Rodriguez Kurt, Shargo Ryan, Ekblad Morgan, Sunkersett Gauri, Karjoo Sara, Betensky Marisol, Wilsey Michael J
From the Johns Hopkins All Children's Hospital, Saint Petersburg, FL.
University of South Florida, Reisterstown, MD.
JPGN Rep. 2023 Mar 13;4(2):e291. doi: 10.1097/PG9.0000000000000291. eCollection 2023 May.
We report a 3-year-old patient with suspected oropharyngeal graft-versus-host disease (GVHD) who developed progressive dysphagia to solids and liquids. The patient has a history of Dyskeratosis Congenita-Hoyeraal-Hreidarsson Syndrome with associated bone marrow failure requiring a nonmyeloablative matched sibling hematopoietic stem cell transplant. Esophagram revealed significant narrowing in the cricopharyngeal region. Subsequent esophagoscopy showed a proximal, high-grade pinhole esophageal stricture that was very difficult to visualize and cannulate. High-grade esophageal strictures are uncommon in very young children with GVHD. We believe the patient's underlying Dyskeratosis Congenita-Hoyeraal-Hreidarsson Syndrome in the setting of inflammatory changes seen in GVHD following hematopoietic stem cell transplant set the stage for a high-grade esophageal obstruction. The patient's symptoms improved with serial endoscopic balloon dilation.
我们报告了一名3岁疑似口咽移植物抗宿主病（GVHD）的患者，该患者出现了对固体和液体进行性吞咽困难。该患者有先天性角化不良-霍耶拉尔-赫雷达尔松综合征病史，并伴有骨髓衰竭，需要进行非清髓性匹配同胞造血干细胞移植。食管造影显示环状咽区域明显狭窄。随后的食管镜检查显示食管近端有一个高级别针孔样狭窄，很难看清和插管。高级别食管狭窄在非常年幼的GVHD患儿中并不常见。我们认为，该患者潜在的先天性角化不良-霍耶拉尔-赫雷达尔松综合征，以及造血干细胞移植后GVHD中出现的炎症变化，为高级别食管梗阻奠定了基础。患者的症状通过连续的内镜球囊扩张得到改善。
