Congenital Anomalies of the Breast: Flashcards
pediatric breast disorders may be categorized into hypoplastic, hyperplastic, and deformational
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Key moves in the correction of tuberous breast deformity
periareolar approach to the breast, with radial scoring of
constriction bands to the deep dermal plane as necessary,
as well as partial subpectoral implant placement and circumareolar reduction of the areola and herniated breast tissue.
The breast is a modified apocrine sweat gland that commences development at around 6 weeks of life
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Portions atrophy, except at the fourth intercostal space, which set the foundation for the primary mammary bud.
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The
lactiferous ducts open into the surrounding ectoderm, which develops into the areola at 3 months
F at 5 months
By 6 months of gestational age, the
basic framework and tubular architecture of the breast can be seen
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With circulating estrogen and progesterone from the placenta, the
lactiferous tissue continues branching into 2 years of life
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The normal
gland remains quiescent from 2 years until puberty
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At birth, the neonatal mammary tissue is functional
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Seventy
percent may secrete colostrum because of rise in estrogen
F Seventy
percent may secrete colostrum because of a rise in prolactin.
The nipples inverted after birth
F The nipples evert soon after birth because of proliferation of the underlying
mesoderm
Inverted nipples that remain until puberty is
not uncommon
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stages as described by Tanner
Tanner stage I, the breast is prepubertal, without appreciable breast parenchyma and slight nipple elevation.
Tanner’s stage 2 begins with thelarche, around 9.7 years, as the nipple-areolar complex (NAC) widens and the breast and nipple become a small mound.
Stage 3 is heralded with a further enlargement, as the breast extends beyond the borders of the areola
in stage 4 The NAC elevates above the breast contour as a secondary mound
In stage 5, the breast achieves the final maturesize and form
Amazia, the absence of
the breast without nipple absence, may occur unilaterally or bilaterally, in isolation or in conjunction with pectoralis hypoplasia
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Amastia is the complete absence of
the breast unit, including the gland and nipple
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athelia often occurs in conjunction with amazia.
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Accordingly, Trier amastia and its suspected mode of inheritance into three groups:
bilateral absence of the breast associated with congenital ectodermal
defects, unilateral absence of the breast, and bilateral absence of the
breast
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In congenital ectodermal defect is there is another abnormality rather than the breast?
a sex-linked recessive disorder, additional abnormalities with the skin and its appendages, the
teeth, and nails are present
Unilateral absence of the breast, when
combined with pectoralis aplasia or hypoplasia, is considered a variant of Poland syndrome
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Implants,
mucocutaneous flaps, and autologous fat transfer are viable methods
of reconstruction of breast hypoplasia
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Careful consideration of familial breast cancer history is mandatory when employing fat transfer techniques.
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tuberous breast is characterized by
■ Constricted skin envelope in the vertical and horizontal
dimensions
■ Deficiency in the base diameter (breast footprint)
■ Elevated inframammary fold (IMF)
■ Short nipple-to-IMF distance (high, tightfold)
■ Herniation of the breast parenchyma through the areola resulting
in enlarged diameter of the NAC. NAC involvement is present in
about 50% of cases8
■ Parenchymal hypoplasia
■ Asymmetry
Grolleau classification scheme based on the initial work by Von Heimburg
Type I Deformity: Only the medial quadrant is absent, the lower
medial edge is shaped like an italic S, and the lateral breast is larger in comparison.
Type II Deformity: Both lower quadrants are deficient, the areola
points down, and the lower pole is constricted.
Type III Deformity: All quadrants are deficient, and the breast base is
constricted both horizontally and vertically, and the breast is
shaped like a tubercle
the cause of lower pole constriction
that anomalies in the superficial fascia in the lower pole of the breast
lead to strong adherence between the dermis and the muscular
plane.
condensation of fascia may be caused by the joining of the deep and outer layers of the
superficial fascia, which collectively envelope the breast, at a higher
level, or a thickening ofpenetrating suspensory ligaments in the area.
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73% of females
presenting forbreast augmentation were found to have tuberous breast
deformity,
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Ninety-eight percent ofthe patients with tuberous breasts
were asymmetrical in size as well
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milder forms are commonly underappreciated and underdiagnosed
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when the soft tissues are distensible and the size goals are attainable, correction can be performed
in a single stage
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Two-stage procedures with expanders are reserved
for the most severe cases when the lower pole skin is severely deficient, the base/IMF is severely constricted, and/or the patient desires
a fuller size.
