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PROGRESSIVE HEMIFACIAL ATROPHY
INTRODUCTION
 Progressive hemifacial atrophy
 characterized by slowly progressive but self-limited atrophy of facial subcutaneous
fat, which can be followed by wasting of associated skin, cartilage, connective or
ocular tissue, muscle, and bone
 First described by Parry (1825). Detailed report by Romberg (1846).
Incidence
 Incidence unknown
 commences usually in the first or second decade of life
 F>M - 1.5 to 1.
 Unilateral in 95 percent of cases.
 R=L
Pathophysiology
 Aetiology unknown. Acquired, not congenital.
 vast majority of cases are sporadic although some familial cases occur, often associated with consanguinity.
 Theories
1. infection
a. chronic localized meningoencephalitis
2. trigeminal peripheral neuritis
a. association of objective sensory loss and neuralgia of the trigeminal nerves
b. chronic cell-mediated vascular injury and incomplete endothelial regeneration along branches of the trigeminal nerve (lymphocytic neurovasculitis) Mulliken
3. scleroderma (autoimmune)
4. cervical sympathetic loss
a. Ablation of the superior cervical sympathetic ganglion in animals can replicate features of the disorder,
b. 1 case report following thorascopic sympathectomy
5. brain dysgenesis
a. abnormal brain magnetic resonance imaging and the association of partial
epilepsy
Differential Diagnosis
 Shares overlapping feature with morphea (localised) scleroderma
 Main differential feature is that scleroderma affects mainly the skin and causes
induration whereas Romberg’s disease affects the subcutaneous tissue and then
surrounding structures.
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Classification (Inigo)
1. Mild
o atrophy of skin and subcutaneous tissue affecting the territory of only one
of the sensory branches of the trigeminal nerve; no bone involvement
2. Moderate
o two trigeminal territories affected, without involvement of the osseous orbital region or maxillary mandibular development
3. Severe
o all three trigeminal territories involved or bone involvement
Clinical
 Atrophic changes began in a localized area of subcutaneous tissue and progresses,
at a variable rate, within the dermatome of one or more branches of the ipsilateral fifth cranial nerve.
 One of the earliest diagnostic signs is spread of a pigmented atrophic area.
 With progression, muscle and then osteo-cartilaginous atrophy occurs. Early onset
usually implies that skeletal atrophy will occur; late onset usually spares the bones.
 Atrophy in the paramedian forehead appears as a line is called “en coup de sabre”
 Atrophy may be heralded by pigmentary changes of hair, skin or iris.
 Advanced disease can spread to the contralateral face, skull, neck or upper extremity.
 In some cases, half of the tongue and the corresponding salivary glands are involved.
 Facial nerve function usually normal although a case report in PRS 1999 noted abnormality in buccal branch of facial nerve near lesion on histology – showed atrophy of neurofibers with vacuole degeneration. Plasma cell and lymphocytic infiltration noted in the deep dermis. On an electron microscopic examination, a high degree of degeneration of myelinated and unmyelinated axons was seen.
 The most common eye symptoms:
 progressive enophthalmos
 restrictive and paralytic ocular muscle pathologies
 Duane syndrome (miswiring of eye muscles)
 Associated other hemi-atrophies can occur:
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1)
2)
3)
4)
congenital ear anomalies
ipsilateral renal, adrenal, ovarian hypoplasia
ipsilateral amastia
Neurologic symptoms, including epilepsy, migraine and facial pain, and brain
lesions on CT and MRI
5) hair (depigmentation, alopecia), skin (hyperpigmentation, vitiligo)
6) loss of ipsilateral teeth, and jaw complications
 Disease said to “burn out” after 2-10 years, although it has been noted to persist for
longer.
Histology
 Histology shows chronic inflammatory change  scar formation and tethering.
 Electron microscopic observation of the biopsied lesional skin revealed lymphocytic infiltrates in neurovascular bundles and abnormalities of vascular endothelium and basement membranes
TREATMENT
 Timing of operation is based on correction of the deformity after cessation of the
ongoing atrophic process, usually at least 1 year after photographic monitoring
records reveal no further progression of loss of volume to the face.
