Akçakuş Mustafa, Güneş Tamer, Kurtoğlu Selim, Cetin Neşide, Ozkul Yusuf, Narin Nazmi, Atabek Mehmet Emre, Uğraş Remzi
Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
Turk J Pediatr. 2004 Apr-Jun;46(2):191-3.
Asymmetric crying facies is caused by congenital hypoplasia or agenesis of the depressor anguli oris muscle. Associations of this facial defect with major congenital anomalies have been reported, most commonly in the cardiovascular system and less frequently involving the genitourinary, musculoskeletal, cervicofacial, respiratory, and rarely, the endocrine system. CATCH 22 is a medical acronym for cardiac defects, abnormal facies, thymic hypoplasia, cleft palate and hypocalcemia and a variable deletion on chromosome 22q11. The deletion within chromosme region of 22q11 may occur in patients with dysmorphologic and cardiological syndromes: DiGeorge syndrome, velo-cardiofacial syndrome and conotruncal anomaly face syndrome. We report a newborn infant who had asymmetric crying facies associated with congenital hypoparathyroidism, severe neonatal hypocalcemia and tetralogy of Fallot. Genetic confirmation of chromosome 22q11 deletion was made.
不对称哭脸综合征是由降口角肌先天性发育不全或缺失引起的。这种面部缺陷与主要先天性异常的关联已有报道,最常见于心血管系统,较少涉及泌尿生殖系统、肌肉骨骼系统、颈面部、呼吸系统,很少涉及内分泌系统。CATCH 22是一个医学首字母缩略词,代表心脏缺陷、面容异常、胸腺发育不全、腭裂和低钙血症以及22q11染色体上的可变缺失。22q11染色体区域内的缺失可能发生在患有畸形和心脏病综合征的患者中:迪格奥尔格综合征、心脏-面综合征和圆锥动脉干异常面容综合征。我们报告了一名患有不对称哭脸综合征的新生儿,其伴有先天性甲状旁腺功能减退、严重新生儿低钙血症和法洛四联症。已对22q11染色体缺失进行了基因确认。