Hemifacial microsomia: a literature review


  • Sebastián| Véliz-Méndez Universidad de Chile
  • Pamela Agurto-V. Hospital Luis Calvo Mackenna
  • Noemí| Leiva-V. Universidad de Chile




Facial asymmetry, Craniofacial anomalies, Hemifacial microsomia


Hemifacial microsomia is the second congenital malformation in prevalence, after cleft lip and palate, and is described as a congenital alteration of the first and second branchial arches. As a condition of wide spectrum, its characteristics are expressed in many different ways and therefore treatments are usually individualized. This topic review discusses its etiology, classification, characteristics, and treatment with mandibular surgery.

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Author Biographies

Sebastián| Véliz-Méndez, Universidad de Chile

DDS, Intern in Craniofacial Malformation, Universidad de Chile School of Dentistry

Pamela Agurto-V., Hospital Luis Calvo Mackenna

Orthodontist. Hospital Luis Calvo Mackenna, Chile

Noemí| Leiva-V., Universidad de Chile

Assistant Professor. Universidad de Chile School of Dentistry. Orthodontist. Hospital Luis Calvo Mackenna, Chile


Cohen MM Jr, Rollnick BR, Kaye CI. Oculoauriculovertebral spectrum: an updated critique. Cleft Palate J 1989; 26(4): 276-286.

Mielnik-Blaszczak M. Hemifacial microsomia. Review of the literature. Dent Med Probl 2011; 40(1): 80-85.

Heike CL, Luquetti DV, Hing AV. Craniofacial microsomia overview. In: Pagon RA, Adam MP, Bird TD editors. GeneReviews® [Internet]. Seattle: University of Washington, Seattle; 1993-2013. Disponible en: http://www.ncbi.nlm.nih.gov/books/NBK5199/.

Hartsfield JK. Review of the etiologic heterogeneity of the oculo-auriculo-vertebral spectrum (Hemifacial Microsomia). Orthod Craniofac Res 2007; 10(3): 121-128.

Poswillo D. The pathogenesis of the first and second branchial arch syndrome. Oral Surg Oral Med Oral Pathol 1973; 35(3): 302-328.

Johnston MC, Bronsky PT. Animal models for human craniofacial malformations. J Craniofac Genet Dev Biol 1991; 11(4): 277-291.

Maruko E, Hayes C, Evans CA, Padwa B, Mulliken JB. Hypodontia in hemifacial microsomia. Cleft Palate Craniofac J 2001; 38(1): 15-19.

Sulik KK, Cook CS, Webster WS. Teratogens and craniofacial malformations: relationships to cell death. Development 1988;103 (Suppl): 213-231.

Wiszniak S, Mackenzie FE, Anderson P, Kabbara S, Ruhrberg C, Schwarz Q. Neural crest cell-derived VEGF promotes embryonic jaw extension. Proc Natl Acad Sci U S A. 2015; 112(19): 6086-6091.

Werler MM, Sheehan JE, Hayes C, Mitchell AA, Mulliken JB. Vasoactive exposures, vascular events, and hemifacial microsomia. Birth Defects Res A Clin Mol Teratol 2004; 70(6): 389-395.

Werler MM, Sheehan JE, Hayes C, Padwa BL, Mitchell AA, Mulliken JB. Demographic and reproductive factors associated with hemifacial microsomia. Cleft Palate Craniofac J 2004; 41(5): 494-450.

Wieczorek D, Ludwig M, Boehringer S, Jongbloet PH, Gillessen-Kaesbach G, Horsthemke B. Reproduction abnormalities and twin pregnancies in parents of sporadic patients with oculo-auriculo-vertebral spectrum/Goldenhar syndrome. Hum Genet 2007; 121(3-4): 369-376.

Ewart-Toland A, Yankowitz J, Winder A, Imagire R, Cox VA, Aylsworth AS et al. Oculoauriculovertebral abnormalities in children of diabetic mothers. Am J Med Genet 2000; 90(4): 303-309.

Wang R, Martinez-Frias ML, Graham JM Jr. Infants of diabetic mothers are at increased risk for the oculo-auriculo-vertebral sequence: A case-based and case-control approach. J Pediatr 2002; 141(5): 611-617.

Werler MM, Starr JR, Cloonan YK, Speltz ML. Hemifacial microsomia: from gestation to childhood. J Craniofac Surg 2009; 20 (Suppl 1): 664-669.

Tasse C, Majewski F, Bohringer S, Fischer S, Ludecke HJ, Gillessen-Kaesbach G et al. A family with autosomal dominant oculo-auriculo-vertebral spectrum. Clin Dysmorphol 2007; 16(1): 1-7.

Kelberman D, Tyson J, Chandler DC, McInerney AM, Slee J, Albert D et al. Hemifacial microsomia: progress in understanding the genetic basis of a complex malformation syndrome. Hum Genet 2001; 109(6): 638-645.

