Xia Chunxia, Zhu Qiang, Yue Changli, Hu Minxia, Li Pingdong, Li Zheng
Department of Diagnostic Ultrasound, Beijing Tongren Hospital, Capital Medical University Department of Pathology, Beijing Tongren Hospital, Capital Medical University Department of Otorhinolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University Department of Diagnostic Ultrasound, Beijing Aerospace General Hospital, Beijing, China.
Medicine (Baltimore). 2017 Nov;96(44):e8500. doi: 10.1097/MD.0000000000008500.
Infantile desmoid fibromatosis of the postcricoid area is a rare disease and is characterized by a proliferation of fibrous tissue with non-metastasis, local infiltration, and a high rate of recurrence after surgical resection. Currently, ultrasound is scarcely used in the hypopharynx and larynx area.
A 4-year-old boy presented with hoarseness, deep voice and snoring for 2∼4 years without any surgical history. On sonography, the lesion was found in the postcricoid area, and the left larynx showed impaired mobility in real time observation. Complete excision with a negative margin in this pivotal anatomic area is impossible, and necessitates a long-time surveillance.
Infantile desmoid fibromatosis of the postcricoid area was diagnosed according to surgery and histopathology.
Local excision was carried out to relieve the upper airway narrowing.
Relieved hoarseness and snoring were reported on the latest follow-up. A residual lesion was seen in the surgical bed and maintained a stable extent on ultrasound and MR imaging after a year.
Considering the non-radiation merit and diagnostic ability, ultrasonography is advocated as a valuable supplementary imaging method to CT, MR and laryngoscopy in the juvenile larynx and hypopharynx.
环状软骨后区婴儿型硬纤维瘤病是一种罕见疾病，其特征为纤维组织增生，无转移、局部浸润，手术切除后复发率高。目前，超声在下咽和喉部区域很少使用。
一名4岁男孩出现声音嘶哑、嗓音低沉和打鼾2至4年，无任何手术史。超声检查发现病变位于环状软骨后区，实时观察显示左侧喉部活动受限。在这个关键解剖区域无法进行切缘阴性的完整切除，需要长期监测。
根据手术和组织病理学诊断为环状软骨后区婴儿型硬纤维瘤病。
进行局部切除以缓解上气道狭窄。
最新随访报告声音嘶哑和打鼾症状缓解。手术床处可见残留病变，一年后超声和磁共振成像显示其范围稳定。
考虑到超声无辐射的优点和诊断能力，提倡将其作为一种有价值的补充成像方法，用于青少年喉部和下咽的CT、磁共振成像及喉镜检查。
