Supernumerary Nipples
Supernumerary Nipples (Polythelia)
Introduction
Supernumerary nipples (polythelia) represent a common congenital malformation characterized by the presence of additional nipples or breast tissue along the embryonic mammary lines.
Key Points
- Prevalence: 0.2-5.6% of general population
- Male:Female ratio approximately 1:1
- More common in certain ethnic groups
- Usually asymptomatic
- May have clinical significance due to associated conditions
Embryology
Developmental Basis
- Embryonic Mammary Ridge:
- Develops during 4th-5th week of gestation
- Extends from axilla to inguinal region
- Forms milk lines bilaterally
- Normal regression except in pectoral region
- Pathogenesis:
- Incomplete regression of mammary ridge
- Persisting mammary precursors
- Genetic factors influence development
- Environmental factors may play role
Clinical Features
Anatomical Distribution
- Location Patterns:
- Most common: Below normal nipple (90%)
- Along embryonic milk lines
- Can be unilateral or bilateral
- Rare ectopic locations possible
- Anatomical Sites:
- Axillary region
- Inframammary region
- Epigastric area
- Inguinal region
Physical Characteristics
- Appearance:
- Size: Usually smaller than normal nipples
- Color: Similar to normal nipples
- Shape: Round to oval
- Surface: May be flat or raised
- Associated Features:
- Areolar tissue may be present
- Underlying breast tissue possible
- Varying degrees of development
- May become more prominent during puberty
Classification
Kajava Classification (1915)
- Class I:
- Complete supernumerary nipple
- Nipple, areola, and glandular tissue
- Class II:
- Nipple and glandular tissue
- No areola
- Class III:
- Areola and glandular tissue
- No nipple
- Class IV:
- Glandular tissue only
- Class V:
- Nipple and areola
- No glandular tissue
- Class VI:
- Nipple only
- Class VII:
- Areola only
- Class VIII:
- Patch of hair only
Associated Conditions
Genitourinary Anomalies
- Renal System:
- Renal agenesis
- Renal cell carcinoma
- Hydronephrosis
- Polycystic kidney disease
- Other Urological Conditions:
- Hypospadias
- Ureteral duplication
- Bladder anomalies
Cardiovascular Conditions
- Congenital heart defects
- Conduction abnormalities
- Cardiomyopathies
Syndromes
- Simpson-Golabi-Behmel syndrome
- Turner syndrome
- Char syndrome
- Fanconi anemia
Diagnosis
Clinical Assessment
- Physical Examination:
- Systematic inspection along milk lines
- Assessment of size and symmetry
- Documentation of location
- Classification according to Kajava
- History Taking:
- Family history
- Associated symptoms
- Developmental history
- Previous medical conditions
Diagnostic Workup
- Imaging Studies:
- Ultrasound of breast tissue
- Renal ultrasound
- Echocardiogram if indicated
- Laboratory Tests:
- Genetic testing if syndromic
- Hormonal studies if indicated
Management
Observation
- Most cases require no treatment
- Regular monitoring during:
- Puberty
- Pregnancy
- Lactation
Surgical Management
- Indications:
- Cosmetic concerns
- Psychological distress
- Symptomatic cases
- Risk of malignancy
- Surgical Options:
- Simple excision
- Subcutaneous mastectomy
- Liposuction (if indicated)
Special Considerations
Pregnancy and Lactation
- Hormonal Response:
- May enlarge during pregnancy
- Potential lactation
- Possible complications
- Management During Pregnancy:
- Monitoring for changes
- Address lactation concerns
- Prevention of complications
Long-term Follow-up
- Regular Monitoring:
- Annual physical examination
- Assessment during hormonal changes
- Screening for associated conditions
- Patient Education:
- Self-examination techniques
- Warning signs
- When to seek medical attention
Disclaimer
The notes provided on Pediatime are generated from online resources and AI sources and have been carefully checked for accuracy. However, these notes are not intended to replace standard textbooks. They are designed to serve as a quick review and revision tool for medical students and professionals, and to aid in theory exam preparation. For comprehensive learning, please refer to recommended textbooks and guidelines.