Brady AF, Winter RM, Wilson LC, Tatnall FM, Sheridan RJ, Garrett C. Hemifacial microsomia, external auditory canal atresia, deafness and Mullerian anomalies associated with acro-osteolysis: a new autosomal recessive syndrome? Clin Dysmorphol 2002; 11(3): 155-161.

Choong YF, Watts P, Little E, Beck L. Goldenhar and cri-du-chat syndromes: a contiguous gene deletion syndrome? J AAPOS 2003; 7(3): 226-227.

Derbent M, Yilmaz Z, Baltaci V, Saygili A, Varan B, Tokel K. Chromosome 22q11.2 deletion and phenotypic features in 30 patients with conotruncal heart defects. Am J Med Genet A 2003; 116A(2): 129-135.

Pruzansky S. Not all dwarfed mandibles are alike. Birth Defects 1969; 1: 120-9.

Kaban LB, Moses MH, Mulliken JB. Surgical correction of hemifacial microsomia in the growing child. Plast Reconstr Surg 1988; 82(1): 9-19.

Vento AR, LaBrie RA, Mulliken JB. The O.M.E.N.S. classification of hemifacial microsomia. Cleft Palate Craniofac J 1991; 28(1): 68-76.

Horgan JE, Padwa BL, LaBrie RA, Mulliken JB. OMENS-Plus: analysis of craniofacial and extracraniofacial anomalies in hemifacial microsomia. Cleft Palate Craniofac J 1995; 32(5): 405-412.

Gougoutas AJ, Singh DJ, Low DW, Bartlett SP. Hemifacial microsomia: clinical features and pictographic representations of the OMENS classification system. Plast Reconstr Surg 2007; 120(7): 112e-120e.

Birgfeld CB, Luquetti DV, Gougoutas AJ, Bartlett SP, Low DW, Sie KC et al. A phenotypic assessment tool for craniofacial microsomia. Plast Reconstr Surg 2011; 127(1): 313-320.

Huisinga-Fischer CE, Zonneveld FW, Vaandrager JM, Prahl-Andersen B. CT-based size and shape determination of the craniofacial skeleton: a new scoring system to assess bony deformities in hemifacial microsomia. J Craniofac Surg 2001; 12(1): 87-94.

Sze RW, Paladin AM, Lee S, Cunningham ML. Hemifacial microsomia in pediatric patients: asymmetric abnormal development of the first and second branchial arches. AJR Am J Roentgenol 2002; 178(6): 1523-1530.

Ongkosuwito EM, van Neck JW, Wattel E, van Adrichem LN, Kuijpers-Jagtman AM. Craniofacial morphology in unilateral hemifacial microsomia. Br J Oral Maxillofac Surg 2012; 51(8): 902-907

Ongkosuwito EM, van Vooren J, van Neck JW, Wattel E, Wolvius EB, van Adrichem LN et al. Changes of mandibular ramal height, during growth in unilateral hemifacial microsomia patients and unaffected controls. J Craniomaxillofac Surg 2013; 41(2): 92-97.

Kitai N, Murakami S, Takashima M, Furukawa S, Kreiborg S, Takada K. Evaluation of temporomandibular joint in patients with hemifacial microsomia. Cleft Palate Craniofac J 2004; 41(2): 157-162.

Gorlin RJ, Cohen MM Jr, Hennekam RCM. Syndromes of the head and neck. 4 ed. New York: Oxford University Press; 2001.

Farias M, Vargervik K. Tooth size and morphology in hemifacial microsomia. Int J Pediatr Dent 1998; 8(3): 197-201.

Ongkosuwito EM, de Gijt P, Wattel E, Carels CE, Kuijpers-Jagtman AM. Dental development in hemifacial microsomia. J Dent Res 2010; 89(12): 1368-1372.

Seow WK, Urban S, Vafaie N, Shusterman S. Morphometric analysis of the primary and permanent dentitions in hemifacial microsomia: a controlled study. J Dent Res 1998; 77(1): 27-38.

Fan WS, Mulliken JB, Padwa BL. An association between hemifacial microsomia and facial clefting. J Oral Maxillofac Surg 2005; 63(3): 330-334.

Heude E, Rivals I, Couly G, Levi G. Masticatory muscle defects in hemifacial microsomia: a new embryological concept. Am J Med Genet A 2011; 155A(8): 1991-1995.

Funayama E, Igawa HH, Nishizawa N, Oyama A, Yamamoto Y. Velopharyngeal insufficiency in hemifacial microsomia: analysis of correlated factors. Otolaryngol Head Neck Surg 2007; 136(1): 33-37.

Dufton LM, Speltz ML, Kelly JP, Leroux B, Collett BR, Werler MM. Psychosocial outcomes in children with hemifacial microsomia. J Pediatr Psychol 2011; 36(7): 794-805.

