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Craniofacial strategies for the management of pachydermoperiostosis – a case report and review of the literature
Journal of Plastic, Reconstructive & Aesthetic Surgery (2009) 62, e511ee513
CORRESPONDENCE AND COMMUNICATION
Craniofacial strategies for the management of pachydermoperiostosis e a case report and review of the literature Pachydermoperiostosis (TouraineeSolenteeGole ´ syndrome) is a very rare genetic osteocutaneous disease characterised by the coarsening of facial features, with thickening and furrowing of the face and scalp (pachydermia), swelling of periarticular tissue and periosteal new bone formation of long bones (periostosis), arthralgia and digital clubbing. The skin of the face, forehead, scalp, hands and feet becomes grossly thickened. The face is drawn into thick folds, with heavy thickened eyelids, which causes the patient to look prematurely aged. The scalp can be thickened and can form a corrugated surface also known as cutis verticis gyrata. It occurs predominantly in males, and the disease usually begins soon after puberty. The disease
Figure 1
pathophysiology remains unclear, although changes usually progress for 5e20 years, after which they remain stable.1 We report the case of an 18-year-old man with pachydermoperiostosis, concerned about his prematurely aged facial appearance prior to starting university education. His condition had stabilised despite rapid progression over the previous 3 years. The skin on his face was greatly thickened and coarse, with deep nasolabial folds and particularly thick folds and deep furrows over his forehead and scalp (Figure 1A). He underwent a mask lift, suspended using forehead and midface Endotine devices. To our knowledge, this is the first reported surgical approach of this kind in this rare condition. A mask subperiosteal facelift was performed via bicoronal and upper buccal sulcus incisions (Figure 2A). The scalp skin and subcutaneous fat was found to be folded and densely adherent to the underlying galea. The forehead was undermined in the subgaleal plane, and a separate pericranial flap was raised with preservation of the supraorbital and supratrochlear nerves. The galea frontalis was excised from the forehead, and the undersurface of the
A. Preoperative appearance. B. Appearance at 1 year following surgery.
1748-6815/$ - see front matter ª 2008 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2008.08.036
e512
Correspondence and communication
Figure 2 A. A mask subperiosteal face lift was performed via bicoronal and upper buccal sulcus incisions. The scalp skin and subcutaneous fat was found to be folded and densely adherent to the underlying galea. B. The galea frontalis was excised from the forehead and the undersurface of the dermis was scored to allow smoothing of the deep forehead rhytids. C. Four Endotine forehead devices were inserted into the frontal bone to suspend the forehead and brow. D. The mask lift continued over the malar, and, in combination with upper buccal sulcus incisions, the midface tissues were elevated off the facial skeleton. Two midface Endotine devices were inserted to lift the middle-third of the face and fixed onto the deep temporal fascia with sutures.
dermis was scored to allow smoothing of the deep forehead rhytids (Figure 2B). A 3-cm strip of excess skin was excised at the level of the bicoronal incision. Four Endotine forehead devices (Coapt Systems, Inc., Palo Alto, California, USA) were inserted into the frontal bone (Figure 2C) to suspend the forehead and elevate the brow. The mask lift continued over the malar and, in combination with upper buccal sulcus incisions, the midface tissues were elevated off the facial skeleton. Two midface Endotine devices were inserted to elevate the middle-third of the face and fixed onto the deep temporal fascia with sutures (Figure 2D). Six months later, a lateral (superficial
musculoaponeurotic system) SMASectomy facelift was performed to improve the lateral facial skin folds (Figure 1B). Surgery to improve aesthetic outcome for pachydermoperiostosis patients has been described since 1965.2 Good results have been reported from direct excision of the skin, supplemented by relaxation incisions under the folds and scalp-reduction techniques.3 More recently, forehead lifting and direct excision of the dermal folds have been described.4 This is the first reported case of an approach using Endotines in combination with mask subperiosteal and lateral SMASectomy facelifts. An open approach was chosen in preference to an endoscopic lift, as it allowed adequate
Correspondence and communication excision of the galea frontalis and scoring of the overlying dermis. The Endotine forehead device is a safe, simple, effective and reliable method of forehead fixation. It avoids recognised problems with metal screws and sutures, which may protrude through the scalp or cause wound irritation and alopecia. The Endotine device is slowly absorbed, and its unique structure provides multiple contact points creating a wider distribution of fixation unlike singlesuture techniques. Our experience has shown that the Endotine device provides a robust fixation, and it is also possible to correct asymmetry and redrape the tissues over the device to improve the aesthetic result in the postoperative period. Fixation techniques that transfer the tension of the suspension to the scalp incisions may result in alopecia. Complications with the Endotine device have been noted.5 Prior to absorption, the device may be palpable or tender in thin-skinned individuals. There may be a need for repositioning owing to asymmetry, or the skin may detach from the Endotine prongs. It is rare for the device to extrude. In conclusion, the use of a combined approach using both mask subperiosteal and SMAS face-lifting techniques together with the resorbable Endotine devices allows reliable elevation and fixation, and treatment of both the
e513 galea frontalis and dermis, thus leading to an improved aesthetic outcome.
