MAMMARY GLAND AND ITS’ BIRTH

ANOMALIES

By: Visith Dantanarayana of Group 201

Introduction

Mammary glands are one of the main appendages of the skin. In fact they are modified sweat glands . Frankly both men and

women have mammary glands or breasts, but the male mammary glands don’t develop due to the high concentration of

testosterone in the blood during pubertal life.

The breasts are the most prominent structures of the anterior thoracic wall. They lie atop the Pectoralis major and minor

muscles, in the subcutaneous tissue. Although it is rudimentary and functionless in men, the mammary glands of a woman

secrete milk and are accessory glands of the female reproductive system. It generally consists of 18 to 20 glandular lobules

and a considerable amount of adipose tissue. In a way of speaking mammary glands have a broad conical shape with the

base located on the anterior thoracic wall and the apex pointing anteriorly away from the thoracic wall. The apex of the

mammary gland has the nipple, which also is the greatest prominence of the mammary gland. The nipple has a base and an

apex. Around the base of it in close proximity to it are a number of Montgomery glands which are sebaceous glands that

secrete a film of protective lipids. The nipple is surrounded by a circular, dark colored, pigmented area known as the

Areola.

The rounded contour of the mammary glands is due to the fat lobules. Embryonically the mammary glands originate partly

from the Mesoblast but also from the Epiblast.

The Mammary glands correct position on the anterior thoracic wall could be easily identified via the following landmarks;

between the 3rd and 6th ribs.

Next we will see about the further development of the female mammary gland.

Mammary gland development is characterized by the unique process by which the epithelium invades the stroma. The

development of the mammary gland occurs mainly after birth. During puberty, tubule formation is coupled with

branching morphogenesis which establishes the basic arboreal network of ducts emanating from the nipple.

Developmentally, mammary gland epithelium is constantly produced and maintained by rare epithelial cells, dubbed as

mammary progenitors which are ultimately thought to be derived from tissue-resident stem cells.

Embryonic mammary gland development can be divided into a series of specific stages. Initially, the formation of the milk

lines that run between the fore and hind limbs bilaterally on each side of the midline occurs around embryonic day 10.5

(E10.5). The second stage occurs at E11.5 when placode formation begins along the mammary milk line. This will

eventually give rise to the nipple. Lastly, the third stage occurs at E12.5 and involves the invagination of cells within

the placode into the mesenchyme, leading to a mammary anlage (biology).

The primitive (stem) cells are detected in embryo and their numbers increase steadily during development .

Pre-pubertal mammary gland development

Postnatally, the mammary ducts elongate into the mammary fat pad. Then, starting around four weeks of age, mammary

ductal growth increases significantly with the ducts invading towards the lymph node. Terminal end buds, the highly

proliferative structures found at the tips of the invading ducts, expand and increase greatly during this stage. This

developmental period is characterized by the emergence of the terminal end buds and lasts until an age of about 7–8 weeks.

Pubertal mammary gland

By the pubertal stage, the mammary ducts have invaded to the end of the mammary fat pad. At this point, the terminal end

buds become less proliferative and decrease in size. Side branches form from the primary ducts and begin to fill the

mammary fat pad. Ductal development decreases with the arrival of sexual maturity and undergoes estrous

cycles (proestrus, estrus, metestrus, and diestrus). As a result of estrous cycling, the mammary gland undergoes dynamic

changes where cells proliferate and then regress in an ordered fashion.

Pregnancy

During pregnancy, the ductal systems undergo rapid proliferation and form alveolar structures within the branches to be

used for milk production.

Lactation

After delivery, lactation occurs within the mammary gland; lactation involves the secretion of milk by the luminal cells in

the alveoli. Contraction of the myoepithelial cells surrounding the alveoli will cause the milk to be ejected through the ducts

and into the nipple for the suckling pups.

At this point I believe it is essential to speak about the role played by the humoral factors during the development of the

mammary glands. Development is caused by activation of estrogen receptor alpha, progesterone receptors , and prolactin

receptors contained in mammary cells. Estradiol, progesterone, and prolactin normally activate the respective receptors that

cause breast growth throughout stages of development. Some phytoestrogens, such as 8-prenylnaringenin, may activate

these estrogen receptors to stimulate mammary gland development. Estrogen receptor expression in the mammary gland is

controlled by the GATA-3 transcription factor. Now let’s discuss this profoundly.

The breasts are principally composed of adipose, glandular and connective tissues. Because these tissues have hormone

receptors, their sizes and volumes fluctuate according to the hormonal changes particular to thelarche (sprouting of

breasts), menstruation (egg production), pregnancy (reproduction), lactation (feeding of offspring), and menopause (end of

menstruation).

Estrogens cause elongated growth of mammary duct cells through activation of estrogen receptor alpha (ER-

α). Progesterone receptor (PR) activation, believed to be specific to progesterone receptor B (PRB), by Progestogens causes

growth of milk producing cells or side branching. Progestogens also cause stimulation of connective breast tissue

or cooper's ligaments. Density, Areolar gland development, and gland lactation development are caused

by prolactin activating the prolactin receptor (PR).

