DISCUSSION
Normally, breast development begins with the formation of the mammary ridge from the ectoderm on the ventral surface of the body during the sixth week of gestation.8 Then, subsequent development of primary mammary buds, secondary mammary buds, and lactiferous systems occur. In the late fetal period, nipple and areola form from the mammary pits.8,9
Breast remains largely unchanged from birth to puberty though the lobular alveolar and the ductal systems grow under the strong influence of estrogen.9 From puberty onwards, normal breast development occurs over two to five years involving all tissues of the breast due to the result of the anterior pituitary hormone through follicle-stimulating hormone, luteinizing hormone, growth hormone, and adrenocorticotrophic hormone along with the estrogen. Estrogen has the strongest effect on ductal growth.8,9 In addition to these, progesterone and prolactin have a role in lobular-alveolar development.10 Similarly, fibrous stroma and fatty tissues also increase in the puberty. However, sometimes massive or excessive growth of the unilateral or bilateral breasts can occur despite the normal level of gonadal hormones. Though the exact cause remains unknown, it is believed to be due to idiopathic end-organ hypersensitivity to the normal level of gonadal hormones.11 Molecular studies regarding the VBH show that PTEN gene which encodes for tumor-suppressing phosphatase protein, which when mutated in the mice showed excessive ductal branching, precocious development, and severely reduced apoptosis in the mammary gland.12 However, such testing is rarely done in the suspected case of VBH. Koves et al 13 reported the case which turned out negative for PTEN mutation.
With the increasing size of the breast in the initial period, the breast becomes unevenly large and pendulous with prominent superficial veins. Nipple and areola are typically stretched and may be difficult to distinguish.8 The increasing size and weight of the breast may cause breast tenderness, difficulty in supporting the weight of the breast, postural problems, respiratory problems, neck pain, back pain, and sometimes decreased sensation of ulnar nerve distribution.5,8,14 Due to chronic irritation of the bra straps, shoulders can be grooved and scarred.5,8Similarly, intertrigo and stretch marks can also be observed.14 Our patient had chest discomfort and pressure sore in the inframammary folds and had grooved shoulders due to bra-straps. Diffuse firmness, or one or more rubbery masses on palpation may be present, and axillary lymph nodes are also not enlarged.8 Such masses were not present in our case. And axillary lymph nodes were not enlarged.
As exemplified in our case, a sudden change of the body shape in comparison to peers, and unfitting clothes may lead to social embarrassment and even depression, which is a major risk that requires psycho-social care to the patient.3,5 In our case, the patient had limited her social activities and stopped going to school due to the embarrassing size of her breasts.
Though cases of VBH have been mentioned in the literature, we could not find the true incidence of VBH and it remains unknown. Being rare, the initial presentation of this entity can easily divert the surgeon to the other pathologies like fibroepithelial tumors (fibroadenoma and phyllode tumor), adolescent macromastia and fibrocystic changes which are more common than VBH in this age group.1,15 Other conditions, like trauma, dermal infiltrative processes such as lymphoma and leukemia cutis, pancolitis, infection, and breast malignancies though rare, should be kept in mind before reaching the final diagnosis.2 Two rare events about the primary and secondary lymphoma mimicking juvenile breast hypertrophies have been reported.16 So, it is necessary to keep the differentials wide.
There are no specific treatment strategies. The common approaches used are either surgical, medical or both.4 Medical management with hormone modulators like tamoxifen citrate, dydrogesterone and, medroxyprogesterone, either alone or post-operative can be used. But their results are variable and side effects cannot be overlooked.3,17
Surgery is the best treatment modality. Though the timing of surgery is debatable, consideration of the surgery should be made to eliminate the physical symptoms, and increase the post-operative potential. Mastectomy, usually subcutaneous with breast reconstruction, or breast reduction surgery with its modifications, free nipple graft, or pedicle-based technique, is generally preferred.4There is decreased chance of recurrence in mastectomy than in reduction mammoplasty, indicating complete removal of breast tissue.4 However, in adolescents, psychological consequences after the procedure cannot be overlooked.
Free nipple graft reduction mammoplasty can be done in selective patients. Patients who need large volume reduction (resected tissue greater than two kgs on each side), as in our case, or sternal notch to nipple distance greater than 40 cm, is generally advocated for breast reduction surgery with free nipple graft technique.18Additionally, elderly patients, obesed patients with gigantomasita, smokers, patients with peripheral vascular disease, or any systemic disease with impaired healing are also advised for reduction mammoplasty with free nipple graft.18 Pedicle-based technique utilizes long fold of pedicle and can compromise the blood supply during large volume resection and lead to increased risk of nipple necrosis again favoring free nipple graft technique.14,18,19Similarly, studies have shown pedicle based technique has increased complications than free nipple graft.19 Also, breast reduction surgery with free nipple graft technique has decreased the chance of recurrence.6,20 And can be done rapidly with minimum blood loss and skin undermining.
Though free nipple graft reduction mammoplasty is preferred in massive gigantomastia (mass resection greater than two Kgs per side), it has the disadvantage of resulting in the loss of lactation, variable return of sensation and contractility of the nipple, graft failure, and at times pigmentary changes of the nipple-areola complex.18,21,22 With careful deliberation in the department, proper counseling of the patient and her family members and adhering to the limited evidence; bilateral breast reduction surgery with free nipple-areola graft was performed in our case.