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.