For example, during the menstrual cycle, the breasts are enlarged by premenstrual water retention and temporary

growth; during pregnancy, the breasts become enlarged and denser (firmer) because of the prolactin -caused

organ hypertrophy, which begins the production of breast milk, increases the size of the nipples, and darkens the skin color

of the nipple-areola complex; these changes continue during the lactation and the breastfeeding periods. Afterwards, the

breasts generally revert to their pre-pregnancy size, shape, and volume, yet might present stretch marks and breast ptosis.

Lactiferous duct development occurs in females in response to circulating hormones. First development is frequently seen

during pre- and postnatal stages, and later during puberty. Estrogen promotes branching differentiation, whereas in

males testosterone inhibits it. A mature duct tree reaching the limit of the fat pad of the mammary gland comes into being

by bifurcation of duct terminal end buds (TEB), secondary branches sprouting from primary ducts and proper duct lumen

formation. These processes are tightly modulated by components of mammary epithelial ECM interacting with systemic

hormones and local secreting factors. However, for each mechanism the epithelial cells' " niche" can be delicately unique

with different membrane receptor profiles and basement membrane thickness from specific branching area to area, so as to

regulate cell growth or differentiation sub-locally. Important players include beta-1 integrin, epidermal growth factor

receptor (EGFR), laminin-1/5, collagen-IV, matrix metalloproteinase(MMPs), heparan sulfate proteoglycans , and others.

Elevated circulating level of growth hormone and estrogen get to multipotent cap cells on TEB tips through a thin, leaky

layer of basement membrane. These hormones promote specific gene expression. Hence cap cells can differentiate

into myoepithelial and luminal (duct) epithelial cells, and the increased amount of activated MMPs can degrade

surrounding ECM helping duct buds to reach further in the fat pads. On the other hand, basement membrane along the

mature mammary ducts is thicker, with strong adhesion to epithelial cells via binding to integrin and non-integrin receptors.

When side branches develop, it is a much more “pushing-forward” working process including extending through

myoepithelial cells, degrading basement membrane and then invading into a periductal layer of fibrous stromal

tissue. Degraded basement membrane fragments (laminin-5) roles to lead the way of mammary epithelial cells

migration. Whereas, laminin-1 interacts with non-integrin receptor dystroglycan negatively regulates this side branching

process in case of cancer. These complex "Yin-yang" balancing crosstalks between mammary ECM and epithelial cells

"instruct" healthy mammary gland development until adult.

There is preliminary evidence that soybean intake mildly stimulates the breast glands in pre- and postmenopausal women.

At menopause, the breasts can decrease in size when the levels of circulating estrogen decline, followed by the withering of

the adipose tissue and the milk glands. Additional to such natural biochemical stimuli, the breasts can become enlarged

consequent to an adverse side effect of combined oral contraceptive pills ; and the size of the breasts can also increase and

decrease in response to the body weight fluctuations of the woman. Moreover, the physical changes occurred to the breasts

often are recorded in the stretch marks of the skin envelope; they can serve as historical indicators of the increments and the

decrements of the size and the volume of a woman's breasts throughout the course of her life.

In the above text you may have come across the Cooper’s Ligament. Well, let us see wh at it is:

Cooper's ligaments (also known as the suspensory ligaments of Cooper and the fibrocollagenous septa) are connective

tissue in the breast that help maintain structural integrity. They are named for Astley Cooper, who first described them in

1840. Their anatomy can be revealed using Transmission diffraction tomography.

Cooper's Suspensory Ligament should not be confused with the pectineal ligament (sometimes called the inguinal ligament

of Cooper) which shares the same eponym. Also, the intermediate fibers and/or the transverse part of the ulnar collateral

ligament are sometimes called Cooper's ligament(s). The ligaments run from the clavicle and the clavipectoral

fascia branching out through and around breast tissue to the dermis of the skin overlying the breast. The intact ligament

suspends the breast from the clavicle and the underlying deep fascia of the upper chest. This has the effect of supporting th e

breast in its normal position, and maintaining its normal shape. Without the internal support of this ligament, the breast

tissue (which is heavier than the surrounding fat) sags under its own weight, losing its normal shape and contour.

Something important about the relationship between the Cooper’s ligament and sagging

Many women have held the mistaken belief that sagging is caused by the failure of the Cooper's ligaments to support the

breast tissue. In fact, sagging is partly determined by genetic factors, but cigarette smoking, a woman's body mass index,

her number of pregnancies, the size of her breasts before pregnancy, and age are all influencing factors.

Many women also believe that wearing a brassiere will prevent their breasts from sagging later in life and that breasts

cannot anatomically support themselves. Bra manufacturers will only claim that bras only affect the shape of breasts while

they are being worn. A bra only provides support to women's breasts while they are wearing the bra.

