Poland syndrome a rare congenital anomaly
An eight-year-old girl first seen at the Paediatric Out-Patient Department of Aminu Kano Teaching Hospital, Kano, with left anterior chest wall deformity since birth and recurrent loss of consciousness of two years duration. There was no history of breathlessness, paroxysmal nocturnal dyspnea or orthopnea. About a year ago, she developed a swelling on the left side of the jaw which was incised by a traditional barber and a thick whitish secretion was drained. However since then mother noticed that the site had been discharging clear fluid intermittently whenever She laughed, coughed, ate or cried. There was history of syncopal attacks lasting for few minutes noticed whenever she was carried on the back and the left side of the chest is compressed and also during play when she is being hit or pushed on the chest. She was a product of full-term gestation and pregnancy was not supervised. The pregnancy and delivery were not adversely eventful. The child was the fourth of the mother's five children in a polygamous family setting of a non-consanguineous marriage. There was no family history of congenital anomalies.\nExamination findings revealed a girl with normal growth parameters and good mental status. She had an actively discharging tract at the region of the angle of the left mandible which was about 2 cm below the left earlobe []. A rudimentary accessory nipple was seen on the left side with depression of the left hemithorax. Pectoralis major muscle, the third, fourth and fifth ribs were absent on the left side []; the left scapula was hypoplastic and displaced upward [], but the left latissimus dorsi was well preserved. No limbs abnormalities were seen. She had normal cardiovascular examination findings.\nShe had a chest radiograph which showed absence of the left 3rd, 4th and 5th ribs []. Abdominal ultrasound, electrocardiogram and echocardiogram were normal. A diagnosis of Poland syndrome, esophago-cutaneous fistula and recurrent cardiac syncope was made. The patient and parents were counseled, and she is awaiting reconstructive surgery for the chest defect.