 The aim of surgical treatment is cosmetic improvement of the facial defect, ie, restoration of normal anatomy and physiology.
Soft tissue augmentation:
1. Autograft
i. Fat dermis grafts
 Donor sites – groin, abdomen, gluteal fold, medial arm, postauricular, supraclavicular
 Dermal fat grafts or injections fat: often have to be repeated multiple times.
ii. Fat graft
 autologous fat grafted into atrophic and barely vascularized recipient tissues (such as in Parry-Romberg syndrome) often degenerates
and partially or wholly disappears.
iii. Fat injections
 Best take using Coleman technique
2. Local flaps
i. Random subcutaneous flaps
ii. Muscle
 Plastysma for lower facial defects
 superiorly pedicled platysma flap swung up into the face to
provide bulk to the affected hemiface.
 3 incisions:
1. supra-clavicular
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2. at the lower border of the mandible
3. in the naso-labial fold
 Temporalis
 Sternocleidomastoid
 Pectoralis major
iii. Pedicled fascio/cutaneous flaps
 Deltopectoral flap for lower 1/3rd
 Temporoparietal fascial flap
 Washio flap
 Original described as non hair bearing postauricular skin
based on the anastomosis between the superficial temporal
artery and the retroauricular artery and is raised without any
delay
 May be modified to include hair bearing skin for reconstruction of beard area in men (PRS 2001)
3. Free flaps
i. Muscle flaps - Lat dorsi, rectus abdominis, serratus
ii. Adipose flaps
 Omentum – gravitation sag not uncommon requiring revision
 Groin flap
1. traditional flap of choice
2. vessel 0.8-1.8mm diameter
3. may share common origin with SIEA in 50%
4. usually arises from the anterolateral aspect of the femoral artery approximately 2.5 cm below the inguinal ligament and
runs laterally before dividing into two branches, 1.5 cm from
its origin.
5. venous drainage is by means of the superficial or cutaneous
system (superficial circumflex iliac vein/superficial inferior
epigastric vein – 2mm), or by means of venae comitantes accompanying the arteries (1mm)
6. posterolateral extent of dissection can be taken past the posterior iliac spine toward the tip of the twelfth rib.
7. In slender individuals, the terminal cutaneous branches of
T12 can often be included in the flap to enable sensory reinnervation
8. Just below the anterior superior iliac spine, the lateral femoral
cutaneous nerve of the thigh pierces the deep fascia. This
nerve may need to be sacrificed in preserving the blood supply to the flap, and patients should be warned of numbness or
paresthesia as a potential complication.
 Scapular, parascapular –
1. advantage over groin flap:
o large calibre and longer vessels
o constant proximal vascular anatomy
o relatively hairless skin in most patients, and minimal
or no functional problems at the donor site.
2. disadvantage
o asensate
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o poor color match
o widening of scar at donor site
3. Longaker uses deepithelialized extended parascapular flaps
with large fascial extensions of the dorsal thoracic fascia. The
fascia can be folded into variable thicknesses to correct subtle
contour defects of the upper lip, medial canthus, eyelids, and
other facial features traditionally difficult to reconstruct.
These extensions can be placed easily across the midline to
interdigitate with normal tissues at the boundary of the facial
deformity. In patients requiring greater skin, fat, and fascial
composite widths, prior tissue expansion of the back cephalad and caudal to the donor site to allow primary closure is
performed. Additional bulk can be obtained by harvesting
portions of regional muscle, such as the teres major muscle, supplied by direct vascular branches
 Anterolateral thigh adipofascial flap
 Superior inferior epigastric artery fasciocutaneous flap
1. offers fairly uniform tissue with a well-hidden donor-site scar.
2. The shortcomings of this flap include an inconsistent and
short vascular pedicle
 Deep Inferior Epigastric Perforator Dermal-Fat or Adiposal Flap
Method
 Paper template of the precise dimensions of the facial defect
 Flap deepithelialised first, then raised
 Preauricular incision
 SupraSMAS dissection – plane is not any harder than normal
1. dissection usually carried out 1cm beyond the limits of the
disease
2. Dissection may be required deep to the alar base at the piriform aperture to allow repositioning of the alar-facial
junction and reconstruction of the deficient alar support
base in an anteroposterior dimension.
