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Infantile mammary duct ectasia: A cause of bloody nipple discharge
Infantile Mammary Duct Ectasia: A Cause of Bloody Nipple Discharge By Gustavo Stringel, Alvin Perelman, and Carmencita Jimenez Dallas, Texas and Ottawa, Ontario, Canada 9 Bloody nipple discharge in infancy has been rarely reported in the medical literature. Its cause is unknown. W e report a three-year-old male infant and a five-monthold female infant with bloody nipple discharge. Because of persistent bloody discharge, a subcutaneous mastectomy was performed in the boy; the problem resolved in the girl after a period of observation. The specimen showed histologic changes identical to those seen in adult mammary duct ectasia. All the endocrinologic work-up was normal. W e suspect that bloody nipple discharge in infancy is underreported. This is a benign condition with histologic changes similar to adult mammary duct ectasia and if persistent, should be properly investigated; biopsy or excision are not indicated. 9 1986 by Grune & Stratton. Inc. INDEX WORDS: Mammary duct ectasia; nipple discharge.
I P P L E DISCHARGE in infancy is extremely
N rare except for the physiologic clear or milky discharge seen in normal newborn babies. ~'2 Bloody nipple discharge was reported by Berkowitz and Inkelis in two infants? a six-week-old male and a sixweek-old female, and also by Fenster in an eightmonth-old male. 4 The cause of this condition in infancy is unknown. We report on a three-year-old boy and a fivemonth-old female infant with bloody nipple discharge. In the five-month-old infant girl, the condition resolved spontaneously while the three-year-old boy required bilateral subcutaneous mastectomy for persistent bloody discharge. The histologic picture was similar to adult mammary duct ectasia. CASE REPORTS
Case 1 A three-year-old boy presented with a bloody discharge from the right nipple. The past history was normal with no evidence of previous breast pathology. Hgb was 11.7 gm and WBC 7.3 x 103; chest x-ray was normal. After three months, because of persistent bleeding, a subcutaneous mastectomy was performed. He recovered uneventfully. The specimen showed prominent ductal ectasia extending down to the collecting tubules. Several dilated ducts contained inspissated eosinophilic secretions as well as clusters of xanthomatous cells, hemosiderin-laden macrophages and occasional needle-shaped cholesterol clefts (Fig 1). The ectatic ducts were surrounded by varying stages of fibrosis. A variable number of lymphocytic and plasma cell infiltrates were present around the ectatic ducts and were usually situated outside the zone of periductal fibrosis (Figs 1 and 2). One year later, he developed a bloody nipple discharge from the left breast. It was again successfully treated with subcutaneous mastectomy. The second specimen showed the same histologic features as the first biopsy.
Journal of Pediatric Surgery, Vol 21, No 8 (August), 1986: pp 671-674
Laboratory investigations showed normal estradiol, estrone, progesterone, FSH, LH, and testosterone levels. Prolactin was slightly elevated on two occasions at 26.3 tzg/L and 25 t~g/L (normal 5 to 13 t~g/L). Thyroid releasing hormone (TRH) stimulation test showed a normal rise of prolactin to 80 #g/L. Thyroid stimulating hormone (TSH) rise was normal. CT scan of the head was normal. A swab from the left nipple discharge grew Staphylococcus epidermidis. After three years of follow-up, he has no further problems.