References 1. Castori M, Sinibaldi L, Mingarelli R, et al. Pachydermoperiostosis: an update. Clin Genet 2005;68:477e86. 2. Shuster MM, Lewin ML, Caplan L. Facial deformity in pachydermoperiostosis; idiopathic hypertrophic osteoarthropathy. Plast Reconstr Surg 1965;35:666e74. 3. Garden JM, Robinson JK. Essential primary cutis verticis gyrata. Treatment with the scalp reduction procedure. Arch Dermatol 1984;120:1480e3. 4. Kara IG. Forehead lifting for cutis verticis gyrata. Plast Reconstr Surg 2003;111:1777e8. 5. Newman J. Safety and efficacy of midface-lifts with an absorbable soft tissue suspension device. Arch Facial Plast Surg 2006;8:245e51.
L.N. Suleman Verjee A.V.H. Greig W.N.A. Kirkpatrick Craniofacial Unit, Chelsea & Westminster Hospital, NHS Trust, 369 Fulham Road, London SW10 9NH, UK E-mail address: [email protected]
CORRESPONDENCE AND COMMUNICATION
Craniofacial strategies for the management of pachydermoperiostosis e a case report and review of the literature Pachydermoperiostosis (TouraineeSolenteeGole ´ syndrome) is a very rare genetic osteocutaneous disease characterised by the coarsening of facial features, with thickening and furrowing of the face and scalp (pachydermia), swelling of periarticular tissue and periosteal new bone formation of long bones (periostosis), arthralgia and digital clubbing. The skin of the face, forehead, scalp, hands and feet becomes grossly thickened. The face is drawn into thick folds, with heavy thickened eyelids, which causes the patient to look prematurely aged. The scalp can be thickened and can form a corrugated surface also known as cutis verticis gyrata. It occurs predominantly in males, and the disease usually begins soon after puberty. The disease
Figure 1
pathophysiology remains unclear, although changes usually progress for 5e20 years, after which they remain stable.1 We report the case of an 18-year-old man with pachydermoperiostosis, concerned about his prematurely aged facial appearance prior to starting university education. His condition had stabilised despite rapid progression over the previous 3 years. The skin on his face was greatly thickened and coarse, with deep nasolabial folds and particularly thick folds and deep furrows over his forehead and scalp (Figure 1A). He underwent a mask lift, suspended using forehead and midface Endotine devices. To our knowledge, this is the first reported surgical approach of this kind in this rare condition. A mask subperiosteal facelift was performed via bicoronal and upper buccal sulcus incisions (Figure 2A). The scalp skin and subcutaneous fat was found to be folded and densely adherent to the underlying galea. The forehead was undermined in the subgaleal plane, and a separate pericranial flap was raised with preservation of the supraorbital and supratrochlear nerves. The galea frontalis was excised from the forehead, and the undersurface of the