3. Malposition and deformity of the ear are addressed similarly
by release of a tethering deficiency of tissue at the base of the
ear followed by placement of vascularized flap tissue as required to build out the base support of the ear.
 Target vessels:
1. superficial temporal vessels (Masaki says may reduce sagging
by using this)
2. facial vessels (preferred by Inigo)
3. superior thyroid artery
4. Others prefer to use ECA due to reports of atrophy involving branches of the external carotid artery (especially the
facial artery)
It has been reported that importation of well-vascularized tissue may interrupt the progression of the disease
problem with muscle flaps (free and pedicled) is unpredictable atrophy
thus adipose flaps are preferred
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o
adipose tissue seem to increase in volume during growth and may also increase with weight gain.
o free flaps tend to be bulky, have tendency to sag, and require thinning and
resuspension.
o Sagging may lead to ectropion of the lower led
o Traditionally flaps are placed subcutaneously between SMAS and skin using a preauricular incision.
o Masaki PRS 2003 has described placing free flap sutured to periosteum to
reduce risk of sagging for lower 1/3rd defect - approached through a
combined preauricular and gingivobuccal sulcus incision.
.
4. Allograft
o Alloderm
5. Alloplastic
iv. Temporary fillers
v. Permanent fillers
1. silicone has been used in the past with significant complications – extrusion, granulomas
2. Goretex
3. Bio-Alcamid described for a moderate severity case (PRS Oct 2005)
vi. Alloplastic filler materials such as polymethylmethacrylate should only be
applied exceptionally because of high rates of morbidity such as infection,
stiffness, and poor cosmesis. (J. Craniomaxillofac. Surg.1994)
Bony augmentation
o
Structural (skeletal) support must either be provided first or as a one-stage reconstruction with soft tissue.
o
Deficiency of the malar prominence cannot always be corrected with soft tissue
alone and often needs bone grafting or alloplastic implant.
1. Autograft
i. Onlay bone grafts are the mainstay.
b. Allopastic
i. Medpor most commonly used alloplast
Management Protocol
Mild
 Fat dermis grafts + fat injection
Moderate
 Free groin flap, parascapular flap, anterolateral thigh flap
 Augment with fat injections at second stage
Severe
 Free flap
 Skeletal augmentation
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o Onlay grafts
Adjunctive procedures
1. enophthalmos
o inferior muscle resection – to bring eye forward
o cutting the periorbita at two equators, advancing the globe in the anterior
periorbita forward, and filling the empty space with thin slices of radiated
cartilage.
2. Orthognathic surgery (Le Fort I; mandible sagittal split).
3. lip thinning
o VY plasties
o Augmentation – VY plasties, temporary filler
4. nose
o rhinoplasty
Radiation induced craniofacial atrophy
 Usually follows treatment of orbital rhabdomyosarcoma, mandibular tumors, parotid tumors, neurofibroma, arteriovenous malformations, extensive hemangiomas, and lymphatic malformations in children
 By releasing the fibrotic and tethered soft tissue, followed by interposing wellvascularized tissues, reconstruction can be optimized. This technique does not involve replacement of irradiated skin. Rather, by restoring contour with wellvascularized underlying tissue, the quality of overlying skin seems to improve in
both quality and color
 In extensive radiotherapy atrophy involving the orbit, maxilla, and mandible.
Jackson recommends:
1. frontotemporal expansion with repositioning of the cranial base
2. orbital expansion and repositioning
3. maxillary surgery with bone grafts to the zygoma as required
4. mandibular lengthening and repositioning
 Bone grafts should be inlay rather than onlay, and soft tissue should be supplied by
free tissue transfer.
 At a second operation, the eye socket and eyelids are reconstructed to allow more
satisfactory rehabilitation with an ocular prosthesis.