Morel Baptiste, Sileo Chiara, Epaud Ralph, Blondiaux Eléonore, Nathan Nadia, Corvol Harriet, Tounian Patrick, Le Pointe Hubert Ducou
*Université Pierre et Marie Curie (Paris 6), Sorbonne Universités †Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital A. Trousseau, Service de Radiologie Pédiatrique, Paris ‡Inserm U955, Equipe 5, Faculté de Médecine, Créteil §Centre Hospitalier Intercommunal de Créteil, Créteil, Centre de Référence des Maladies Respiratoires Rares ||Assistance Publique Hôpitaux de Paris (AP-HP), Service de Pneumologie Pédiatrique, Hôpital Armand Trousseau, Centre National de Référence des Maladies Respiratoires Rares ¶Institut National de la Santé et la Recherche Médicale (INSERM), UMR_S 933 #Institut National de la Santé et la Recherche Médicale (INSERM), UMR_S 938, CDR Saint-Antoine, Université Pierre et Marie Curie (Paris 6), Sorbonne Universités **AP-HP, Hôpital Armand Trousseau, Service de Nutrition et Gastroentérologie Pédiatriques, Paris, France.
J Pediatr Gastroenterol Nutr. 2016 Aug;63(2):195-9. doi: 10.1097/MPG.0000000000001175.
Sarcoidosis is a multisystem, granulomatous inflammatory disease affecting both pediatric and adult patients. So far in children, very few radiological descriptions of abdominal sarcoidosis manifestations have been reported. The present study describes the frequency and the appearance of abdominal radiologic manifestations in pediatric patients with histologically proven sarcoidosis.
We reviewed retrospectively all of the radiological examinations of 22 patients ages 1 to 15 years at diagnosis with proven sarcoidosis evaluated in a university pediatric hospital between 1994 and 2014. The locations of biopsies and the angiotensin-converting enzyme level were reported. The size, shape, and parenchymal homogeneity of the liver and spleen, the presence of abdominal lymph nodes, and abnormalities of the gastrointestinal tract were tabulated.
The study included 22 children (mean age: 9.9 ± 2.8 years). The liver was the most frequent location of biopsy (12/22), even without radiological involvement. Abdominal manifestations were present in 11 of 22 children with sarcoidosis. Hepatomegaly was the most frequent abnormality, reported in 8 of 11 cases either homogeneous (n = 7) or nodular (n = 1). Homogeneous lymph node enlargement was noted in 6 of 11 cases and splenomegaly in 4 of 11 cases. No calcification was observed. All patients with initial abdominal sarcoidosis had simultaneous thoracic involvement.
Abdominal manifestations in children sarcoidosis are frequent but often nonspecific. Nodular hepatosplenomegaly is rare. All of our patients with abdominal abnormalities had a more specific associated thoracic involvement. Awareness of this association could assist the clinicians in assessing the initial diagnosis of abdominal sarcoidosis in children.
结节病是一种多系统的肉芽肿性炎症性疾病，可累及儿童和成人患者。到目前为止，关于儿童腹部结节病表现的放射学描述报道极少。本研究描述了经组织学证实的结节病患儿腹部放射学表现的频率及特征。
我们回顾性分析了1994年至2014年在一家大学儿科医院确诊为结节病的22例1至15岁患者的所有放射学检查结果。报告了活检部位及血管紧张素转换酶水平。记录了肝脏和脾脏的大小、形态及实质均匀性、腹部淋巴结情况以及胃肠道异常。
该研究纳入22名儿童（平均年龄：9.9±2.8岁）。肝脏是最常进行活检的部位（12/22），即便在无放射学受累表现时也是如此。22例结节病患儿中有11例出现腹部表现。肝肿大是最常见的异常，11例中有8例出现，其中7例为均匀性肿大，1例为结节状肿大。11例中有6例出现均匀性淋巴结肿大，11例中有4例出现脾肿大。未观察到钙化。所有最初有腹部结节病表现的患者同时伴有胸部受累。
儿童结节病的腹部表现较为常见，但通常不具有特异性。结节状肝脾肿大罕见。我们所有有腹部异常表现的患者均伴有更具特异性的相关胸部受累。认识到这种关联有助于临床医生评估儿童腹部结节病的初步诊断。
