Imai Yoshimichi, Kochi Shoko, Nakajo Tetsu, Yamada Atsushi
Department of Plastic and Reconstructive Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
Clinics for Maxillo-Oral Disorders, Dental Center, Tohoku University Hospital, Sendai, Japan.
Plast Reconstr Surg Glob Open. 2019 May 3;7(5):e2211. doi: 10.1097/GOX.0000000000002211. eCollection 2019 May.
Hypoglossia-hypodactyly, or aglossia-adactylia syndrome with or without limb anomalies, is an extremely rare congenital condition. It is characterized by a narrow, V-shaped mandibular dental arch with micrognathia, and is typically challenging to treat. We have previously reported 3 patients with hypoglossia-hypodactyly syndrome without limb anomalies who were treated with transverse distraction osteogenesis at the mandibular symphysis during childhood. In this report, we present the long-term prognoses of these 3 cases, until 18 years of age. Of the 3 total cases, sufficient and stable results in occlusion, speech, mastication, and facial appearance were obtained in 2 cases with subsequent orthodontic treatments. Similar results were not observed in the remaining case when continuous orthodontic treatments were not performed. Based on these results, we propose that the transverse distraction osteogenesis procedure at the mandibular symphysis during childhood could be a reliable method to correct the V-shaped mandibular arch of hypoglossia-hypodactyly syndrome, when subsequent orthodontic treatments are provided during the patient's growing period.
舌下腺发育不全-指趾减少症，即伴有或不伴有肢体异常的无舌-无指（趾）综合征，是一种极其罕见的先天性疾病。其特征为下颌牙弓狭窄呈V形且伴有小颌畸形，通常治疗具有挑战性。我们之前报道过3例无肢体异常的舌下腺发育不全-指趾减少症综合征患者，他们在儿童期接受了下颌联合处的横向牵张成骨治疗。在本报告中，我们展示了这3例患者至18岁的长期预后情况。在这3例患者中，2例通过后续正畸治疗在咬合、言语、咀嚼和面部外观方面获得了充分且稳定的效果。在未进行连续正畸治疗的其余1例患者中未观察到类似结果。基于这些结果，我们提出，当在患者生长发育期进行后续正畸治疗时，儿童期下颌联合处的横向牵张成骨手术可能是纠正舌下腺发育不全-指趾减少症综合征V形下颌弓的可靠方法。
