Patient Report Supernumerary nipple: Should we be alert? Ayse Esra Yilmaz, 1 Evren Sarifakioglu, 2 Guzide Dogan, 1 Banu Cakir, 3 Musemma Karabel, 1 Canan Gorpelioglu 2 and Sadi Turkay 1 Departments of 1 Pediatrics, 2 Dermatology and 3 Radiology, Fatih University Faculty of Medicine, Ankara, Turkey Key words malformations, ovarian cyst, renal anomalies, rib anomalies, supernumerary nipples, syndrome. Supernumerary nipples (SN) are common minor congenital anomalies. SN pose no medical threat, however they can be associated with other medical conditions especially kidney and urinary tract malformations. We present here a female infant with SN with interesting associated malformations. In our patient, umbilical hernia, umbilical granuloma, spina bifida occulta and bilateral ovarian cyst accompanied the accessory nipple, instead of the expected renal or urinary system anomalies. Case Report A 53-day-old female infant presented with the complaint of a skin fold present in the lower back ever since birth. Her mother was 28 years old and gave birth to her vaginally at the 40th week of pregnancy following a healthy pregnancy. The infant was the mother’s second pregnancy and second living offspring. Postna- tal history was unremarkable. The family history was also unre- markable. The mother and father were non-consanguineous. Physical examination findings of the mother and father were normal. No family history of similar physical anomalies, malig- nancy, cardiovascular or renal diseases was present. On physical examination the infant’s weight (6 kg; 75–90%), height (61 cm; 75–90%), and head circumference (43 cm; 75–90%) were normal; her vital findings were stable, her general condition was well and she was active and lively. Approximately 2 cm below the right nipple, an accessory nipple without areola was seen (Fig. 1). Umbilical hernia and umbilical granuloma were present. Gluteal cleft was in bifid form. On her right hip, pili asymmetry was observed. No atypical facial appearance, skeletal anomaly or mental–motor retardation findings were found. In laboratory investigations, hematological and biochemical abnormalities were normal. On radiological examination, in ultrasonographic screening of the whole abdomen, no anomaly was seen apart from ovarian cysts. In the ultrasonographic examination of the accessory nipple, it was observed to consist solely of the nipple, without breast tissue. According to the supernumerary nipple classification by Kajava, 1 the supernumerary nipple in our patient was classified as polythelia. Serial graphics were taken for a probable skeletal anomaly, but no anomalies were found. Echocardiography, taken to determine a possible cardiac anomaly (like mitral valve prolapse), was normal. Transfontanel ultrasonography was also normal. In a magnetic resonance imaging investigation of the sacral region, spina bifida occulta (Fig. 2), slight dysplasia in the right hip, two bilateral ovarian cysts on the left (11 and 8 mm) and one ovarian cyst on the right (9 mm) were established. Correspondence: Ayse Esra Yilmaz, MD, Department of Pediatrics, Fatih University Faculty of Medicine, Alparslan Turkes Caddesi No: 57/Ankara/Turkey. Email: aysesra@yahoo.com Received 10 June 2009; revised 13 October 2009; accepted 15 December 2009. Fig. 1 Supernumerary nipple. Pediatrics International (2010) 52, e190–e191 doi: 10.1111/j.1442-200X.2010.03120.x © 2010 Japan Pediatric Society A22