A. Preoperative appearance. B. Appearance at 1 year following surgery.
1748-6815/$ - see front matter ª 2008 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2008.08.036
e512
Correspondence and communication
Figure 2 A. A mask subperiosteal face lift was performed via bicoronal and upper buccal sulcus incisions. The scalp skin and subcutaneous fat was found to be folded and densely adherent to the underlying galea. B. The galea frontalis was excised from the forehead and the undersurface of the dermis was scored to allow smoothing of the deep forehead rhytids. C. Four Endotine forehead devices were inserted into the frontal bone to suspend the forehead and brow. D. The mask lift continued over the malar, and, in combination with upper buccal sulcus incisions, the midface tissues were elevated off the facial skeleton. Two midface Endotine devices were inserted to lift the middle-third of the face and fixed onto the deep temporal fascia with sutures.
dermis was scored to allow smoothing of the deep forehead rhytids (Figure 2B). A 3-cm strip of excess skin was excised at the level of the bicoronal incision. Four Endotine forehead devices (Coapt Systems, Inc., Palo Alto, California, USA) were inserted into the frontal bone (Figure 2C) to suspend the forehead and elevate the brow. The mask lift continued over the malar and, in combination with upper buccal sulcus incisions, the midface tissues were elevated off the facial skeleton. Two midface Endotine devices were inserted to elevate the middle-third of the face and fixed onto the deep temporal fascia with sutures (Figure 2D). Six months later, a lateral (superficial
musculoaponeurotic system) SMASectomy facelift was performed to improve the lateral facial skin folds (Figure 1B). Surgery to improve aesthetic outcome for pachydermoperiostosis patients has been described since 1965.2 Good results have been reported from direct excision of the skin, supplemented by relaxation incisions under the folds and scalp-reduction techniques.3 More recently, forehead lifting and direct excision of the dermal folds have been described.4 This is the first reported case of an approach using Endotines in combination with mask subperiosteal and lateral SMASectomy facelifts. An open approach was chosen in preference to an endoscopic lift, as it allowed adequate
Correspondence and communication excision of the galea frontalis and scoring of the overlying dermis. The Endotine forehead device is a safe, simple, effective and reliable method of forehead fixation. It avoids recognised problems with metal screws and sutures, which may protrude through the scalp or cause wound irritation and alopecia. The Endotine device is slowly absorbed, and its unique structure provides multiple contact points creating a wider distribution of fixation unlike singlesuture techniques. Our experience has shown that the Endotine device provides a robust fixation, and it is also possible to correct asymmetry and redrape the tissues over the device to improve the aesthetic result in the postoperative period. Fixation techniques that transfer the tension of the suspension to the scalp incisions may result in alopecia. Complications with the Endotine device have been noted.5 Prior to absorption, the device may be palpable or tender in thin-skinned individuals. There may be a need for repositioning owing to asymmetry, or the skin may detach from the Endotine prongs. It is rare for the device to extrude. In conclusion, the use of a combined approach using both mask subperiosteal and SMAS face-lifting techniques together with the resorbable Endotine devices allows reliable elevation and fixation, and treatment of both the
e513 galea frontalis and dermis, thus leading to an improved aesthetic outcome.
References 1. Castori M, Sinibaldi L, Mingarelli R, et al. Pachydermoperiostosis: an update. Clin Genet 2005;68:477e86. 2. Shuster MM, Lewin ML, Caplan L. Facial deformity in pachydermoperiostosis; idiopathic hypertrophic osteoarthropathy. Plast Reconstr Surg 1965;35:666e74. 3. Garden JM, Robinson JK. Essential primary cutis verticis gyrata. Treatment with the scalp reduction procedure. Arch Dermatol 1984;120:1480e3. 4. Kara IG. Forehead lifting for cutis verticis gyrata. Plast Reconstr Surg 2003;111:1777e8. 5. Newman J. Safety and efficacy of midface-lifts with an absorbable soft tissue suspension device. Arch Facial Plast Surg 2006;8:245e51.
L.N. Suleman Verjee A.V.H. Greig W.N.A. Kirkpatrick Craniofacial Unit, Chelsea & Westminster Hospital, NHS Trust, 369 Fulham Road, London SW10 9NH, UK E-mail address: [email protected]