Dr. Christine Haycock, a respected surgeon at the New Jersey Medical School and an expert in sports medicine, said that

"Cooper's ligaments have nothing to do with supporting breast tissue... They just serve to divide the breast into

compartments." She noted that most women's breasts begin to droop with age and that extremely large -breasted women are

generally more affected. However, sagging is not related to ligaments or dependent on breast size.

Pare away the fiction and fears, and the pros and cons of the bra come down to this: If a woman chooses to wear one

because it makes her feel good -- more supported, more under control or just prettier -- more power to her... Haycock

suggests that women let pain be their guide when deciding whether to wear a bra during exercise, and when choosing a

particular style.

Pathologically heavy breasts may cause pain in the woman's upper thoracic area, but this may be due to a poorly-fitting bra.

A number of reports state the 80–85% of women are wearing the wrong bra size. While large-breasted women may be

uncomfortable exercising without a bra, Dr. Haycock said that “It’s not doing any lasting damage to chest muscles or breast

tissue.” Her research found that small-breasted women, “those who

wore an A cup were frequently most comfortable with no bra at all."

In middle-aged women, breast ptosis is caused by a combination of

factors. If the woman has had children, postpartum hormonal changes

will cause the depleted milk glands to atrophy. Women who

experience multiple pregnancies repeatedly stretch the skin envelope

during engorgement while lactating. In addition, after the birth of

each child, the voluminous milk glands diminish in size, contributing

further to sagging. As a woman's breasts grow in size during repeated

pregnancies, the Cooper's ligaments that maintain the position of

the mammary glands against the chest are stretched and gradually

lose strength. Breast tissue and suspensory ligaments may also be

stretched if the woman is overweight or loses and gains weight.

On the right side is a diagram depicting Breast Ptosis or Breast

Swagging.

The Tail of Spence (Spence's tail, axillary process or axillary tail) is an extension of the tissue of the breast that extends

into the axilla.

It is named after the Scottish surgeon James Spence.

Breast Quadrants

For the Anatomical location and description of tumors, the surface of the breast is divided into four quadrants. This

classification is expressed like the areas on a dial:

The upper inner quadrant – from 12 to 3

The lower inner quadrant – 3 to 6

The lower outer quadrant – 6 to 9

The upper outer quadrant – 9 to 12

This division into quadrants is crucial when it comes to the lymphatic drainage of the mammary glands.

This can be expressed in a diagram to make it more vivid and

comprehensible.

Vasculature and innervation of the Mammary gland

1. The Arterial supply

The Arterial Blood supply, which brings both oxygen and

nutrients to the mammary gland is done by the following

arteries:

a) Medial Mammary branches of the perforating

branches and Anterior Intercostal branches of the Internal

Thoracic artery, originating from the Subclavian artery

b) Lateral Thoracic and Thoracoacromial arteries,

branches of the Axillary Artery.

c) Posterior Intercostal arteries, branches or the

Thoracic Aorta in the second, third and fourth intercostal

spaces.

2. The Venous Drainage

Forms a circle around the base of the nipple, which is

named as Haller’s Circle. From this circle, large tributaries

transmit blood to the circumference of the gland. The

venous blood is mainly drained into the following veins:

a) The Axillary Vein

b) Internal Thoracic Vein

Above is the Arterial supply of the Mammary Gland.

The diagram to the right depicts both the Arterial supply and the Venous Drainage.

The Lymphatic Drainage of the Mammary gland

The Lymphatic drainage of the mammary gland is very important because of its’ role in the metastasis of carcinomas. The

division of the breast into breast quadrants actually help us to comprehend this phenomenon better. Generally it can be

summarized as follows:

The Nipple, areola and lobules of the glands drain to the SUBAREOLAR LYMPHATIC PLEXUS.

The Lateral quadrants of the mammary glands (more than 75% of the lymph) drain to the AXILLARY LYMPH

NODES, initially to the PECTORAL (ANTERIOR) NODES for the mos t part, however, some lymph may drain

directly to the other Axillary nodes or even to the INTERPECTORAL, DELTOPECTORAL, THE

SUPRACLAVICULAR OR THE INFERIOR DEEP CERVICAL NODES.

The Medial quadrants drain to the PARASTERNAL NODES or to the opposite Breast.

The lymph from the lower quadrants pass deeply to the INFERIOR PHRENIC NODES.

The skin of the breast, except the nipple and areola drain into the AXILLARY, INFERIOR DEEP CERVICAL,

AND INFRACLAVICULAR NODES, and also into the PARASTERNAL LYMPH NODES of both sides.

Lymph from the Axillary nodes drain into the Supraclavicular and Infraclavicular Lymph nodes after which they drain into

the Subclavian Trunk, which also happens drain lymph from the entire upper limb.

Lymph from the Parasternal nodes enter the BRACHIOMEDIASTINAL TRUNK, which drains lymph from the Thoracic

viscera.

The termination of these Lymphatic trunks vary, but traditionally they merge with the JUGULAR TRUNK, which happens

to drain lymph from the head and neck and form a small RIGHT LYMPHATIC DUCT on the Right side, whereas on the

left side they both terminate in the THORACIC DUCT.