Case 2 A five-month-old healthy female presented with a bloody discharge from the left nipple (Fig 3). Drug ingestion or breast manipulation was denied. On physical examination, she had a small nodule palpable under the left areola. Culture of the left nipple discharge grew Staphylococcus epidermidis. The bloody nipple discharge was intermittent and completely stopped at eight months of age without treatment. After one year of follow-up, she has not had any further nipple discharge and examination of the breast is normal. DISCUSSION
Transient breast enlargement is commonly seen in full-term newborn infants of either sex and is often associated with clear or milky discharge; this physiologic breast enlargement is due to passive hormonal stimulation from mother across the placental circulation. It usually resolves completely within a few weeks. Manipulation of the breast could prolong this condition and lead to trauma and infection, t-3 Occasionally, the infant will be left with small breast induration for a few months but further nipple discharge is very rare. 1'2 Chronic cystic mastitis has been mentioned as a possible cause of bloody nipple discharge and intraductal cysts in children but the frequency or age distribution is not specified) Intraductal papilloma, a common cause of bloody nipple discharge in adults occasionally occurs in chil-
From the Department of Surgery, Division of Pediatric Surgery, University of Texas Health Science Center at Dallas, and the Departments of Pediatrics and Pathology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada. Presented at the 34th Annual Meeting of the Surgical Section of the American Academy of Pediatrics, San Antonio, Texas, October 19-20, 1985. Address reprint requests to Gustavo Stringel, MD, Department of Surgery, Division of Pediatric Surgery, University of Texas Health Science Center at Dallas, 5323 Harry Hines, Dallas, TX 75235. 9 1986 by Grune & Stratton, Inc. 0022-3468/86/2108-0002503.00/0 671
672
Fig 1. Dilated duct on the right contains proteinaceous secretions and red blood cells. Residual chronic inflammatory infiltrates are still present and characteristically located external to the zone of periductal fibrosis ( • 110).
dren. We could find only one reported case in a child under 4 years of age. 6 M a m m a r y duct ectasia was described by Haagensen. 7 It is a benign condition characterized by dilatation of the subareolar duct system, with inflammatory reaction and fibrosis. It was initially believed to be a disease of older women but it has been recently reported to occur in young women s-l~ and also in m e n . 11,12
Possible etiologic factors are chronic inflammation of the periductal stroma with obliteration of the lumenS; congenital abnormalities of nipple and duct
STRINGEL ET AL
systemt~ infection, 9 t r a u m a ) 3 and autoimmune reaction) 4 Specific etiology remains unknown. Although bloody discharge in infancy has been previously reported, 3'4 its cause is unclear and is regarded as similar to that seen in pregnancy. On the other hand, transient duct ectasia has been said to occur in normal newborn infants as a result of transplacental passage of maternal hormones that stimulates the neonatal b r e a s t : In our three-year-old male patient, simple breast excision was undertaken because of persistent bleeding; the histology in the resected specimen was identical to the one described in m a m m a r y duct ectasia. Later occurrence of bleeding from the other breast was worrisome since we thought that an abnormal hormonal stimulus could be the cause. Exhaustive investigations were normal except for elevated serum prolactin measured on two separate occasions but the T R H stimulation test was normal. Local stimulation as a cause of hyperprolactinemia was ruled out since the prolactin levels were measured several weeks after surgery. A C A T scan excluded a prolactinoma. Prolactin does not cause discernable breast pathology except for galactorrhea and thus was felt to be an incidental finding. In the five-month-old female, infant biopsy or excision was thought to be contraindicated due to the age and sex of the patient. Other investigations were not undertaken since her bloody discharge resolved very rapidly. Bloody nipple discharge can be associated with malignancy in the adult population t5:6 but carcinoma of the breast is extremely rare in children and to our knowledge not yet reported in infants under three of age. 2'3'17For this reason, biopsy or excision of the breast to rule out malignancy should be avoided in this age group.
Fig 2. Inspissated contents of larger duct include needle-like cholesterol clefts. Inflammatory process is no longer evident around these larger ducts (• 70).
INFANTILE MAMMARY DUCT ECTASIA
673
A
B
?
Fig 3.
(A) A five-month-old infant with (B) bloody nipple discharge.
Karl et a117 reported 17 cases of juvenile secretory carcinoma of the breast. The ages ranged from 3 to 17 years; all these children presented with an asymptomatic breast mass and none had nipple discharge. Infection has been implicated as a possible etiologic factor in m a m m a r y ductal ectasia. 8'13Our two patients "grew Staphylococcus epidermidis. T h e y were not treated with antibiotics because we thought this organism was a contaminant from the skin. In the adult population, m a m m a r y ductal ectasia is treated by local excision in the female 8'1~ and simple mastectomy in the male. H'12 In children, bloody discharge should be m a n a g e d expectantly with repeated clinical assessments. If it
persists or the breast continues to enlarge, appropriate endocrinologic work-up should be undertaken preferably by a pediatric endocrinologist. Breast biopsy or excision should be avoided in female infants since this could cause permanent destruction of the developing breast. 3 In male infants, when they continue to have pain, bloody nipple discharge, or breast enlargement, a subcutaneous mastectomy will resolve the problem when other measures have failed. Stimulation or massage of the breast should be avoided. Cultures should be taken and appropriate antibiotics administered when indicated.
