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Surgical Approach in a Patient With Agnathia-Otocephaly Complex: Three-Stage Mandibular Distraction Protocol.

Abstract

Agnathia-otocephaly complex (AOC) is a rare malformation complex of the first pharyngeal arch that is characterized by agnathia/dysgnathia, microstomia, aglossia/hypoglossia and variable displacement of the ears. Only 11 post-infancy patients with severe AOC have been described in the literature, and the incidence of this malformation complex is estimated to be 1 per 70,000 births. In this brief clinical study, the authors describe the case of an 18-year-old female diagnosed with AOC who underwent a 3-step mandibular distraction protocol with an external distraction device. The surgical protocol the authors used was unique in that we first placed a tissue expander in the submental area to enlarge the skin envelope in an effort to mitigate skeletal relapse from soft tissue forces. Furthermore, the way in which the authors slowed the activation of the distraction device to allow for soft tissue healing behind the pins was a novel component of the patient's treatment. The 3-step mandibular distraction protocol the authors present in this study increased the length of the mandible by 20 mm, and nearly doubled the size of the patient's mandible from an initial volume of 3.62 cm to a post-operative volume of 6.89 cm. Future surgeries will aim to improve the function of our patient's expanded mandible. Most important of all, the surgical treatment authors are presenting led to a significant improvement in our patient's physical appearance and 3d quality of life.

Authors+Show Affiliations

College of Medicine.Department of Otolaryngology, University of Arkansas for Medical Sciences, Little Rock, AR.Department of Plastic Surgery, Vanderbilt University Medical Center, Nashville, TN.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31634311

Citation

Gonzalez, Santiago R., et al. "Surgical Approach in a Patient With Agnathia-Otocephaly Complex: Three-Stage Mandibular Distraction Protocol." The Journal of Craniofacial Surgery, 2019.
Gonzalez SR, Jones JK, Golinko MS. Surgical Approach in a Patient With Agnathia-Otocephaly Complex: Three-Stage Mandibular Distraction Protocol. J Craniofac Surg. 2019.
Gonzalez, S. R., Jones, J. K., & Golinko, M. S. (2019). Surgical Approach in a Patient With Agnathia-Otocephaly Complex: Three-Stage Mandibular Distraction Protocol. The Journal of Craniofacial Surgery, doi:10.1097/SCS.0000000000005945.
Gonzalez SR, Jones JK, Golinko MS. Surgical Approach in a Patient With Agnathia-Otocephaly Complex: Three-Stage Mandibular Distraction Protocol. J Craniofac Surg. 2019 Oct 18; PubMed PMID: 31634311.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Surgical Approach in a Patient With Agnathia-Otocephaly Complex: Three-Stage Mandibular Distraction Protocol. AU - Gonzalez,Santiago R, AU - Jones,John K, AU - Golinko,Michael S, Y1 - 2019/10/18/ PY - 2019/10/22/entrez PY - 2019/10/22/pubmed PY - 2019/10/22/medline JF - The Journal of craniofacial surgery JO - J Craniofac Surg N2 - Agnathia-otocephaly complex (AOC) is a rare malformation complex of the first pharyngeal arch that is characterized by agnathia/dysgnathia, microstomia, aglossia/hypoglossia and variable displacement of the ears. Only 11 post-infancy patients with severe AOC have been described in the literature, and the incidence of this malformation complex is estimated to be 1 per 70,000 births. In this brief clinical study, the authors describe the case of an 18-year-old female diagnosed with AOC who underwent a 3-step mandibular distraction protocol with an external distraction device. The surgical protocol the authors used was unique in that we first placed a tissue expander in the submental area to enlarge the skin envelope in an effort to mitigate skeletal relapse from soft tissue forces. Furthermore, the way in which the authors slowed the activation of the distraction device to allow for soft tissue healing behind the pins was a novel component of the patient's treatment. The 3-step mandibular distraction protocol the authors present in this study increased the length of the mandible by 20 mm, and nearly doubled the size of the patient's mandible from an initial volume of 3.62 cm to a post-operative volume of 6.89 cm. Future surgeries will aim to improve the function of our patient's expanded mandible. Most important of all, the surgical treatment authors are presenting led to a significant improvement in our patient's physical appearance and 3d quality of life. SN - 1536-3732 UR - https://www.unboundmedicine.com/medline/citation/31634311/Surgical_Approach_in_a_Patient_With_Agnathia-Otocephaly_Complex:_Three-Stage_Mandibular_Distraction_Protocol L2 - http://dx.doi.org/10.1097/SCS.0000000000005945 DB - PRIME DP - Unbound Medicine ER -