Marys Medicine

Lipofiling and modified "kligman's formula" for the treatment of parry-romberg syndrome

Journal of Surgery & Transplantation Science
Bringing Excellence in Open Access*Corresponding author
Ioannis Liapakis, OpsisClinical Plastic and
Reconstructive Surgery, 48 Anogion St., 71304, Therissos,
Lipofiling and Modified Heraklion-Crete, Greece, Tel: 30-69-32934051; Email: Submitted: 11 May 2016
"Kligman's formula" for the Accepted: 23 May 2016
Published: 27 May 2016
Treatment of Parry-Romberg ISSN: 2379-0911
2016 Liapakis et al.
Liapakis IE1, Christopoulos A2, Tzouganakis AC1, and Paschalis
• Parry-Romberg syndrome Department of Plastic and Reconstructive Surgery, Hel enic Anticancer Institute, Greece • Hemifacial atrophy Department of Plastic and Aesthetic, Monash Institute of Pharmaceutical Sciences, Greece • Kligman's formula Department of Ophthalmology, Harvard University, USA • Ascorbic acid• Lipofiling • Skin hyperpigmentation Parry-Romberg syndrome is a degenerative disease characterized by progressive hemifacial atrophy of the skin, muscle and bones of the forehead, eyes, mouth, nasolabial crease and the upper/lower maxilla. It causes skin hyperpigmentation and trigeminal neuralgia. The syndrome has higher prevalence in women between the ages of 5-15 and it stabilizes within 2-10years. It results in significant facial deformity, which affects the patient's self esteem and social appearance. Aim of the study: We present a case of Parry-Romberg syndrome with facial deformity that was treated with lipofilling and bleaching of the skin using
a modified "Kligman's formula" cream. Materials and Methods: A 21years old female with left hemifacial atrophy was diagnosed with Parry-Romberg syndrome at age of 15, which has
been stable for the last 2years. The patient was treated with lipofiling of 20cc of fat in the left forehead, nose, nasolabial crease, cheek, and mental region. Treatment of skin hyperpigmentation performed for 1 month every night (to eliminate the erythema), using a modified "Kligman's formula" cream with added 5% ascorbic acid and applied gradually to the skin: the 1st week applied 1hour before sleep, the 2d week 2hours before sleep, the 3d week 3hours before sleep and the 4th week all the night long. The day after and every day after the treatment, a hydration/sun care cream (with SPF over 30) applied to the skin in order to eliminate the irritation/dehydration of the skin.
Results: The appearance of the hemifacial atrophy and skin hyperpigmentation was significantly improved using lipofiling and the bleaching cream. We
followed the patient 6 months after the procedure, since there is no evidence that more time is needed to evaluate the stability of the results. No complications occured during and after the procedure. Conclusion: Facial deformities derived from Parry-Romberg syndrome can be successfully treated with fat transfer and bleaching of the skin. More studies
are required to demonstrate the efficacy of this procedure.
Parry-Romberg syndrome is an acquired progressive hemifacial atrophy. It is a rare disorder (1/700,000), which affects mostly women. In the modern literature it first described by Parry and Romberg [1, 2], however, there evidence of its existence in ancient times (Figure 1). It affects the skin and subcutaneous tissue and, in some cases, extends to the muscles, cartilages or underlying bones [3]. Typically, it begins during the first two decades of life [4, 5] and progresses for 2-10 year during the active phase before stabilization, however, it has also been reported in older patients in their 5th or 6th decade of life. [1, 6]. It is characterized by dermatologic, and, in rare cases, neurologic clinical changes on one side of the face, including trigeminal neuralgia, skin hyperpigmentation with mouth, eye, even hair involvement [4, 5, 7-10]. Parry-Romberg syndrome leads to scar- Figure 1 "Fayum" probably with Parry-Romberg syndrome from
like skin changes, thinning of the skin and subcutaneous tissue 70bC at Egypt.
Cite this article: Liapakis IE, Christopoulos A, Tzouganakis AC, Paschalis EI (2016) Lipofiling and Modified "Kligman's formula" for the Treatment of Parry-
Romberg Syndrome. J Surg Transplant Sci 4(3): 1028.