REFERENCES
1. McKiernan JF, Hull D: Breast development in the newborn. Arch Dis Child 56:525-529, 1981 2. Dewhurst S: Breast disorders in children and adolescents. Pediatr Clin Am 28:287-308, 1981 3. Berkowitz CD, lnkelish SH: Bloody nipple discharge in infancy. J Pediatr 103:755-758, 1983 4. Fenster DL: Bloody nipple discharge. J Pediatr 104:640-641, 1984 5. Ravitch M: The breast, in Ravitch MM, Welch KJ, Benson CD, et al (eds): Pediatric Surgery. Chicago, Year Book Medical, 1979, pp 401-406
6. Simpson JS, Barson AJ: Breast tumors in infants and children. Can Med Assoc J 101:100-102, 1969 7. Haagensen CD: Mammary duct ectasia: A disease that may stimulate carcinoma. Cancer 4:749-761, 1951 8. O'Brien PH, Kreutner A: Another cause of nipple discharge mammary duct ectasia with periductal mastitis. Surg 48:577-578, 1982 9. Dixon JM, Anderson T J, Lumsden AB, et al: Mammary duct ectasia. Br J Surg 70:601-603, 1983 10. Izzidien AY: Mammary duct ectasia. Clin Notes 27:361362, 1982
674
11. Mansel RE, Morgan WP: Duct ectasia in the male. Br J Surg 66:660-662, 1979 12. Chan KW, Lau WY: Duct ectasia in the male breast. Aust N Z J Surg 25:173-176, 1984 13. Thomas WG, Williamson RCN, Davies JD, et al: The clinical syndrome of mammary duct ectasia. Br J Surg 69:423-425, 1982 14. Davies JD: Histological study of mamae in oestrogenised rats
STRINGEL ET AL
after mammary isoimmunisation. Br J Exp Pathol 53:406-414, 1972 15. Murad TM, Contesso G, Mouriesse H: Nipple discharge from the breast. Ann Surg 195:259-264, 1982 16. Chaudary MA, Millis RR, Davies GC, et al: Nipple discharge. Ann Surg 195:651-655, 1982 17. Karl SR, Ballantine TV, Zaino R: Juvenile secretory carcinoma of the breast. J Pediatr Surg 20:368-371, 1985
I P P L E DISCHARGE in infancy is extremely
N rare except for the physiologic clear or milky discharge seen in normal newborn babies. ~'2 Bloody nipple discharge was reported by Berkowitz and Inkelis in two infants? a six-week-old male and a sixweek-old female, and also by Fenster in an eightmonth-old male. 4 The cause of this condition in infancy is unknown. We report on a three-year-old boy and a fivemonth-old female infant with bloody nipple discharge. In the five-month-old infant girl, the condition resolved spontaneously while the three-year-old boy required bilateral subcutaneous mastectomy for persistent bloody discharge. The histologic picture was similar to adult mammary duct ectasia. CASE REPORTS
Case 1 A three-year-old boy presented with a bloody discharge from the right nipple. The past history was normal with no evidence of previous breast pathology. Hgb was 11.7 gm and WBC 7.3 x 103; chest x-ray was normal. After three months, because of persistent bleeding, a subcutaneous mastectomy was performed. He recovered uneventfully. The specimen showed prominent ductal ectasia extending down to the collecting tubules. Several dilated ducts contained inspissated eosinophilic secretions as well as clusters of xanthomatous cells, hemosiderin-laden macrophages and occasional needle-shaped cholesterol clefts (Fig 1). The ectatic ducts were surrounded by varying stages of fibrosis. A variable number of lymphocytic and plasma cell infiltrates were present around the ectatic ducts and were usually situated outside the zone of periductal fibrosis (Figs 1 and 2). One year later, he developed a bloody nipple discharge from the left breast. It was again successfully treated with subcutaneous mastectomy. The second specimen showed the same histologic features as the first biopsy.
