关键词: Congenital hemifacial hypertrophy Hemifacial hypertrophy Hyperpigmentation Hypertrichosis

来  源:   DOI:10.1007/s12070-024-04525-x   PDF(Pubmed)

Abstract:
A case of congenital hemifacial hypertrophy is described. This rare condition is more prevalent in males, and it is characterized by facial asymmetry. Sometimes, Hemifacial hypertrophy can lead to obstruction of the respiratory airway which may prove lethal. Here we made an attempt to present a complicated female case of true congenital hemifacial hypertrophy with its clinical, radiological presentation and surgical treatment. No single theory for hemifacial hypertrophy explains the etiology adequately. A 19-month-old female child was referred to our hospital with difficulty in breathing. She had Hemifacial hypertrophy presents with neck swelling, enlarged ear pinna with hyperpigmentation, and hypertrichosis. Radiological imaging was done, and it was diagnosed as congenital hemifacial hypertrophy. A mass obstructing the oropharynx with tonsillar enlargement was noted. Transoral endoscopic coablator-assisted excision of the oropharyngeal mass with tonsillar excision was done and the airway was secured. The neck fatty mass was excised externally. Follow-up was done for two years. Congenital Hemifacial hypertrophy is a rare congenital condition and has a good prognosis. Generally, Hemifacial hypertrophy presents with neck swelling, enlarged ear pinna with hyperpigmentation, and hypertrichosis. sometimes when presented with respiratory obstruction it can prove fatal which can be managed by securing the airway immediately. Here this case was managed with endoscopic surgical excision of obstruction and no further complications were noted.
UNASSIGNED: The online version contains supplementary material available at 10.1007/s12070-024-04525-x.
摘要:
描述了一例先天性面肌肥大。这种罕见的情况在男性中更为普遍,它的特点是面部不对称。有时候,面部肥大可导致呼吸道阻塞,这可能是致命的。在这里,我们试图提出一个复杂的女性病例的真正的先天性面肌肥大的临床,放射学表现和手术治疗。没有关于半面肌肥大的单一理论可以充分解释病因。一名19个月大的女童因呼吸困难被转诊到我们医院。她有颈部肿胀的半面肥大,耳廓增大伴色素沉着过度,和多毛症。做了放射成像,被诊断为先天性面肌肥大。发现肿块阻塞口咽,扁桃体肿大。经口内镜消融仪辅助切除口咽肿块并切除扁桃体,并固定了气道。从外部切除颈部脂肪块。后续工作进行了两年。先天性面肌肥大是一种罕见的先天性疾病,预后良好。一般来说,侧面部肥大表现为颈部肿胀,耳廓增大伴色素沉着过度,和多毛症。有时当出现呼吸阻塞时,可以证明是致命的,可以通过立即固定气道来管理。在此情况下,通过内窥镜手术切除梗阻进行了处理,没有发现进一步的并发症。
在线版本包含补充材料,可在10.1007/s12070-024-04525-x获得。
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