Congenital Anomalies of the Breast: Flashcards
pediatric breast disorders may be categorized into hypoplastic, hyperplastic, and deformational
T
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
T
Portions atrophy, except at the fourth intercostal space, which set the foundation for the primary mammary bud.
T
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
T
With circulating estrogen and progesterone from the placenta, the
lactiferous tissue continues branching into 2 years of life
T
The normal
gland remains quiescent from 2 years until puberty
T
At birth, the neonatal mammary tissue is functional
T
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
T
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
T
Amastia is the complete absence of
the breast unit, including the gland and nipple
T
athelia often occurs in conjunction with amazia.
T
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
T
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
T
Implants,
mucocutaneous flaps, and autologous fat transfer are viable methods
of reconstruction of breast hypoplasia
T
Careful consideration of familial breast cancer history is mandatory when employing fat transfer techniques.
T
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.
t
73% of females
presenting forbreast augmentation were found to have tuberous breast
deformity,
T
Ninety-eight percent ofthe patients with tuberous breasts
were asymmetrical in size as well
T
milder forms are commonly underappreciated and underdiagnosed
T
when the soft tissues are distensible and the size goals are attainable, correction can be performed
in a single stage
T
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.
T
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
T
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.
T
Mandrekas and Zambacos utilized a periareolar incision to exteriorize the lower half of the breast
T
Abbate, Fan, and Nahabedian described a new technique using a central
mound mastopexy via an inverted-T incision
T
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
T
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
T
In patients with severe tuberous breasts, correction of the IMF is mandated because of a short nipple-to-IMF distance.
T
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.
T
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
T
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
T
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
T
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
T
Classically,
Poland syndrome was defined as the absences of the pectoralis
major and ipsilateral hand abnormality
T
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.
T
The mild variant of Poland syndrome is more common than the
classic full presentation
T
The moderate variant of Poland syndrome represents the classic
form
T
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
T
CT scans to evaluate the structural abnormalities.
T
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%
T
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.
T
Poland syndrome can be associated with Mobius and KlippelFeil syndrome
T
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