Cloonan YK, Kifle Y, Davis S, Speltz ML, Werler MM, Starr JR. Sleep outcomes in children with hemifacial microsomia and controls: a follow-up study. Pediatrics 2009; 124(2): e313-321.

Takahashi-Ichikawa N, Susami T, Nagahama K, Ohkubo K, Okayasu M, Uchino N et al. Evaluation of mandibular hypoplasia in patients with hemifacial microsomia: a comparison between panoramic radiography and three-dimensional computed tomography. Cleft Palate Craniofac J 2013; 50(4): 381-387.

Jayaratne YS, Lo J, Zwahlen RA, Cheung LK. Three-dimensional photogrammetry for surgical planning of tissue expansion in hemifacial microsomia. Head Neck 2010; 32(12): 1728-1735.

Birgfeld CB, Saltzman BS, Luquetti DV, Latham K, Starr JR, Heike CL. Comparison of two-dimensional and three-dimensional images for phenotypic assessment of craniofacial microsomia. Cleft Palate Craniofac J 2013; 50(3): 305-314.

Meazzini MC, Brusati R, Diner P, Gianni E, Lalatta F, Magri AS et al. The importance of a differential diagnosis between true hemifacial microsomia and pseudo-hemifacial microsomia in the post-surgical long-term prognosis. J Craniomaxillofac Surg 2011; 39(1): 10-16.

Meazzini MC, Brusati R, Caprioglio A, Diner P, Garattini G, Gianni E et al. True hemifacial microsomia and hemimandibular hypoplasia with condylar-coronoid collapse: diagnostic and prognostic differences. Am J Orthod Dentofacial Orthop 2011; 139(5): e435-447.

Association AC-P-C. Parameters for the Evaluation and treatment of patients with cleft lip/palate or other craniofacial anomalies. Revised Edition 2009. Original Publication Cleft Palate-Craniofacial Journal 1993; 30 (Suppl 1).

Ohtani J, Hoffman WY, Vargervik K, Oberoi S. Team management and treatment outcomes for patients with hemifacial microsomia. Am J Orthod Dentofacial Orthop 2012; 141 (Suppl 4): S74-81.

Vargervik K, Oberoi S, Hoffman WY. Team care for the patient with cleft: UCSF protocols and outcomes. J Craniofac Surg 2009; 20 (Suppl 2): 1668-1671.

Liu SYC, Good P, Lee JS. Chapter 94 - Surgical care of the hemifacial microsomia patient. Current therapy in oral and maxillofacial surgery. Saint Louis: W.B. Saunders; 2012, p. 828-834.

Reina-Romo E, Sampietro-Fuentes A, Gomez-Benito MJ, Dominguez J, Doblare M, Garcia-Aznar JM. Biomechanical response of a mandible in a patient affected with hemifacial microsomia before and after distraction osteogenesis. Med Eng Phys 2010; 32(8): 860-866.

Lo J, Cheung LK. Distraction osteogenesis for the craniomaxillofacial region. Part 2: a compendium of devices for the mandible. Asian J Oral Maxillofac Surg. 2007; 19(1): 6-18.

Burstein FD. Resorbable distraction of the mandible: technical evolution and clinical experience. J Craniof Surg 2008; 19(3): 637-643.

Sakamoto Y, Ogata H, Nakajima H, Kishi K, Sakamoto T, Ishii T. Role of solid model simulation surgery for hemifacial microsomia. Cleft Palate Craniofac J 2013; 50(5): 623-626.

Ow A, Cheung LK. Skeletal stability and complications of bilateral sagittal split osteotomies and mandibular distraction osteogenesis: an evidence-based review. J Oral Maxillofac Surg 2009; 67(11): 2344-2353.

Birgfeld CB, Heike C. Craniofacial microsomia. Semin Plast Surg 2012; 26(2): 91-104.

Lara-García TU, Ulfe I, Rodriguez JC, Dogliotti PLV. Protocolo de seguimiento de pacientes con microsomía hemifacial. Prensa Med Argent 2002; 89(5): 414-423.

Van Strijen PJ, Breuning KH, Becking AG, Perdijk FBT, Tuinzing DB. Complications in bilateral mandibular distraction osteogenesis using internal devices. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003; 96(4): 392-397.

Meazzini MC, Mazzoleni F, Bozzetti A, Brusati R. Comparison of mandibular vertical growth in hemifacial microsomia patients treated with early distraction or not treated: follow up till the completion of growth. J Craniomaxillofac Surg 2012; 40(2): 105-111.

Gursoy S, Hukki J, Hurmerinta K. Five year follow-up of mandibular distraction osteogenesis on the dentofacial structures of syndromic children. Orthod Craniofac Res 2008; 11(1): 57-64.



How to Cite

Véliz-Méndez, S., Agurto-V., P., & Leiva-V., N. (2016). Hemifacial microsomia: a literature review. Revista Facultad De Odontología Universidad De Antioquia, 27(2), 404–424. https://doi.org/10.17533/udea.rfo.v27n2a9

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