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西罗莫司成功治疗伴有喘鸣的咽后淋巴管瘤:一例报告

Successful Management of Retropharyngeal Lymphangioma with Stridor Using Sirolimus: A Case Report.

作者信息

Razali Siti Sarah Che Mohd, Mohd Nik Khairani Nik, Ibrahim Fairuz Mohd, Hamid Suzina Sheikh Ab

机构信息

Department of Otorhinolaryngology- Head and Neck Surgery, Hospital Sultanah Nur Zahirah, Kuala Terengganu, Terengganu, 20400 Malaysia.

Department of Otorhinolaryngology- Head & Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia, Health Campus, 16150 Kota Bharu, Kelantan, Malaysia.

出版信息

Indian J Otolaryngol Head Neck Surg. 2024 Feb;76(1):1298-1301. doi: 10.1007/s12070-023-04273-4. Epub 2023 Oct 24.

Abstract

Lymphatic malformations (LMs) are one of the congenital malformations of the lymphatic system in the body. The patient usually presents with head and neck swelling, airway compression, and/or airway obstruction. The diagnosis of retropharyngeal LMs can be challenging due to their rare occurrence. We report a case of a five-month-old boy diagnosed with retropharyngeal LMs. He presented with a three-day history of fever, cough, and stridor and was initially treated for acute bronchiolitis. A lateral neck radiograph revealed prevertebral widening, suggesting retropharyngeal collection. The patient's condition worsened, requiring intubation in the operating room and proceeding with aspirations and drainage. However, the symptoms recurred after a few days, necessitating re-intubation, repeated aspirations and drainage procedures. The patient was intubated, and the neck's magnetic resonance imaging (MRI) confirmed retropharyngeal LMs. An elective tracheostomy was performed and was treated with sirolimus. The patient had a successful tracheostomy decannulation and showed no recurrence during follow-up.

摘要

淋巴管畸形(LMs)是人体淋巴系统的先天性畸形之一。患者通常表现为头颈部肿胀、气道受压和/或气道阻塞。由于咽后淋巴管畸形很少见,其诊断可能具有挑战性。我们报告一例5个月大男孩被诊断为咽后淋巴管畸形的病例。他有3天的发热、咳嗽和喘鸣病史,最初被诊断为急性细支气管炎并接受治疗。颈部侧位X线片显示椎体前方增宽,提示咽后积液。患者病情恶化,需要在手术室进行插管,并进行抽吸和引流。然而,几天后症状复发,需要再次插管、重复抽吸和引流操作。患者接受了插管,颈部磁共振成像(MRI)证实为咽后淋巴管畸形。随后进行了择期气管切开术,并使用西罗莫司进行治疗。患者气管切开术拔管成功,随访期间未复发。

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