Download Lipodystrophy

Lipodystrophies
 Heterogeneous group of diseases clinically characterized by a congenital or
acquired loss of fat in circumscribed, partial, or diffuse areas of the body.
Considerations
 Congenital vs Acquired
 Partial vs Generalised
 lipohyperplastic (increased fat cells) or Lipohypertrophy (Fat fat cells)
 Lipoatrophy (reduced or deficient fat cells) or Lack of Fat (starvation)
Congenital
Congenital generalized lipoatrophy
Familial partial lipodystrophy - Kobberling-Dunnigan syndrome
 born with normal fat distribution but notice a loss of subcutaneous fat in the
extremities and trunk with early puberty.
 This is followed by increased fat in the face and neck as puberty is completed
 visceral fat and interfascicular intramuscular fat depots are preserved
 defect in lamin A gene
Acquired
Lawrence syndrome(Acquired generalised lipodystrophy)
 Onset during childhood
 severe fat loss, including retro-orbital fat and supportive fat in their hands,
feet, and genital area.
 Most develop diabetes 4 years after onset
Barraquer-Simons Syndrome (Acquired partial lipodystrophy) – PRS Jan 2001
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Usually females (4:1), usually follows febrile illness
Subcutaneous symmetrical fat loss is usually limited to the face, trunk, and
upper extremities.
Simultaneously, fat hypertrophy occurs in the lower extremities.
Seldom associated with insulin resistance
Nephropathy- type II membranoproliferative glomerulonephritis in 20%
Complement anomalies with low serum C3 due to C3 nephritic factor, which
is an immunoglobulin G that augments C3 activation, may be found in the
disease and are probably involved in the pathogenesis of the nephropathy
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Differential
 Clinically, bilateral Romberg disease(rare) and Barraquer-Simons syndrome
are difficult to distinguish.
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Romberg disease may affect any or all of the facial tissues, such as skin,
subcutaneous fatty tissue, musculature, cartilage, and bone, whereas
Barraquer-Simons syndrome involves primarily adipose tissue
Management
 Treatment is entirely symptomatic initially – referral to perdiatrician,
nephrologist.
 Prognosis is dependent on the amount of nephrotic changes in the kidneys.
 Process of fat atrophy must be finished before any reconstructive procedure.
 Traditionally
 Bone grafts, cartilage grafts, and silicone have been used to fill facial
depressions.
 Fat, dermis- fat, and dermis-fascia-fat grafts, although they often
undergo significant reabsorption, have also been used to fill facial
defects.
 These methods have been abandoned as they achieve an unnatural
result or only temporary improvement because of the large area that
must be filled
 More recent, trend has been to use flaps
 local de-epithelialized flaps
 Local muscle flap
o Temporalis muscle reported to be very good (Serra, Ann Plast
Surg 1994)
o deepithelialized musculocutaneous pectoralis major flap or
deltopectoral flap
 Free flap
o deepithelialized groin, or scapular/parascapular flap
o Double paddle dermis-fat radial forearm free flap – advantage of
one-stage operation using one flap (Endo, Ann Plast Surg 1994)
o 2 free deepithelialised TRAM flap (Goosens PRS 1995)
o 2 free rectus muscle flap - in comparison with adipose tissue,
muscle tissue does not show a tendency for ptosis because of its
consistency and firm attachment of the muscle surface to the
surrounding tissues (Goossens Microsurg 2002)
o 2 free anterolateral thigh flaps – staged procedure (Guelinckx
PRS 2000) - the presence of fascia allows for adequate fixation to
superficial temporal fascia, thus preventing sagging of the flap.
Placing the fascia of the flap toward the skin guarantees a smooth
cheek contour after thinning of the flap.
o vasculitis and perivasculitis of the facial artery and vein has
been noted (as in Rombergs), thus recipient vessels need to
chosen carefully
Barraquet-Simons (acquired partial lipodystrophy) treated with bilateral staged ALT.