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All the patients in the tuberous breast required expansion
8% of the total or 30% of
Groleau type III tuberous breast required expansion
For adequate release, bands at the base of the breast
parenchyma and along the IMF are radially dissected from deep to
the superficial until the deep dermis to the point visual and palpable
release of the lower pole
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Dinner and Dowden depend on parenchymal release only for the tuberous breast
F Dinner and Dowden advocated full-thickness skin and
glandular incisions with transposition flaps, as they considered the
the skin itself to be constricting
Puckett and Concannon15 as well as Riberio et al. 16 described horizontal transection of the gland with folded-down internal flaps based on the
subareolar tissue or posterior chest wall, respectively, to reconstitute
the lower pole.
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Mandrekas and Zambacos utilized a periareolar incision to exteriorize the lower half of the breast
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Abbate, Fan, and Nahabedian described a new technique using a central
mound mastopexy via an inverted-T incision
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The parenchymal herniation
through the NAC was managed by de-epithelizing the central mound
followed by imbrication sutures to create a tight dermal base and
minimize the risk for future herniation
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Volume correction is alawys required because of the prevalence of
asymmetries
F Volume correction is often required because of the prevalence of
asymmetries
The use of anatomical implants for tuberous breast can be considered to increase the volume in the lower pole
and for tailoring of height, width, and projection
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In patients with severe tuberous breasts, correction of the IMF is mandated because of a short nipple-to-IMF distance.
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In such situations, effacement of the existing IMF is necessary to avoid the double-bubble deformity by lysing fascia! connections
between the dermis and superficial fascia.
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Location of the new IMF in the tuberous breast
by Tebbett based on implant volume,Mallucci and Brandford ICE principle
contralateral IMF or
the ridges made by the patient’s ideal bra to roughly determine where
the IMF will sit
some
authors remove excess gland from the deepest aspect of the subareolar
gland to keep the implant size similar and prevent future herniation
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subglandular implants have been associated with increased rates of capsular
contracture with visible implant margins and rippling in patients
because of a paucity of parenchyma
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In patients with a tuberous breast deformity, a dual-plane
III, which is defined as prepectoral undermining to the lower edge
of the NAC
F In patients with a tuberous breast deformity, a dual-plane
III, which is defined as prepectoral undermining to the upper edge
of the NAC
When a patient presents with severe ptosis and a less
severe tuberous deformity, a circumvertical mastopexy can be performed as needed using a tailor-tack approach
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Poland syndrome are always is unilateral
F Generally, cases are unilateral, but bilateral anomalies have been
described
Female more than male
F . Sporadic cases are hallmarked by 2 to 3: I higher incidence
in males, with 60% to 75% affecting the right side
Familial cases have equal incidence in sexes and laterality
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Classically,
Poland syndrome was defined as the absences of the pectoralis
major and ipsilateral hand abnormality
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The percentage of poland syndrome in breast hypoplasia
Poland syndrome is involved in 14% of
breast aplasia.
In the mild form, structural abnormalities may only
be appreciated radiographically.
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The mild variant of Poland syndrome is more common than the
classic full presentation
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The moderate variant of Poland syndrome represents the classic
form
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The moderate variant is characterized by
hypoplasia of breast parenchyma, high
IMF, and an underdeveloped and superiorly displaced NAC, with
the absence of the anterior axillary fold
The severe variant of Poland
syndrome represents the most challenging to reconstruct and is
characterized with a marked deformity of the chest wall with tight
chest skin and axillary webbing
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CT scans to evaluate the structural abnormalities.
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prominent posterior fold was evident
on clinical examination why?
compensatory hypertrophy of the teres
major that simulated the normal contraction of the latissimus dorsi
muscle
The frequency of hand abnormalities with Poland syndrome is
13.5% to 56%
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30% of syndactyly is a result of Poland syndrome.
F 10% of syndactyly is a result of Poland syndrome.
Perecentage of Aplasia of the ribs and cartilage
involving rib segments two to five often leads to severe chest depression
11%-25%
Percentage of lung hernation
Eight percent of patients may have
lung herniation.