Liapakis et al. (2016) Bringing Excellence in Open Access with discoloration of the hair or alopecia areata, atrophy of the orbital, palpebral, zygomatic and masseter muscles. The final result is craniofacial asymmetry, affecting the self-esteem of the patient and their social acceptance [11, 12]. Several surgical techniques have been previously reported for the treatment of this comdition. For mild cases with soft tissue involvement, fat/dermal grafts or dermal fillers including calcium hydroxylapatite, can be used to correct volume loss [13, 14]. For severe cases with asymmetry, it is advised to wait for stabilization of the condition and then proceed surgically to correct the asymmetry [14-16], with free flap transplantation, alloplastic implants, medial canthopexy or septorhinoplasty. Figure 2 Intraoperative photo from the lipofiling areas.
The aim of this case report is to present a mild case of Parry- Romberg syndrome using lipofiling and skin bleaching with a modified "Kligman's formula" cream.
A 21 years old Caucasian female patient appeared at our clinic (OpsisClinical Plastic and Reconstructive Surgery) with left hemifacial atrophy. She was first diagnosed with Parry-Romberg syndrome when she was 15years old and she has been stable for at least 2years.
Cannulas used for liposuction (upper) and lipofiling (lower) at the face.
The patient was treated by lipofiling of a total of 20cc of fat at the left forehead, nose, nasolabial crease, cheek, and mental region (Figure 2). The adipose tissue used for the filing was harvested from the patient's anterior abdominal wall. After local anesthesia was administered, the subdermal tissue lifted away from the underlying structures at the abdomen region by gently grasping the skin. Autologous fat collection was performed with rapid palindromic movement at the subcutaneous tissue of 10cc syringe 1mm in diameter with multiple holes, resulting in an atraumatic and bloodless collection of fat [17, 18]. Each 10 cc syringe was gently placed into a sterilized sleeve and centrifuged at 3000rpm for 2-3min to collect the fat, which was separated into three layers: the top-layer, composed mainly of oil with triglycerides derived from the destroyed adipocytes; the middle- Figure 4 Patient with Parry Romberg pre- and 6 months
layer, composed of purified fat suitable for transplantation; and the bottom-layer, composed of serum and blood debris. The top layer of oil was removed and the bottom layer was drained. The skin and muscle may be involved, however, in more severe refined fat cells were administered to the underlying tissues cases, ocular and/or neurologic manifestation may be present using a blunt tip cannula in 1.7mms in diameter (Figure 3). The [19]. In younger patients, bony atrophy may require immediate deposition of the fat was linear in small parts per passage in intervention [20-22], but recent studies strongly suggest that order to maximize cell survival.
treatment should be initiated only after disease stabilization Subsequently, skin hyperpigmentation was treated for one [13-16]. If treatment begins too early, additional surgeries may month with a cream containing 5% ascorbic acid in the original be required [3] to accommodate disease progression, such as lipofiling, medial canthopexy or septorhinoplasty. The type of surgical reconstruction almost always depends on the severity of the deformity. In mild to moderate hypoplasia, lipofiling may The hemifacial atrophy and the skin hyperpigmentation were be adequate, but in more severe cases, dermal fat grafts, silicone significantly improved with no recurrence 6 months after the implants or adipofacial free flaps may be required in conjunction treatment (Figure 4).
with skeletal support.
Currently, the use of fat transfer via liposuction was proposed as the "gold standard" for the correction of Parry-Romberg Parry-Romberg syndrome is a progressive degenerative syndrome [24, 25]. The method was first described by Illouz et disease causing facial deformation, which may impact patient's al. [26] and was popularized with the introduction of tumescent self esteem and social appearance. In mild cases, only the fat, topical anesthesia by Klein et al [27]. However, Coleman at al. J Surg Transplant Sci 4(3): 1028 (2016)