Benign symmetric lipomatosis (Madelung disease) – PRS Feb 2006
 M>F (4:1)
 prominent, symmetrical masses on the neck, shoulders, arms and uppers parts
of the trunk
 associated with alcoholism and insulin resistance
 may complain of compressive symptoms (shortness of breath, dysphagia) and
difficulty moving neck
 Main theory - mitochondrial dysfunction of brown fat
 goal of surgery is debulking of the fatty mass to provide a better, acceptable
cosmetic appearance
 Treat with open lipectomy
 open excision preferred over liposuction as lipomatous deposits are almost
always located beneath the platysma
 a safe dissection plane can be achieved with a skin-platysma flap elevation.
HIV-associated lipodystrophy
 characterized by central adiposity and peripheral fat wasting,
dyslipidaemia, impaired glucose tolerance, and insulin resistance in HIV
patients treated with protease inhibitors
 Mechanisms include mitochondrial dysfunction, and binding to cytoplasmic
retinoic-acid binding protein type 1, may prevent a cascade of molecular
events leading to peripheral adipocyte differentiation and apoptosis.
 peripheral lipoatrophy- wasting in the face, arms, legs, and buttocks
 central lipohypertrophy - fat accumulation in the abdomen,visceral fat, breasts,
or dorsocervical region (“buffalo hump”)
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Syndromes of HIV lipodystrophy
1) cervicodorsal fat pad enlargement ("buffalo hump")
2) truncal obesity (also known as "protease paunch" or "Crix-belly," in
reference to Crixivan, a protease inhibitor)
3) breast hypertrophy in women; gynecomastia in adult males
4) fat deposits on the anterior neck and lateral mandibular region
5) a fat-wasting syndrome of the nasolabial folds and midface
6) a fat-wasting syndrome of the extremities
may improve by changing drugs but only slowly and incompletely
HIV-positive individuals experience periods of elevation and remission of
viral load during the course of their illness, and consultation with each
patient's infectious disease specialist regarding the safety of operating on
the patient is invaluable.
Postponement of the surgical procedure is prudent should preoperative
laboratories reveal inversion of the normal 2:1 CD4: CD8 ratio, or when
patients report increased fatigue or recent weight loss
Management lipohypertrophy
i. Tumescent suction lipectomy (PRS Oct 1999)
 Liposuction of hypertrophied cervicodorsal fat pad and of adipose tissue
deposited in the submental, mandibular, and lateral cheek regions of the
face and in the abdomen
 Ultrasound preferable due to dense fibrous septa in these fat deposits
Management of facial atrophy
 15-80% of patients on HAART develop facial lipoatrophy within 10 months
of initiating therapy.
 Facial lipoatrophy is the most frequent and distressing sign for patients
receiving anti-HIV therapy with good virologic and immunologic status.
 Treatment-related lipoatrophy has been cited as a reason to delay the initiation
of antiretroviral therapy and has been reported to contribute to a reduction in
patient adherence to therapy
i. Cheek implants
ii. Poly-L-lactic acid (New-Fill or Sculptra in USA) injections
 approved indication by FDA 2004
 biodegradable, biocompatible synthetic polymer
 intradermal injection
 acts both immediately as a filler proportionnally to the injected
volume, and on a longer term due to a locally induced neocollagenosis
which persists after the PLA resorption.
 most frequent event was palpable persistant, non-visible subcutaneous
micronodules which did not bother the patients.
 Effects up to 2 years
 improved overall appearance in an average of 3 treatment sessions.
iii. Fat grafts
 50 percent of transferred fat volume is lost during the first 3 to 5 months
after transfer
iv. Dermafat grafts (PRS Jan 2004)
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from the abdominal wall to malar pockets through a transoral approach
dissect supraperiosteal, graft placed fat side up and tied over bolsters
 dermal side up – better contour, better vascularity
 dermal side down – better ptosis prevention
grafts up to 1 cm in thickness perform very well in the normal face, with
virtually no loss of volume when fixed against an adequate nutritive
substrate.
aim to overcorrect