Dextrocardia is present in 5.6% ofpatients, which
rises to 9.6% when the deformity was on the left side.
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Poland syndrome can be associated with Mobius and KlippelFeil syndrome
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Renal ultrasounds are recommended for all patients with aplasia of the pectoralis why?
Because of acro-pectoral-renal defect, an association
with aplasia of the pectoralis major exists with renal anomalies (e.g.,
unilateral renal agenesis or duplication of the urinary collecting system)
Certain cancers are known to exist with Poland
syndrome
T leukemia, non-Hodgkin lymphoma, cervical cancer, leiomyosarcoma, and lung cancer.
Breast cancer cannot develop in poland patients
F Despite being hypoplastic, breast cancer
has been reported to occur in patients with Poland syndrome and
therefore standard monitoring is required
We cannot reconstruct the tuberous breast with a defect in the chest wall
When residual chest wall
defects persist, custom silicone implants can be considered.
simple chest deformity included using the latissimus dorsi muscle
to contour the chest wall followed by placement of a thin mammary
prosthesis to correct the subclavicular hollow and to provide a more
tolerable feel and appearance.
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The use oflipofilling to correct contour abnormalities has become a
cornerstone in treatment of mild Poland deformity
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Fat grafting
has made the correction of mild to moderate contour abnormalities,
such as improvement of the subclavicular hollow and absence of the
anterior axillary fold,
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Despite using lipo fulling we should use custom implant
F Lipofilling can
also, be used to camouflage chest abnormalities and may obviate the
need for custom silicone devices
Lipfulling can be used at the onset of puberity
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Transfers may be staged at 4- to 6-month intervals to simulate natural breast growth
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LIip fulling is contraindication in breast cancer
Fat grafting techniques have also been
used in patients with a history of breast cancer with long-term follow-up demonstrating oncologic safety
when i should use breast expander in poland?
When there is a
soft-tissue deficiency and the IMF is high and tight, placement of tissue
expanders can be beneficial
The expansion of the breast lower pole may
bring down a superiorly malpositioned NAC
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ADM can be used in Poland syndrome
F no descriptions of the use of ADM to secure the implant and to
reduce the incidence of capsular contracture in Poland syndrome.
The pedicled latissimus dorsi flap in combination with a prosthetic
device has been the most common form of reconstruction in women
with Poland syndrome
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reported a 0% revision
rate of patients treated with this technique.
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latissimus dorsi flap facilitated the creation of an anterior axillary fold
and also camouflaged the subclavicular hollow.
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The latissimus muscle
may be reinserted anteriorly on the humerus, simulating the pectoralis major insertion.
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66% of patients required a contralateral symmetry procedure in LD flap reconstraction
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the latissimus transfers were well tolerated
without upper extremity weakness
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The recipient vessels in all patients of polanf syndrom were the internal mammary
vessels.
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Four
to six months are required for volume equalization of the omentum flap
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percentage of patients who need breast implants after omentum flap
Eighty percent of patients required additional implants
When correction of the infraclavicular territory was necessary, an
ipsilateral free gracilis flap was anastomosed to the internal
mammary vessels
f anastomosed to the thoracodorsal
vessels.
When correcting for breast volume, the free gracilis flap was
placed in a retro glandular position and anastomosed to the internal
mammary vessels
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Because of the incidence of vascular anomalies in the recipient
vessels, preoperative angiography should be strongly considered
when proceeding with free tissue transfer options. in poland
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Anterior thoracic hypoplasia vs poland
anterior thoracic hypoplasia presents with hypoplasia
of the ipsilateral breast, superior displacement of the NAC, and posterior displacement of the ribs with sunken chest wall but a normal
pectoralis major and sternum position
All patients of Anterior thoracic hypoplasia were
treated with a partial submuscular anatomical implant
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Virginal hypertrophy characterized by rapid, excessive, and
the unyielding proliferation of one or both breasts in the adolescent years
for at least 6 months
breasts may grow as large as 13 to 23 kg
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Virginal hypertrophy, can occur before puberty.
F Virginal hypertrophy,
which occurs after a few months after puberty, is differentiated from
prepubertal hypertrophy, which is before puberty and is usually bilateral.