Journal of Pediatric Surgery, Vol 21, No 8 (August), 1986: pp 671-674
Laboratory investigations showed normal estradiol, estrone, progesterone, FSH, LH, and testosterone levels. Prolactin was slightly elevated on two occasions at 26.3 tzg/L and 25 t~g/L (normal 5 to 13 t~g/L). Thyroid releasing hormone (TRH) stimulation test showed a normal rise of prolactin to 80 #g/L. Thyroid stimulating hormone (TSH) rise was normal. CT scan of the head was normal. A swab from the left nipple discharge grew Staphylococcus epidermidis. After three years of follow-up, he has no further problems.
Case 2 A five-month-old healthy female presented with a bloody discharge from the left nipple (Fig 3). Drug ingestion or breast manipulation was denied. On physical examination, she had a small nodule palpable under the left areola. Culture of the left nipple discharge grew Staphylococcus epidermidis. The bloody nipple discharge was intermittent and completely stopped at eight months of age without treatment. After one year of follow-up, she has not had any further nipple discharge and examination of the breast is normal. DISCUSSION
Transient breast enlargement is commonly seen in full-term newborn infants of either sex and is often associated with clear or milky discharge; this physiologic breast enlargement is due to passive hormonal stimulation from mother across the placental circulation. It usually resolves completely within a few weeks. Manipulation of the breast could prolong this condition and lead to trauma and infection, t-3 Occasionally, the infant will be left with small breast induration for a few months but further nipple discharge is very rare. 1'2 Chronic cystic mastitis has been mentioned as a possible cause of bloody nipple discharge and intraductal cysts in children but the frequency or age distribution is not specified) Intraductal papilloma, a common cause of bloody nipple discharge in adults occasionally occurs in chil-
From the Department of Surgery, Division of Pediatric Surgery, University of Texas Health Science Center at Dallas, and the Departments of Pediatrics and Pathology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada. Presented at the 34th Annual Meeting of the Surgical Section of the American Academy of Pediatrics, San Antonio, Texas, October 19-20, 1985. Address reprint requests to Gustavo Stringel, MD, Department of Surgery, Division of Pediatric Surgery, University of Texas Health Science Center at Dallas, 5323 Harry Hines, Dallas, TX 75235. 9 1986 by Grune & Stratton, Inc. 0022-3468/86/2108-0002503.00/0 671
672
Fig 1. Dilated duct on the right contains proteinaceous secretions and red blood cells. Residual chronic inflammatory infiltrates are still present and characteristically located external to the zone of periductal fibrosis ( • 110).
dren. We could find only one reported case in a child under 4 years of age. 6 M a m m a r y duct ectasia was described by Haagensen. 7 It is a benign condition characterized by dilatation of the subareolar duct system, with inflammatory reaction and fibrosis. It was initially believed to be a disease of older women but it has been recently reported to occur in young women s-l~ and also in m e n . 11,12
Possible etiologic factors are chronic inflammation of the periductal stroma with obliteration of the lumenS; congenital abnormalities of nipple and duct
STRINGEL ET AL
systemt~ infection, 9 t r a u m a ) 3 and autoimmune reaction) 4 Specific etiology remains unknown. Although bloody discharge in infancy has been previously reported, 3'4 its cause is unclear and is regarded as similar to that seen in pregnancy. On the other hand, transient duct ectasia has been said to occur in normal newborn infants as a result of transplacental passage of maternal hormones that stimulates the neonatal b r e a s t : In our three-year-old male patient, simple breast excision was undertaken because of persistent bleeding; the histology in the resected specimen was identical to the one described in m a m m a r y duct ectasia. Later occurrence of bleeding from the other breast was worrisome since we thought that an abnormal hormonal stimulus could be the cause. Exhaustive investigations were normal except for elevated serum prolactin measured on two separate occasions but the T R H stimulation test was normal. Local stimulation as a cause of hyperprolactinemia was ruled out since the prolactin levels were measured several weeks after surgery. A C A T scan excluded a prolactinoma. Prolactin does not cause discernable breast pathology except for galactorrhea and thus was felt to be an incidental finding. In the five-month-old female, infant biopsy or excision was thought to be contraindicated due to the age and sex of the patient. Other investigations were not undertaken since her bloody discharge resolved very rapidly. Bloody nipple discharge can be associated with malignancy in the adult population t5:6 but carcinoma of the breast is extremely rare in children and to our knowledge not yet reported in infants under three of age. 2'3'17For this reason, biopsy or excision of the breast to rule out malignancy should be avoided in this age group.
