Abstract：
Lateral proboscis is a rare congenital condition characterized by a cylindrical protuberance on the nasofrontal region accompanied by abnormal nasal development on the affected side. We aimed to describe the management of the lateral proboscis in staged repair. A 7-year-old girl came with a tube-like projection on the left medial canthal region and nasal agenesis on the ipsilateral side. She was diagnosed with lateral proboscis, left microphthalmia, lower eye lid coloboma, and asymmetry in the orbital region. The patient has undergone 3 major surgeries at our institution. The first surgery involved the deconstruction of the tube to form the left nasal body and nostril. The second operation involved trimming of the new nose form and the excision of the bony protrusion directly beneath the base of the pedicle through bifrontal craniotomy. The remaining bone defect was closed using a pericranial flap. The orbital floor was reconstructed using titanium mesh. The third operation involved nasal reconstruction using a costal cartilage graft to create a dorsal nasal and alar framework. The patient healed with no complications, had become less reserved and her grades improved significantly after the operation. Further appointments are being scheduled to evaluate growth distortion and the resulting facial asymmetry. Surgical correction will be planned thereafter to further reconstruct the facial features. Evaluation of patient is necessary to explore possible clinical outcomes and corresponding treatment options. Multidisciplinary management is highly recommended, involving plastic surgeons, neurosurgeons, ophthalmologists, pediatricians, and pediatric psychiatrists in order to improve patient\'s quality of life.
摘要：
外侧长鼻是一种罕见的先天性疾病，其特征是鼻额叶区域有圆柱形突起，并伴有患侧的异常鼻发育。我们旨在描述分阶段修复中外侧长鼻的管理。一个7岁的女孩在左内侧can区出现管状突起，在同侧出现鼻腔发育不全。她被诊断为侧鼻,左小眼症,下眼睑缺损，和轨道区域的不对称性。患者在我们机构接受了3次重大手术。第一次手术涉及解构管，形成左鼻体和鼻孔。第二次手术包括修剪新的鼻子形式，并通过双额开颅术切除椎弓根底部正下方的骨突起。使用颅周皮瓣闭合剩余的骨缺损。使用钛网重建轨道地板。第三次手术涉及使用肋软骨移植物进行鼻重建，以创建背侧鼻和鼻翼框架。病人痊愈了，没有并发症，变得不那么保守，手术后她的成绩显著提高。正在安排进一步的约会，以评估生长失真和由此产生的面部不对称性。此后将计划手术矫正以进一步重建面部特征。有必要对患者进行评估，以探索可能的临床结局和相应的治疗方案。强烈建议多学科管理，涉及整形外科医生，神经外科医生,眼科医生,儿科医生,和儿科精神科医生，以提高患者的生活质量。
