbilical and inguinal hernia. Besides these, one infant was born to a diabetic mother, and had atrial septal defect and the four other children had 4p deletion, Klinefelter syndrome, isolated CD4 deficiency and Treacher-Collins like facial appearance, respectively Although many of these abnormalities were reported in association with ACF, cerebellar atrophy, sternal cleft, cranial bone defect, infant of diabetic mother, 4p deletion, Klinefelter syndrome, isolated CD4 deficiency and Treacher-Collins like facial appearance were not previously published.

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Lahat E, Heyman E, Barkay A, Goldberg M. Asymmetric crying facies and associated congenital anomalies: prospective study and review of the literature. J Child Neurol. 2000 Dec;15(12):808-10.

Congenital asymmetric crying facies, a minor congenital anomaly due to absence or hypoplasia of the depressor anguli oris muscle on one side of the mouth, is associated at times with major congenital anomalies, most commonly in the cardiovascular system. In a prospective study of 5532 infants born at the Assaf Harofeh Medical Center, Israel, during 12 months (January to December 1998), 17 infants (an incidence of 0.31%) had asymmetric crying facies. One of the affected infants had ventricular septal defect and another infant had VATER (vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia) syndrome. No noxious obstetric perinatal factors could be identified. Family history was unremarkable in all cases. Diagnostic work-up performed in all of the affected infants failed to reveal an additional congenital malformation. Asymmetric crying facies is a minor isolated finding in most of the cases; however, a thorough search for other congenital malformations, especially of the cardiovascular system, should be performed.

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More PubMed results on asymmetric crying facies.

Created 10/28/14; reviewed 08/04/15, 03/18/16 – articles added to PubMed collection.

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