.

The above diagram depicts the lymph nodes.

The diagram onto the left also depicts the lymphatic drainage with their usual direction.

The study of the Lymphatic drainage of the mammary gland is by the way the most important portion of it. This is because

of the metastasis of carcinomas. A carcinoma is a cancerous development in the epithelium, and metastasis is whereby

small cellular portions of this carcinoma spreading to other parts of the body, thus, spreading the cancer, and when it comes

to breast cancer, this process of metastasis is likely to happen via the

Lymphatic vessels.

Metastasis via the lymphatic nodes is clearly depicted in the diagram on the

Right.

Breast cancer as we know occurs in five major stages. They are; o – stage, 1 – stage, 2 – stage, 3 – stage and the 4th stage.

Stage 0 is more or less Precancerous and in situ, therefore, its’ mortality rate is almost 0. Stages 1, 2 and 3 are malignant

but the carcinoma is located within the boundaries of the mammary gland and metastasis doesn’t take place. Thus, again the

mortality rate is about 35%. However, Stage 4 breast cancer is also known as invasive and highly metastatic, thus it spreads

rapidly, rising the mortality rate up to a stunning 90%. Early diagnosis of the malignant carcinoma would facilitate the

successful elimination of the danger. In some cases of breast cancer, a woman suffering from it also gets accustomed to

Ovarian Cancer. This is mainly because this metastasis of the carcinoma, but in this case the lymphatic drainage is strictly

involved. As we discussed earlier a little portion of lymph is drained into the lymph nodes in the Abdominal cavity, well,

these lymph nodes have easy access to the Inguinal Lymph nodes, which lies almost adjacent to the Ovarian Lymph nodes,

through which the carcinoma cells could infiltrate the Ovaries. This metastasis is easily comprehendible with the diagram

given at the bottom.

This graph depicts some statistics that were obtained throughout the year of 2013.

Now let’s take a look at the breast glands of other mammals. Firstly, I think it’s essential to mention that the presence of

mammary glands to feed the young is a unique characteristic of mammals.

MAMMAL ANTERIOR

(THORACIC)

INTERMEDIATE

(ABDOMINAL)

POSTERIOR

(INGUINAL)

TOTAL

GOAT, SHEEP, HORSE AND

GUINEA PIG

0 0 2 2

CAT 2 2 4 8

DOG 4 2 2 OR 4 8 OR 10

RAT 6 2 4 12

PIG 6 6 6 18

MOUSE 6 0 4 10

PROBOSCIDEANS AND PRIMATES

2 0 0 2

According to the above table, most of the mammals have more than two mammary glands, here the word “most” implies

the smaller mammals because the large mammals; such as the horse, have only two but mostly in the Inguinal region.

The reason for this phenomenon could be expressed as a result of evolution. Theoretically one mammary gland is for one

baby. A mammal having, for instance 4 mammary glands would depict that it would give birth to four infants at a time.

Now, considering a pig, which has 18 mammary glands implies that a swine would give birth to 18 piglets at a time, thus

the necessity for 18 mammary glands is evident.

However, humans are supposed to be mammals who occupy the highest position in the mammalian hierarchy. A woman

would under usual circumstances give birth to only two babies at most. Therefore on ly two mammary glands would be

necessary.

Next we shall discuss about certain birth anomalies of the human mammary glands.

Birth anomalies of the human mammary glands can be categorized as follows:

Polymastia and Polythelia

Gynecomastia

Congenital breast Hypoplasia or Aplasia, which can be divided further:

Poland’s Syndrome, Idiopathic breast asymmetry, Tubular breast and Amastia

Poland’s Syndrome

Poland syndrome (also Poland's syndrome, Poland's syndactyly, Poland sequence, and Poland's anomaly) is a rare birth

defect characterized by underdevelopment or absence of the chest muscle (Pectoralis) on one side of the body, and usually

also webbing of the fingers (cutaneous syndactyly) of the hand on the same side (the ipsilateral hand). Poland syndrome

most often affects the right side of the body, and occurs more often in males than in females.

It is usually considered a unilateral condition. Some have claimed that the term can be applied in bilateral presentation, but

others recommend using alternate terminology in those cases. In the above paragraph I mentioned about the Pectoralis

muscles, well, usually the absence of the sternal head of the Pectoralis major muscle is the most ap propriate diagnosis of

the syndrome. It was first named in 1962 by Patrick Clarkson, a New Zealand-born British plastic surgeon working at Guy's

Hospital and Queen Mary's Hospital, London. He noticed that three of his patients had both a hand deformity and an

underdeveloped breast on the same side. He discussed this with his colleague at Guy's Hospital, Dr. Philip Evans, who

agreed that the syndrome was "not widely appreciated". Clarkson found a reference to a similar deformity published

by Alfred Poland, over a hundred years earlier in Guy's Hospital reports, in 1841. Clarkson was able to find the hand

specimen dissected by Poland, which was still held in the hospital pathology museum. Poland had dissected a convict called

George Elt, who was said to be unable to draw his hand across his chest. Poland noted the chest wall deformity, and this

was illustrated in his article; the hand was also dissected and preserved for posterity in Guy's Hospital museum where it

remains today. It cannot be truly said that Poland described this syndrome because he only described one isolated case.