Fig 2. Inspissated contents of larger duct include needle-like cholesterol clefts. Inflammatory process is no longer evident around these larger ducts (• 70).
INFANTILE MAMMARY DUCT ECTASIA
673
A
B
?
Fig 3.
(A) A five-month-old infant with (B) bloody nipple discharge.
Karl et a117 reported 17 cases of juvenile secretory carcinoma of the breast. The ages ranged from 3 to 17 years; all these children presented with an asymptomatic breast mass and none had nipple discharge. Infection has been implicated as a possible etiologic factor in m a m m a r y ductal ectasia. 8'13Our two patients "grew Staphylococcus epidermidis. T h e y were not treated with antibiotics because we thought this organism was a contaminant from the skin. In the adult population, m a m m a r y ductal ectasia is treated by local excision in the female 8'1~ and simple mastectomy in the male. H'12 In children, bloody discharge should be m a n a g e d expectantly with repeated clinical assessments. If it
persists or the breast continues to enlarge, appropriate endocrinologic work-up should be undertaken preferably by a pediatric endocrinologist. Breast biopsy or excision should be avoided in female infants since this could cause permanent destruction of the developing breast. 3 In male infants, when they continue to have pain, bloody nipple discharge, or breast enlargement, a subcutaneous mastectomy will resolve the problem when other measures have failed. Stimulation or massage of the breast should be avoided. Cultures should be taken and appropriate antibiotics administered when indicated.
REFERENCES
1. McKiernan JF, Hull D: Breast development in the newborn. Arch Dis Child 56:525-529, 1981 2. Dewhurst S: Breast disorders in children and adolescents. Pediatr Clin Am 28:287-308, 1981 3. Berkowitz CD, lnkelish SH: Bloody nipple discharge in infancy. J Pediatr 103:755-758, 1983 4. Fenster DL: Bloody nipple discharge. J Pediatr 104:640-641, 1984 5. Ravitch M: The breast, in Ravitch MM, Welch KJ, Benson CD, et al (eds): Pediatric Surgery. Chicago, Year Book Medical, 1979, pp 401-406
6. Simpson JS, Barson AJ: Breast tumors in infants and children. Can Med Assoc J 101:100-102, 1969 7. Haagensen CD: Mammary duct ectasia: A disease that may stimulate carcinoma. Cancer 4:749-761, 1951 8. O'Brien PH, Kreutner A: Another cause of nipple discharge mammary duct ectasia with periductal mastitis. Surg 48:577-578, 1982 9. Dixon JM, Anderson T J, Lumsden AB, et al: Mammary duct ectasia. Br J Surg 70:601-603, 1983 10. Izzidien AY: Mammary duct ectasia. Clin Notes 27:361362, 1982
674
11. Mansel RE, Morgan WP: Duct ectasia in the male. Br J Surg 66:660-662, 1979 12. Chan KW, Lau WY: Duct ectasia in the male breast. Aust N Z J Surg 25:173-176, 1984 13. Thomas WG, Williamson RCN, Davies JD, et al: The clinical syndrome of mammary duct ectasia. Br J Surg 69:423-425, 1982 14. Davies JD: Histological study of mamae in oestrogenised rats
STRINGEL ET AL
after mammary isoimmunisation. Br J Exp Pathol 53:406-414, 1972 15. Murad TM, Contesso G, Mouriesse H: Nipple discharge from the breast. Ann Surg 195:259-264, 1982 16. Chaudary MA, Millis RR, Davies GC, et al: Nipple discharge. Ann Surg 195:651-655, 1982 17. Karl SR, Ballantine TV, Zaino R: Juvenile secretory carcinoma of the breast. J Pediatr Surg 20:368-371, 1985