The differential diagnosis
fibroadenoma, phyllodes tumor, lymphedema,
endocrine conditions, rheumatologic diseases, and lymphoma.
the ultimate treatment for breast hypertrophy is medical
The ultimate treatment, however, remains surgical commonly includes reduction mammaplasty and in some cases, mastectomy.
tamoxifen administration for 4 months has been shown
to be the most effective in retarding or arresting breast growth
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recurrence of breast growth is sevenfold
higher following a reduction mammaplasty compared with mastectomy
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It is best to defer reduction mammaplasty
until the later years of adolescence to reduce the risk of recurrence and
revision surgery
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breast-feeding in patients with breast hypertrophy with and without reduction mammaplasty to be equivalent (~60%)
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During adolescence, 75% to 95% of breast lesions are fibroadenomas.
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Giant fibroadenoma refers to when the tumor is larger than
10 cm
F Giant fibroadenoma refers to when the tumor is larger than
5 cm in diameter and/or weighs more than 500 g
Ultrasound may reveal
a well-circumscribed avascular mass.
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Timing is dictated by rate of growth
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Treatment
involves removing the lesion in the well-demarcated plane. To fill in
large cavities, tissue rearrangement techniques are required with consideration to the pedicle and nipple perfusion.
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Pathologically, fibroadenoma is difficult to distinguish from phyllodes tumor
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Polythelia is relatively common and occurs in up to 5.6% of male
and female patients along the milk lines
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Type of inheritance in polythelia
These cases are generally sporadic; however, cases of familial inheritance patterns have been reported
Why patient with polythelis shou;d do US ?
They may be associated with nephrourologic abnormalities
and therefore a urinalysis and renal ultrasound is necessary.
Excision
is typically performed before puberty begins with an elliptical excision
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Polymastia most commonly in the axilla with
a prevalence of 1 % to 2%.Although sporadic, familial cases and
associated renal abnormalities have been identified
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Polymastia is the same of ectopic breast tissue,
Polymastia differs from ectopic breast tissue, as ectopic breast tissue is outside the mammary ridges.
Unlike polythelia, polymastia is usually not identified until after puberty, pregnancy or lactation, and when hormonal
influences enlarge the breast tissue
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These lesions generally present
with discomfort during certain points of the menstrual cycle
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This
accessory breast tissue may be excised and closed primarily over a
drain as needed. If excision is forgone, then screening for malignancy
is necessary because breast cancer can occur with the accessory tissue
at an equal rate to the natural breast.
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Gynecomastia incidance
overall incidence of 32% to 36% and up to 65% in adolescent male
Bilateral disease occurs in 25% to 75% of
individuals
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most cases of gynecomastia during adolescents
are drug induced
F most cases of gynecomastia during adolescents
are idiopathic
the most common cause in those >40 years old is most
often drug induced
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elderly (>65) when both a decline in androgen
and aromatization peripherally of testosterone to estrogen occur
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Simon and
Kahn described a classification system
Grade 1: slight breast enlargement without redundancy,
Grade 2A: moderate breast enlargement without skin redundancy,
Grade 2B: moderate breast enlargement with marked skin redundancy, Grade 3: marked breast enlargement and skin redundancy.**
Webster
described a classification based on tissue type
Type I: Presence ofglandular tissue
Type II: Presence of fatty and glandular mix
Type III: Presence of simple fatty tissue
Rohrich described the most utilitarian method for classifying
gynecomastia, based on the amount and character of breast hypertrophy and degree ofptosis
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The risk of
breast cancer in patients with gynecomastia remains equivalent when
compared with the normal male population
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The incidence of breast
cancer is 60 times higher in patients with gynecomastia associated with Klinefelter syndrome when compared with the general population and thus necessitates mastectomy.
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Tamoxifen has been successful in the treatment of fatty gynecomastia in which there is a
palpable nodule under the areola.
F Tamoxifen has been successful in the treatment of glandular gynecomastia in which there is a
palpable nodule under the areola.
Liposuction as a primary
modality over surgical excision, reducing the incidence of postsurgical contour abnormalities and asymmetry.
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the subdermal
and subareolar regions are suctioned for maximal skin retraction and
disruption of the fibroconnective tissues
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What is saucer-type deformity?