Clarkson published his series of three cases and named the syndrome after Poland in his article.

Depicted on the right is a photograph of Dr. Alfred Poland.

The cause of Poland syndrome is unknown. However, an interruption of the embryonic blood supply to the arteries that lie

under the collarbone (subclavian arteries) at about the 46th day of embryonic development is the prevailing theory.

Poland’s Syndrome is known to be the most frequent cause of breast hypoplasia. As mentioned earlier in this text it is a

unilateral incident which has high prevalence of occurring in the right than in the left in about 3:1. The reason for this

however, is unknown.

The Poland’s Syndrome is also sporadic and occurs 1:20,000 – 30,000 births. The treatment for this syndrome is fairly

limited and the only option available is reconstructive surgery which could be done via two distinct methods.

Thoracic Wall Reconstruction

The goal of thoracic wall reconstruction is to provide a stable bony platform. Few patients present a significant defect of t he

rib cage. If the defect is large and the chest wall unstable, split rib grafts are inserted to bridge the gap. The anterior skin

flap may be adherent to the pleura; caution is required during the dissection to avoid inadvertent thoracic penetration. In the

majority of cases, rib flattening or single space defects do not require bone grafts. Camouflage may be obtained with a

custom-made prosthesis, particularly in cases where a muscle transfer is necessary to recreate the anterior axillary fold and

good coverage of the prosthesis is provided. Insertion of prosthesis under a skin flap with little subcutaneous tissue has an

unnatural appearance and a higher complication rate such as displacement, late seroma, discomfort, and extrusion. Contour

restoration of the chest can also be obtained with de-epithelialized dermal-adipose flaps or with lipo-filling without muscle

transfer if the anterior axillary fold is present. Rib cartilages are often more prominent on the affected side, but reshapin g is

seldom necessary, particularly in women; when a breast augmentation is done, the deformation becomes less noticeable.

Pectoralis Muscle Reconstruction

The decision to transfer a functioning muscle to replace the missing Pectoralis muscle depends on the presence or absence

of the anterior axillary fold. When the superior portion of the Pectoralis muscle and axillary fold are present, soft tissue

augmentation alone is necessary and it can be provided with a muscle, myocutaneous, cutaneous, or perforator flap, with fat

grafting or with prosthesis, depending on the quality of the overlying skin flap and the amount of breast tissue present. The

decision to harvest the ipsilateral latissimus dorsi muscle for volume only has to be weighed against the possible functional

loss particularly in athletes.

When the axillary fold is missing, a dynamic muscle transfer is needed. The ipsilateral latissimus dorsi muscle is the

preferred donor. It is transposed as a pedicled flap on the thoracodorsal vessels and nerve to the anterior ches t and

reinserted on the humerus. The donor site deformity is minimal because the posterior axillary fold is preserved with the

tendon of the teres major. A midlateral thoracic incision is used to harvest the latissimus and elevate the anterior skin flap

for muscle insertion. Another short incision is made on the inner arm to reattach the tendon on the humerus with a bone

anchor device. The latissimus muscle is larger and thinner than the Pectoralis muscle so it is folded on itself to provide

more bulk, particularly in the subclavicular area and laterally at the level of the axillary fold. Ohjimi also suggests twisting

the latissimus on itself to augment the anterior axillary fold. In cases of severe depression in the subclavicular area, a de -

epithelialized skin paddle can be transferred with the muscle to increase the thickness . The arm is kept adducted for 6

weeks postoperatively. Shorter incisions have been reported with endoscopic surgery, but it makes the procedure more

technically challenging. Free innervated muscle transfers are needed in cases of absent ipsilateral latissimus. Alternatives

are the contralateral latissimus or free transverse myocutaneous gracilis flap, which can provide more soft tissue bulk.

This mammogram shows the absence of the Pectoralis major muscle on the left and the normal breast on the right.

Idiopathic Asymmetry

The initiation of thelarche may occur on one side and proceed at a faster rate for unknown reasons. In mos t

Cases, both breasts become relatively equal in volume by the end of puberty. A small degree of breast asymmetry is not

uncommon or abnormal; however, a marked inequality of breast volume can be noticeable. Houston noted that patients

experience difficulty in concealing asymmetry greater than 33%, with everyday attire. Idiopathic breast asymmetry is

classifiable into six categories: unilateral hypoplasia, asymmetrical hypoplasia, unilateral hyperplasia, asymmetric

hyperplasia, hyperplasia/hypoplasia and hypoplasia associated with chest wall deformities. Unilateral hypoplasia is most

common and may vary from the minimal idiopathic form to severe Poland syndrome. Associated with breast hypoplasia is

a small and cephalad-displaced NAC, and in rare instances, the NAC is absent. Etiologies of breast asymmetry have been

described, including differential end organ response to hormonal stimulation during development, tumors, medications and

iatrogenic causes, including operations, radiation and trauma.

