Tuck J S, Bisset R A, Doig C M
Department of Radiology, Booth Hall Children's Hospital, Manchester.
Clin Radiol. 1991 Oct;44(4):260-4. doi: 10.1016/s0009-9260(05)80192-8.
Achalasia of the cardia is an uncommon condition with an incidence of 0.1 cases per year per 100,000 population under 14 years. A review of experience at Booth Hall Children's Hospital over the past 10 years has revealed six cases of achalasia in children. This includes two cases of the rare syndrome of achalasia, alacrima and ACTH insensitivity (also known as Triple A syndrome) and its variants. There are 23 previously reported cases of Triple A syndrome and a further eight cases of its variants reported in the world literature. One-third of these cases (10 out of 31) have associated neurological abnormalities. The clinical and radiological features of achalasia are reviewed. The importance of barium studies in making the diagnosis is emphasized. However, early cases may show only spasm or incoordination, and manometry is required to confirm the diagnosis in these equivocal cases. In this series only three of the six barium studies were diagnostic and the remainder were diagnosed by manometry; all cases were subsequently confirmed by histology. Delay in diagnosis may result in severe lung damage due to repeated aspiration; this occurred in one of the six cases and was the cause of the child's death.
贲门失弛缓症是一种罕见疾病，在14岁以下每10万人口中的发病率为每年0.1例。对布斯霍尔儿童医院过去10年经验的回顾显示，有6例儿童贲门失弛缓症。其中包括2例罕见的贲门失弛缓症、无泪症和促肾上腺皮质激素不敏感综合征（也称为三A综合征）及其变异型。世界文献中先前报道了23例三A综合征病例，另有8例其变异型病例。这些病例中有三分之一（31例中的10例）伴有神经学异常。本文对贲门失弛缓症的临床和放射学特征进行了综述。强调了钡剂检查在诊断中的重要性。然而，早期病例可能仅表现为痉挛或不协调，在这些不明确的病例中需要通过测压来确诊。在本系列中，6例钡剂检查中只有3例具有诊断价值，其余通过测压确诊；所有病例随后均经组织学证实。诊断延迟可能因反复误吸导致严重肺损伤；6例中有1例发生了这种情况，这是该儿童死亡的原因。
