CASE REPORT
Unilateral
intra-areolar
polythelia:
a rare anomaly
G. M. Baratelli and S. Vischi Delegctkne Alto Lorio Grmvdotta (Cottto), Delega~iotte di Uggirrte Trevmo (Cotno), Lrgct Italiattn per la Lotto contro i Tutttori, Ito!\ S U M MA R Y. Intra-areolar polythelia (IAP) is a rare anomaly of breast development characterized by the presence of two nipples located within an areola of normal size. The first two cases of sporadic, unilateral IAP in women are reported. In one case lactation and nursing from both of the doubled intra-areolar nipples had occurred.
INTRODUCTION Accessory nipples (polythelia) and accessory breast tissue (polymastia) are anomalies of breast development. Supernumerary nipples are usually rudimentary and located along the milk line, extending from the axilla to the groin. Two cases of unilateral in&a-areolar polythelia (IAP) are described, an extremely rare condition of breast development characterized by two nipples located within an areola of normal size.
CASE REPORTS Case 1 A 36-year-old. para 3 + 0. healthy woman, examined in a breast cancer screening programme, was found to have two nipples within the areola of her right breast (Fig. I ). The double nipples were similar in size and both appeared to be normally developed. The size of the right breast and its areola was normal and similar to the left. The pectoralis major muscles were present bilaterally and normally developed. Ultrasound failed to demonstrate any urinary system abnormality. She had no family history of similar anomalies. At age 28 she had delivered a full term, normal child; during the subsequent 3 months she lactated and the child nursed from both nipples of the right breast and from the single nipple of the left.
Fig. 1 X4&
Case
I : two similar
and normally
developed
nipples
in the same
Neither surgical correction of the anomaly nor any other treatment was advised because the anomaly caused this woman neither physiological nor psychological dysfunction.
Case 2 A 26-year-old, nulliparous, healthy woman, examined in the same breast cancer screening programme as in Case I. was found to have two nipples within the areola of her right breast (Fig. 2). The double nipples were dissimilar in size. One of the nipples was located centrally in the right areola and appeared normal, the second was significantly smaller and was located at the border of the areola. The size of
Addrc~ss ~orre.v~on~lr,~c,e ro: G. M. Baratelli. Delegazione Alto Lario, Lega Italiana per la Lotte contra i Tumori 220 IS Gravedona (Coma). Italy. Tel.: +39 344 89505. +39 344 92 I I I; Fax: +39 344 ,%72X. +39 3JJ X3396: E-mail:
[email protected]
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52
Fig. 2
The Breast
Case 2: two dissimilar
nipples
located
within
the areola.
along the milk lines that normally regress during the third month of intrauterine development. Intra-areolar polythelia (IAP) is an extremely rare anomaly. Nine cases of bilateral IAP are in the literature.‘” One case of unilateral IAP occurring in a male has been reported.’ Familial IAP has been described and may be associated with other anomalies such as breast or pectoralis muscle hypoplasia and renal pelvis or ureteral reduplication.6 To our knowledge no cases of sporadic, unilateral IAP in women have been reported. It is believed that the embryologic origin of LAP is a dichotomy of the developing nipple during foetal life. In the two cases we have reported, the double nipples were located within normal size areolae with normally developed breasts. In one case lactation and nursing from both intra-areolar nipples proved the existence of two independent and normally developed lactiferous systems.
Acknowledgement the right breast and its areola was normal and similar to the left. The pectoralis major muscles were bilaterally present and normally developed. Ultrasound failed to demonstrate urinary system abnormality. She had no family history of similar anomalies and no treatment was advised.
COMMENT Reports of developmental breast anomalies are not uncommon. Absence of a breast (amastia) or of a nipple (athelia) are rare. Supernumerary nipples, usually located along the milk line and sometimes accompanied with breast tissue, are found in 2-6% of adult women.’ They are believed to arise from the persistence of ectodermal ridges
The authors wish to thank Dr Russell for his friendly assistance in preparing
N. Sacco (Portland, the manuscript.
Oregon,
USA)
References I. Osborne M P. Breast development and anatomy. In: Herris J R. Hellman S. Henderson I C, Kinne D W. Breast Diseases. 2nd ed Philadelphia: Lippincott, 1991, pp 1. 2. Abramson D J. Bilateral intra-areolar polythelia. Arch Surg 1975: 1 IO: 1255. 3. Brightmore T. Bilateral double nipples. Br J Surg 1972; 59: 55. 4. Butts D C. Paired nipples. JAMA 1954; 154: 1371. 5. De Cholnoky T. Supernumerary breast. Arch Surg 1939: 39: 926. 6. Rintala A. Norio R. Familial intra-areolar polythelia with mammary hypoplasia. Stand Reconstr Surg 1982; 16: 287-291. 7. Urbani C E, Berti R. Sporadic unilateral intra-areolar polythelia. Report of an additional case and review of the literature. Acta derm Venorol 1996: 76: 156.