Breast asymmetry may cause physical discomfort as well as psychiatric embarrassment. Early surgical correction may be

warranted. Postponing corrective surgery for adolescents with significant asymmetry may be psychologically detrimental

and unnecessary. Plastic surgeons use reconstructive techniques to create improved symmetry. Hypoplastic breasts are

augmented and may require tissue expansion as a first stage. Tissue expansion preceding placement of a permanent implant

allows descent and expansion of the Hypoplastic breast and NAC, as well as expansion of the deficient soft-tissue envelope.

Particularly in young patients, the expansion process may take place over years until the opposite, unaffected breast reaches

maturity. In more severe cases of hypoplasia, the latissimus may be transposed over top of the implant to improve contour

and decrease risk of contracture. Correction or camouflage of underlying chest wall deformities may be necessary. The

unaffected breast may have ptosis requiring a mastopexy to achieve improved symmetry.

The main cause for Idiopathic Asymmetry is usually unknown, but it definitely is a birth anomaly. One of the most reliable

reasons for Idiopathic Asymmetry is the under-development of the Cooper’s ligament, which holds the key for suspending

the gland in the subcutaneous tissue, firmly attached to it.

Apart from the general information given above, one more fact must be noted here. Idiopathic Asymmetry can also be

classified into six categories:

Unilateral Hypoplasia

Asymmetrical Hypoplasia

Unilateral Hyperplasia

Asymmetrical Hyperplasia

Hyperplasia / Hypoplasia

Hypoplasia associated with chest wall deformities

Amastia

Amastia is a situation where the total absence of the mammary gland/ glands is seen. It could be unilateral or bilateral. This

is due to the failed development of the mammary ridges or the total absence of them. However, it has another variant

known as Athelia, where the total absence of nipples is seen.

The absence of a glandular breast tissue is a very rare occurrence, thus the current estimation is that it s’ occurrence could be

narrowed down to 7 among 30,000 or 40,000 births . In 1939, Froriep first reported a case of Amastia.

According to Lin and Nguyen, there are different presentations of Amastia and each can be attributed to different

underlying pathologies. Ectodermal defects may cause bilateral absence of breasts in males and females and is related to the

failure of development of the ectodermal layer and its appendages. Bilateral Amastia may be an isolated occurrence or be

associated with other anomalies of the palate and upper extremities. Unilateral Amastia may be a variant of Poland

syndrome and should be treated accordingly. Associated abnormalities include cleft palate, hypertelorism, anomalous

pectoral muscles, upper limb deformities and abnormalities of the genitourinary tract. In most cases Amastia is associated

with Dominant autosomal hereditary diseases for instance; Ectodermal Dysplasia and most importantly Mayer –

Rokitansky – Kuster – Hauser syndrome which is usually accompanied by severe Vaginal and Uterine agenesis.

Treatment of Amastia and Athelia may follow the principles of postoncologic breast reconstruction with special attention

paid to the placement of the inframammary fold and nipple. Tissue expansion with autologous and/or prosthetic breast

reconstruction techniques is available and should be chosen based on patient and surgeon preference. Autologous

reconstruction with abdominal, dorsal, or gluteal tissues may be chosen based on body habitus and patient preference.

However, in the pediatric population the gluteal tissue transfers may be preferable due to the general availability of soft

tissue and the less conspicuous scarring. Abdominal wall weakening and the frequent unavailability of adequate soft tissue

make the abdomen a less-appealing donor site in adolescents and young adults as compared with the more mature adults

seen with breast cancer. Prosthetic reconstruction with tissue expanders and permanent implants follows the same

principles as adult reconstruction and can be employed at the surgeon's discretion.

Tubular or Tuberous Breast

Tuberous breast is a term first coined by Rees and Aston. The breast has normal function but abnormal morphology. In this

case we could describe it as a hypoplastic breast with a constricted ring around its’ base, herniation of glandular tissue in to

the areola is also seen. Deficient skin envelope and inframammary fold malposition is also seen. With the narrowed

transverse breast diameter and base constriction, the breast appears to herniate into an oversized and protuberant areola.

Hence, it has another name: Snoopy nosed Syndrome.

The condition may be unilateral or bilateral, the exact cause is unknown, but one prevailing hypothesis is that Cooper’s

Ligament malformation around the nipple region would sharp-shoot it, but so many other reasons also may be involved.

Patients consult with plastic surgeons to correct their deformity. Treatment objectives include expanding the base

circumference and the skin of the lower hemisphere, releasing constricting skin tightness at the areolar junction, lowering

the inframammary fold, increasing breast volume and height and decreasing areolar diameter. A periareolar approach

allows alteration of the areolar diameter and division and widening of breast tissue to increase breast base diameter. A

tissue expander or implant under the divided breast tissue assists in improving deficient breast volume.

