Ruiz RO; Portela DL; Gonella HA; Pinheiro LF; Rives JM; Gerenutti M

Case Report

Austin Plastic Surg Open Access. 2018; 1(1): 1002.

Parry-Romberg Syndrome, a New Therapeutic Alternative: Case Report

Ruiz RO¹*, Portela DL¹, Gonella HA¹, Pinheiro LF², Rives JM³ and Gerenutti M⁴

¹Department of Pontifical Catholic University, Sorocaba, SP, Brasil

²Department fo São Paulo´s Red Cross Hospital, SP, Brasil

³Private Practice, Paris, France

⁴Department the Development and Evaluation of Bioactive Substances, University of Sorocaba, Sorocaba Brasil

*Corresponding author: Ruiz RO, Department of Pontifical Catholic University, Sorocaba, SP, Brasil

Received: March 22, 2018; Accepted: May 04, 2018; Published: May 11, 2018

Introduction

Background: The use of the acellular dermal matrix is already known in traumas of diverse natures, its presentation for the injectable use has helped in the therapy of several pathologies, and one of them is the Parry-Romberg Syndrome (PRS).To present a treatment to facial asymmetry and aesthetic improvement of a patient with the associated treatment of fat graft and injectable dermal matrix.

Case Presentation: One female patient, a carrier of the syndrome for ten years, treated several times without success. This time the acellular dermal matrix was applied in hemiface affected by the pathology, to support the facial middle third and to return the lost volume, on the non-affected side was injected for the best symmetrization.

Conclusion: The result after 180 days remained very acceptable as to the parameter of symmetry, and aesthetic improvement of the patient’s face, there was a great increase in self-esteem. The result with fat graft and dermal matrix remain satisfactory after 180 days.

Keywords: Parry-Romberg Syndrome; Dermal Matrix; Facial Symmetry; Fat Graft

Abbreviations

PRS: Parry-Romberg Syndrome

Background

Hemifacial muscle atrophy or Parry-Romberg Syndrome (PRS) was first described by Calleb Hillier Parry, an English physician, in 1825 and disseminated by Moritz Heinrich Romberg, a German physician, in 1845. It is a rare, autoimmune syndrome which is prevalent in younger than 20 years old patients. It has a slight occurrence in females. No racial distinction was observed [1].

Several signs can be observed in its appearance, which according to the severity can be classified as mild, moderate and severe. These symptoms are diverse, such as skin hardening, mainly above the maxillary region, dyschromic spots on facial or scalp, facial pain, an appearance of white hair. These can progress to bone deformities, poor tooth and gum formation and alopecia. They can get worse and lead to dermal, bony and muscular atrophies. Sometimes it presents intracranial vascular malformations and convulsive conditions, which indicates cerebral compromise [2-8].

Clinical treatment lends itself to control neurological and ocular symptoms, and pharmacotherapeutic treatment seeks better swallowing and phonation. The surgical treatment aims at an aesthetic improvement and correction of the facial asymmetry, and several surgical techniques have been attempted. Dermal, cartilaginous, facial and bone grafts, multiple flap rotation, alloplastic fillers and fat graft may be used [9-12]. Most patients need repeated procedures or fat grafts for the correction of asymmetry.

The dermal matrix, which was described in 1981 by Burke and Yannas [13], consisting of a layer of acellular bovine dermis covered by a silicone blade, being very used to cover uninfected and nonsecretive traumatic lesions, completing the treatment after a few days with partial skin grafting [14-16]. Under went modernization, since 2017 if it has a single presentation layer, where the silicone layer was removed and allows the accomplishment of skin grafting in a single procedure in traumatic injuries or burns [17-23]. Today we have an injectable presentation [24-26]. That can be used to treat facial asymmetry resulting from the described syndrome.

To present a treatment to facial asymmetry and aesthetic improvement of a patient with associated treatment of fat graft and injectable dermal matrix.

Case Presentation

Female patient, 45 years old, presenting Parry-Romberg syndrome for 10 years, with seven fat grafts already performed in the plastic surgery service of Sorocaba, presenting important facial asymmetry. This treatment done in São Paulo’s Red Cross Hospital.

All preoperative exams were performed and were within the parameters of normality. he areas of liposuction were previously marked on the inner side of the thigh and on the face, demarcating the areas to be treated, with continuous lines marking the bony protrusions and between the lines were the areas that would be approached by means of intradermal injections, promoting the Filling, thus recovering the facial volume (Figure 1).

Figure 1: Making of the areas to be treated, the solid lines mark the bony facial protrusions and between the lines is the area to be treated intradermally as dermal filler.

    
    
    Figure 1:  Making of the areas to be treated, the solid lines mark the bony
facial protrusions and between the lines is the area to be treated intradermally
as dermal filler.

With the patient in horizontal dorsal decubitus under narcosis, asepsis, and antisepsis of the entire face, lower limbs and abdominal region were promoted. A liposuction of 40ml fat was performed on the inner thigh surfaces using a 50ml syringe. After centrifugation of the contents of the liposuction, 9ml of pure fat was separated, adding 9ml of the injectable dermal matrix and reserved for later use in the face (Figure 2).

Figure 2: A) Centrifuge with the tubes with aspirated material. B) Homogenization of fat with the injectable matrix.

    
    
    Figure 2:  A) Centrifuge with the tubes with aspirated material. B)
Homogenization of fat with the injectable matrix.

Facial treatment began with Bolus injection using microcannula of 2mm in diameter and length ranging from 10 to 15 cm from the pure injectable dermal matrix in the regions of bony protrusion on the side affected by the pathology to promote abutments and mandibular contour, as shown in (Figure 3).

Figure 3: Schematic drawing on photo illustrating the injection points of the “pure” matrix in Bolus, more deeply, promoting pillar of facial support and reinforcing the bony protrusions. The fan lines demonstrate how the intradermal injections of homogenized fat with matrix. Drawing on photos of Christian Gaul and Roberta Fontelles Philomeno-model Caroline Ribeiro.

    
    
    Figure 3:  Schematic drawing on photo illustrating the injection points of
the “pure” matrix in Bolus, more deeply, promoting pillar of facial support
and reinforcing the bony protrusions. The fan lines demonstrate how the
intradermal injections of homogenized fat with matrix.
Drawing on photos of Christian Gaul and Roberta Fontelles Philomeno-model
Caroline Ribeiro.

After this maneuver, homogenized fat was used with the matrix as soft part filler, making intradermal injections, in a fan refining the treatment of the middle third. The contralateral side was also treated with fat homogenized with the dermal matrix for an improved symmetrization.

The patient was discharged from hospital 24 hours after the procedure with compressive meshes in the liposuction region, without dressings on the face. Photos of the patient were taken in the pretreatment and serially until the sixth month in which maintenance of the result is observed (Figure 4).

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Citation: Ruiz RO, Portela DL, Gonella HA, Pinheiro LF, Rives JM and Gerenutti M. Parry-Romberg Syndrome, a New Therapeutic Alternative: Case Report. Austin Plastic Surg Open Access. 2018; 1(1): 1002.

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Parry-Romberg Syndrome, a New Therapeutic Alternative: Case Report

Introduction

Abbreviations

Background

Case Presentation

Discussion and Conclusions

Declarations

Acknowledgements

Competing Interests

References