腸性囊腫併多發性先天性畸形之一例報告

Enterogenous Cyst Associated with Multiple Congenital Anomalies-a Case Report-

林隆煌(Lung-Huang Lin) ； 陳德輝(tou-Hwei Chen) ； 沈慶村(Ching-Tsuen Shen) ； 林永和(Yung-Ho Lin) ； 侯書文(Shu-Wen How)

《Acta Paediatrica Sinica》 23卷4期 (1982/12) Pp. 254-258

摘要

腸性囊腫乃一種極罕見之先天性異常，本院發現一個病例，伴有膈疝氯，裏頭有小腸，部分嵌入胸椎裂黏接硬膜，脊柱在胸椎1-4有前裂，並有脊髓異常，以及先天性心臟病等多發性畸形，在國內尚屬首見，故提出報告。

並列摘要

Enterogenous cyst is a rare congenital anomaly, which may contain microscopically the histological structures of epithelium, connective tissue, gland, muscle, cartilage, bone etc. Many kinds of congenital anomalies associated with the enterogenous cyst had been reported. Here a case of enterogenous cyst with complex anomalies inclding diaphragmatic hernia, spina bifida and congenital heart disease is presented and discussed. A 2,300gm female premature baby, G2 P2, was a product of a 30-year-old woman after 37 weeks' gestation through a normal spontaneous delivery. Because of apnea and cardiac standstill since her birth, cardiopulmonary resuscitations were performed immediately, and endotracheal intubation with high oxygen concentration inhalation followed without delay. Physical examination at the Neonatal Intensive Care Unit revealed that the baby was on respirator with FiO2 100%, I:E ratio 1:2 rate 40/min, inspiratory pressure 20 cmH2O. The left shoulder was depressed and retracted during inspiration. The maximal cardiac apical impulse was felt at the right fourth intercostal space, in the midclavicular line. The liver was palpable 8cm below the right costal margin. The four extremities were paralytically flaccid. Chest roentgenograms showed an air shadow about 4×4cm in the mediastinum, thoracic spina bifida from T1 to T4 and missing left first to fourth ribs. The heart rate was 137/min and regular. EKG revealed PR interval 0.12 sec, and progressively decreasing R amplitude from V6R to V6. In spite of active resuscitation and supportive treatment, respiratory acidosis persisted and the patient expired 50 hours after birth. Autopsy revealed a diaphragmatic hernia in the mediastinum containing the small intestine. One end of the intestine became a diverticulum and the other end attached to the thoracic spinal cord forming an enterogenous cyst. Microscopically the cyst contained the histological structures of cartilage, bronchial epithelium and gland.

並列關鍵字

enterogenous cyst ； diaphragmatic hernia ； spina bifida