Above on the left is the diagram of a breast with Snoopy nosed Syndrome and on the right is the diagram of a normal

breast.

Polymastia and Polythelia

It’s also known as supernumerary nipples and breasts. First we will discuss briefly about Polythelia.

Polythelia is the presence of an extra nipple or many of them. This is quite a common breast anomaly . However, the

occurrence of these supernumerary nipples take place along a particular line, which is known as the Milk line, whic h

extends from the Axilla across the normal nipple to the center of the inguinal ligament. The significance of this Milk Line is

important for the correct comprehension of the anomaly. The Milk Line is paired and it extends across the Thoracic,

Abdominal and the Inguinal regions. The occurrence of Mammary glands in other mammals as well as in humans takes

place along this line. However, in the case of humans only the thoracic section is occupied for this purpose as a woman

usually gives birth to two babies at a time. Therefore, the occurrence of supernumerary nipples could imply a genetic

mutation whereby it demonstrates a trait that we passed a long time ago in the road of Evolution.

The milk lines are depicted in the diagram on the left.

Polythelia could commonly occur in both men and women and it could be either unilateral or bilateral but in most cases it is

bilateral. In men the supernumerary nipple could be mistaken for a mole and neglected but it is harmless and it won’t create

much of a social distress for that person.

However, if the supernumerary nipple appeared in a woman it could cause significant discomfort during weaning and

lactation. In this case a woman could consult a cosmetic surgeon and have the

supernumerary nipples amputated.

The occurrence of Polymastia is more or less similar to Polythelia except for

the fact that it is more common in women than in men.

Polymastia has an even lower incidence than Polythelia, and also occurs

along the embryonic milk line. Like Polythelia, it is usually sporadic, but can

have familial inheritance. Unlike Polythelia, Polymastia can be associated

with other congenital anomalies, particularly thoracic and renal.

Treatment of Polymastia is variable and depends on the size and location of

the supernumerary breast gland and nipple. Simple mastectomy is the option

of choice in patients who present with a third distinct breast mound; however,

disruption of the inframammary fold and soft tissue envelope of the

remaining breast should be prevented when possible. In cases where the

accessory breast is adjoined to the native breast, tissue-sparing techniques

with skin de-epithelialization and accessory nipple excision can usually

restore the mound to a normal appearance and location.

Corrective surgery for Polymastia should be performed when breast

development is complete and final breast tissue volumes have been achieved.

It is often difficult to predict the form and position of the accessory breast

mound and nipple and early excision may ultimately compromise the

eventual outcome.

Cases of Polymastia are depicted here along with its’ treatment.

Gynecomastia

Gynecomastia is derived from the Greek terms gynec (feminine) and mastos(breast). The literal translation, male breasts,

relates to any condition that results in excessive development of breast tissue in males. Males may rarely develop breast

cancer, but this is often associated with testicular atrophy.

Male breast volume is composed of a combination of ductal and stromal tissue, commonly referred to as glandular tissue,

and an increase in adipocygland cells and fat cells. Some patients may have a lot of lose skin and hanging of the breasts

due to that.

The first recorded description of a reduction mammaplasty was by Paulas of Aegina in the seventh century AD, who

referred to the condition as an "effeminacy of men." Several medical and surgical treatment s of gynecomastia were

described in the 1800s.

Gynecomastia results in an increase in breast tissue in males that, when problematic, is readily detectable by other

individuals. The increased tissue may be breast glandular tissue, adipose (fatty) in nature, or a combination of the two. This

results in significant functional and psychological limitations. The physical deformation may also be exquisitely painful. As

a general rule, the glandular tissue is significantly more painful than the fatty tissue. Situa tions like gym class may require

children or adolescents to remove their shirts in the presence of other students. This can put a boy with gynecomastia in

danger not only of embarrassment but also of physical harm.

Most patients have never heard of this condition until the family physician identifies it. The physician may be unaware of

the possible causes of the condition and its psychological impact. After initial presentation, boys are frequently advised to

ignore the gynecomastia and are told that it will go away. Fortunately, in most instances, cases of minimal subareolar

pubertal-onset gynecomastia do regress as puberty progresses.

Individuals with no regression or even progression of the deformity often receive little or no understanding about the shame

and humiliation they experience. Coaches, sergeants, physicians, parents, and peers (both boys and girls) can inflict damage

out of ignorance, cruelty, or both. Postural and clothing modifications to mask the deformity are the norm in these patients

from puberty through adulthood. For example some patients may permanently slouch to hide the gynecomastia and will

avoid wearing T shirts.

Gynecomastia can occur in persons of any age. During adolescence, males develop firmness around the breast as the breast

bud enlarges due to the hormonal fluxes of puberty. The subareolar firmness which normally develops regresses with time.

Breast tissue is typically present on a microscopic level in male patients; a small amount of breast tissue is normal. The

visible appearance of breast tissue in a male is abnormal. Breast tissue increases with Animal food intake. Dairy, Meat Fish

and Eggs increase the estrogen levels of the body. The effect increases with ageing.