Liapakis et al. (2016) Bringing Excellence in Open Access [28] made the most critical modification, improving the overall glycolic acid can promptly depigment the skin [38]. Kojic acid 2% success of the technique. He reported the use of a fat aspiration combined with hydroquinone 2% was shown to be superior to and re-application protocol, with careful aspiration and glycolic acid 10% and hydroquinone 2% [39]. The combination centrifugation, which increased the survive rate of the cells and of hydroquinone 5%, tretinoin 0.1%, and dexamethasone 0.1% engraftment [29, 30]. This method is particularly more efficacious in a hydrophilic ointment was first introduced by Dr Albert [31] for treatment of the malar, cheek and chin region, but not as Kligman as the "Kligman's formula" in 1975 [40] and has been successful for treatment of the upper/lower lip and the temporal extensively used since for post-inflammatory hyperpigmentation region. However, Matsumoto [32] and Yoshimura [3], described (PIH), chloasma, melasma, age spots, scars and nevi of Ota or Huri improved outcomes of cell survival, angiogenesis, and adipose [41]. Hydroquinone inhibits the conversion of dopa to melanin by tissue regeneration by enrichment of the aspirated fat cells with blocking the activity of tyrosinase [42]. As suggested by Jimbow adipose-derived stem cells (ASCs). In our study, liposuction was et al, hydroquinone interferes with DNA and RNA synthesis, carefully performed at the anterior abdominal wall and cells were degrades melanosomes and destroys melanocytes [42, 43]. In rare purified with centrifugation. Only the middle band containing cases it can cause allergic reaction and contact dermatitis, [44- purified fat was selected for transfer, while the top and bottom 46] nail bleaching, and ochrosis-like pigmentation [47, 48]. Even bands were discarded. Application of these cells to the hemifacial though hydroquinone has been shown to cause animal toxicity atrophy was achieved at sequential administration of 20ccs of fat. [49], no complications have been reported in humans [50]. The lipofiling was very careful, after aspiration with successive Alternative bleaching agents, such as kojic acid or azelaic acid, movements of the canula since there is evidence of severe are useful but not as potent as hydroquinone [51,52]. Tretinoin complications even blindness after filler/fat application at the 0.05%-0.1% reduces pigmentation by inhibiting tyrosinase nose/forehead areas [34]. We followed the patient 6 months transcription and by interrupting melanin synthesis [53], but, after the procedure, since there is no evidence that more time is in some cases, may cause erythema or increase pigmentation needed to evaluate the survival of the fat [35, 36]. due to the irritation of the skin [54]. The tretinoin eliminates A secondary effect of Parry Romberg syndrome is skin pigment and increases keratinocyte proliferation by preventing hyperpigmentation. The etiology is not fully understood, however, oxidation of hydroquinone, therefore it improves epidermal combination treatments can improve the skin's pigment [37]. For penetration. Furthermore, adding topical corticosteroids reduces example, azelaic acid combined with 0.05% tretinoin or 15-20% the irritation of the other agents and inhibits melanin synthesis by decreasing cellular metabolism [55]. The addition of ascorbic acid to the original "Kligman's formula" can increase the activity and efficacy of the hypopigmenting agents by improving the anti-oxidant effect and collagen stimulation (Figure 5, 6). In this study, skin hyperpigmentation was treated using 5% ascorbic acid into the "Kligman's formula". The treatment performed for 1 month every night (to eliminate the erythema) and applied gradually to the skin: the 1st week applied 1hour before sleep, the 2nd week 2hours before sleep, the 3rd week 3hours before sleep and the 4th week all the night long. The day after and every Figure 5 Patient before and 1 month after treatment with cream
day after the treatment, a hydration/sun care cream (with SPF (modified "Kligman's formula" and 5% ascorbic acid).
over 30) applied to the skin in order to eliminate the irritation/ dehydration of the skin.
Parry-Romberg syndrome is a degenerative disease that leads to soft tissue atrophy as well as the skin hyperpigmentation. Surgical intervention is often required using fat transfer in conjunction with skin bleaching. We report a case of a patient treated with lipofiling and a modified cream which resulted to significant improvement and stable results for at least 6 months. Our results suggest that adding 5% ascorbic acid at the original "Kligman's formula" may improve the skin bleaching, however, more studies are required to establish the power of the results.
Figure 6 Patient before and 1 month after treatment with cream
(modified "Kligman's formula" and 5% ascorbic acid).
J Surg Transplant Sci 4(3): 1028 (2016) Liapakis et al. (2016) Bringing Excellence in Open Access 11. Stone J. Parry-Romberg syndrome. Pract Neurol. 2006; 6:185-188.
14. Cox SE, Soderberg JM. Idiopathic hemifacial atrophy treated with serial injections of calcium hydroxylapatite. Dermatol Surg. 2010; 36: 19. Gorlin R, Cohen N, Hennekam R. Syndromes with unusual facies: Well- Known Syndromes. Syndromes of the Head and Neck. 4th ed. New York, NY: Oxford University Press, 2001:977-1038.
J Surg Transplant Sci 4(3): 1028 (2016) Liapakis et al. (2016) Bringing Excellence in Open Access 46. Ennes SB, Paschoalick RC, Mota De Avelar Alchorne M. A double- blind, comparative, placebo-controlled study of the efficacy and tolerability of 4% hydroquinone as a depigmenting agent in melasma. J Dermatolog Treat. 2000; 11:173-179.
Cite this article
Liapakis IE, Christopoulos A, Tzouganakis AC, Paschalis EI (2016) Lipofiling and Modified "Kligman's formula" for the Treatment of Parry-Romberg Syndrome. J Surg Transplant Sci 4(3): 1028. J Surg Transplant Sci 4(3): 1028 (2016)



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