Overzealous subareolar resection may lead to a
saucer-type deformity under the areola. It is therefore advisable to leave
a layer of fibrous tissue under the areola for a smooth contour to avoid
this complication.
An alternative to excision is to use an arthroscopic
shaver to treat the excess subareolar tissue
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Postoperatively, compression garments are worn
for at least 6 weeks, and heavy exercise should be avoided for 1 month.
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Gynecomastia requires complete medical workup
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primitive mammary
ridges come from epidermal cell
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lactiferous tissue formed by 8 weeks
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NAC involvement is present in
about 50% of cases of tuberous breast
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Mandrekas come with the idea of a ring theory
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ring theory
histologically
demonstrating a dense constricting ring spanning from the periphery
of the NAC to the lower part of the breast representing a thickening
of the superficial fascia
Restoration of a normal nipple-to-IMF distance through
expansion and/or mastopexy and lowering of the IMF
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For adequate release, bands at the base of the breast
parenchyma and along the IMF are radially dissected from deep to
the superficial until the deep dermis to the point visual and palpable
release of the lower pole
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In KOLKER AND COLLINS classification type II Skin Envelope Sufficient
F Inferior insufficiency
In KOLKER AND COLLINS classification type II Iframamery fold medial and lateral elevation
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In KOLKER AND COLLINS classification type III Elevation of the entire inframammary Fold or fold absence
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In patients with less
severe tuberous deformities, the IMF is well defined, and further lowering the IMF may be counterproductive
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Rees and Aston
first described radial scoring on the undersurface of the breas
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Minor criteria of Poland
■ Hypoplasia or absence of the breast
■ Absence of the nipple
■ Absence of axillary hair
■ Absence of adjacent muscles (e.g., latissimus dorsi, serratus anterior, external oblique, deltoid, infraspinatus, supraspinatus, and
pectoralis minor)
■ Absence of costal cartilage and anterior ribs
■ Absent subcutaneous tissue
■ Axillary webbing
■ Ipsilateral brachydactyly, brachysyndactyly, or amelia
Foucras et al. and categorized the severity of poland disease to mild,
moderate, and severe
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The spectrum may range from the webbing of the fingers, fusion of the carpal bones, absence of phalanges, and syndactyly to shortened forearm and/or upper arms in poland
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The etiology ofPoland syndrome
the
leading theory suggests by the sixth week ofgestation, when the limb
bud is adjacent to the chest wall, interruption ofthe embryonic blood
supply results in hypoplasia ofthe subclavian artery or its branches
Obstruction of internal thoracic artery leading to the absence of the sternocostal head of the pectoralis major
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Obstruction of brachia! artery leading
to hand anomalies
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suprascapular artery leading to elevation or
winging ofthe scapula, Sprengel deformity
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Skeletal deformities should be addressed before breast reconstruction.
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indications for chest wall reconstruction in Poland
wall depression, inadequate protection of
the mediastinum, and paradoxical movement of the chest wall
The timing of breast reconstruction for patients with Poland syndrome
commonly delayed
until late adolescence or adulthood when breast development is complete, with the pedicle latissimus dorsi flap most commonly used
fat grafting and tissue expander placement at earlier ages to relieve psychosocial morbidity.
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Fat grafting can be used in severe Poland syndrome
F in mild and moderate cases
The low morbidity of this procedure has permitted its safe use in adolescent females at the onset of
puberty (fat grafting )
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modern imaging modalities
are able to differentiate between microcalcification due to fat grafting
and malignancy
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When there is a
soft-tissue deficiency and the IMF is high and tight, placement of tissue
expanders can be beneficial.
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Most common cause of free flap loos in Poland syndrome is the venous outflow anomalies in the subscapular system
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the diagnosis od giant fibroadenoma is clinical, confirmation may be obtained with a fine-needle aspiration
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Giant Fibroadenoma is painless
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The risk of
breast cancer in patients with gynecomastia remains equivalent when
compared with the normal male population.
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the double-bubble deformity is not caused by disruption of an IMF ligament but by
failure to fully detach the superficial fascia! system from the
dermis
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suggesting continued weight loss would resolve gynecomastia is
inappropriate.
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Histologically, phyllodes
tumor shows more stromal proliferation.
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adolescent phyllodes tumor is less aggressive and therefore can be
excised with a small rim for a surgical margin
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