Nydick et al reported 65% of boys "may have the problem" but cautioned that it typically resolves. Webster noted the

incidence of gynecomastia to be around 8% in a series of naval patients, while Williams noted that 40% of men examined in

his series of autopsies had gynecomastia to some degree. Approximately 40% of healthy men and up to 70% of hospitalized

men have palpable if not visible breast tissue. The incidence of some degree of palpable breast tissue in males increases to

more than 60% in those in the seventh decade of life in one series.

Physiologic gynecomastia occurs primarily in newborns and in adolescents at puberty. In t he newborn, the neonatal breast

results from the action of maternal estrogens, placental estrogens, or both in concert. The increased breast tissue usually

disappears in a few weeks. Neonatal gynecomastia is not a problem that requires surgical interventio n.

Adolescent gynecomastia, by definition, is initiated during puberty. The median age of onset is 13 years. Breast tissue

growth is often asymmetrical, and the breasts are frequently tender. Adolescent gynecomastia usually regresses by the latter

teen years. Note that the normal course during puberty is for a palpable, often visible, mass below the areola that begins to

resolve in the mid teen years. While continued visible enlargement in the size of the breast is not normal in a teenager,

residual palpable gynecomastia may be present in one or both breasts through the mid teen years. The authors would stress

that the norm would be progressive diminution of any visible or palpable deformity through this period. In each case, the

clinician must evaluate the degree of tissue present, the clinical presentation, and the physical and psychological effects on

the patient.

Pathologic gynecomastia may be due to testosterone deficiency, increased estrogen production, or increased conversion of

androgens to estrogens. The pathological conditions associated with gynecomastia include congenital anorchia,Klinefelter

syndrome, testicular feminization, hermaphroditism, adrenal tumors, liver disorders, pituitary tumors, and malnutrition.

Many pharmacological agents have been linked to gynecomastia. These drugs can be categorized by their mechanisms of

action. The first type is drugs that act exactly like estrogens (eg, diethylstilbestrol, birth control pills, digita lis, estrogen-

containing cosmetics). The second type is drugs that enhance endogenous estrogen formation (eg, gonadotropins,

progesterone, clomiphene). The third type is drugs that inhibit testosterone synthesis and action (eg, ketoconazole,

metronidazole, and cimetidine). The final type is drugs that act by unknown mechanisms (eg, isoniazid, [6] methyldopa,

captopril, tricyclic antidepressants, diazepam, marijuana, heroin). While heavy marijuana u se has been linked to

gynecomastia in rats, the relationship in humans is at best poorly documented. Chronic alcohol abuse may result in

hepatocellular destruction and scarring which may result in gynecomastia. Adult patients should be routinely questioned

about alcohol abuse or addiction.

A link between testicular atrophy, Klinefelter syndrome, and breast cancer has been noted. Longstanding, stable

gynecomastia in an otherwise healthy male does not require an extensive medical workup.

In boys, the main sex hormone is testosterone, which is secreted by the testes. In girls, the main sex hormone is estrogen,

which is secreted by the ovaries. However, both hormones are secreted in both sexes. Some production of estrogen occurs

in the testes, and some production of testosterone occurs in the ovaries. Gynecomastia has long been considered the result

of an imbalance between estrogens, which stimulate breast tissue, and androgens, which antagonize this effect. An

alteration in the normal ratio of estrogen to androgen has been found in patients with gynecomastia in association due to

many different reasons.

Estradiol is the growth hormone of the breast in women, and an excess of estradiol leads to the proliferation of breast tissu e.

Under normal circumstances, most estradiol in men is derived from the peripheral conversion of testosterone and adrenal

estrogen. The basic mechanisms of physiologic gynecomastia have been postulated to represent a decrease in androgen

production, an absolute increase in estrogen production, and an increased availability of estrogen precursors for peripheral

conversion to estradiol.

Gynecomastia has 3 recognized pathological patterns, as follows:

The first type, the florid type, is characterized by an increase in the number and length of du cts, proliferation of

ductal epithelium, periductal edema, a highly cellular fibroblastic stroma and hypervascularity, and the formation

of pseudolobules. The florid type is the most common in patients with gynecomastia of less than 4 months'

duration.

The second type, the fibrous type, is characterized by dilated ducts with minimal proliferation of epithelium, an

absence of periductal edema, and an almost acellular fibrous stroma without adipose tissue. The fibrous type is the

most common in patients with gynecomastia that lasts for 4-12 months.

The third type, the intermediate type, is an overlapping pattern of both the florid and the fibrous types.

Surgical Therapy

The objectives of surgical management for breast gynecomastia are (1) to restore the normal male breast contour and (2) to

correct deformity of the breast, nipple, or areola. The surgical options for the patient with gynecomastia are mastectomy,

liposuction-assisted mastectomy, or a

combination of the 2 approaches. Most patients

receive maximal benefit from a combined

approach.

The diagram onto the left demonstrates a man

with gynecomastia.