Clinical Outcomes of Utilizing a "W"-shaped Incision in the Management of Forehead Skin Thickening Induced by Hypertrophic Osteoarthropathy.

BACKGROUND: Hypertrophic osteoarthropathy (HOA) is a rare and intricate hereditary disease. The appearance and functional deformity of the forehead caused by thickened folds are the main clinical manifestations of patients with hypertrophic osteoarthropathy. The cause of this disease is still unknown. Currently, surgical treatment has become one of the best strategies, mainly for improving the appearance of the forehead. There has been no literature report on the use of "W"-shaped skin flap resection for thickened forehead skin in patients with hypertrophic osteoarthropathy. METHODS: All cases of hypertrophic osteoarthropathy in our department in the last 7 years, and previous literature on hypertrophic osteoarthropathy, were reviewed. RESULTS: A total of 5 cases of hypertrophic osteoarthropathy in our department (mean age 21 years, all male patients) were reviewed. All patients underwent open surgery to remove the thickened skin on the forehead or the wrinkles and gyrus-shaped scalp. The jagged skin tissue was removed (8-9) cm × (1-2.5) cm × 0.5 cm. The folds and thickness of the frontal skin of the patients were greatly improved after the operation. Patient satisfaction with the treatment outcomes was unanimous. However, one case experienced a postoperative wound infection during follow-up. The utilization of the "W"-shaped excision technique allowed for the maximal removal of excessively diseased tissue, thereby facilitating a smoother resolution of the depression. CONCLUSIONS: A total of 5 cases of hypertrophic osteoarthropathy were treated in our department, and all of them underwent frontal skin "W"-shaped excision, which was safe, feasible, and practical, and the postoperative results were satisfactory. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Juvenile Disease Processes Affecting the Forelimb in Canines.

Several juvenile diseases affect the canine forelimb. The most common are hypertrophic osteodystrophy, panosteitis, and retained cartilaginous core. Panosteitis and hypertrophic osteodystrophy tend to be self-limiting, with a good long-term prognosis, although severe cases can develop. These diseases may recur during growth. Severe cases of hypertrophic osteodystrophy can lead to angular limb deformities and may even be fatal. Retained cartilaginous cores can be benign with no evidence of clinical signs and be found incidentally on radiographs. However, if they disrupt the distal ulnar physis, angular limb deformities may persist requiring surgical intervention with a corrective osteotomy.

Feet Deformity and Gait Disturbance in a Patient with Pachydermoperiostosis (PDP). Case Study.

Pachydermoperiostosis is a rare condition representing a primary form of hypertrophic osteoarthropathy. It presents in different stages. Patients often overlook early symptoms, because they are benign. The most common manifestations are clubbing of the fingers and toes, skin thickening with characteristic folds on the face and head and widening of joints accompanied by radiological changes. Surgical treatment is not often needed, and, consequently, there are no strict guidelines on surgical management, which is mainly based on case report ana-lysis. This paper presents a case of surgical management of pachydermoperiostosis.

Comprehensive Surgical Strategies for the Management of Pachydermoperiostosis.

The cosmetic and functional deformities induced by thickened folds and furrows are the main complaints of patients in pachydermoperiostosis (PDP). However, traditional drug therapy only has a limited effect for its unclear genetic pathogenesis. Surgical treatment has now become one of the optimal strategies mainly aiming for its cosmetic improvement. Here we describe a comprehensive management of PDP approached with surgical procedure thereby showing markedly improved cosmetic outcome. Experientially, the surgical treatment of PDP is effective and may be preferentially recommended for PDP patients especially of severe degree.

Hypertrophic osteoarthropathy as a complication of pulmonary tuberculosis / Osteoartropatía hipertrófica como complicación de la tuberculosis pulmonar

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Pachydermoperiostosis: aesthetic treatment of prematurely aging face with facelift and botulinum toxin a.

Pachydermoperiostosis is a rare syndrome that hinders patients' quality of life thru its aesthetics manifestations and functional obstacles. Many techniques for addressing and correcting aesthetic defects associated with pachydermoperiostosis have been introduced, including facelift surgery. This case presentation includes treatment of facial pachydermoperiostosis and restoration of facial aesthetics via treatment with facelift, skin muscle excision, and botulinum toxin A.

Tratamento cirúrgico da paquidermoperiostose primária - relato de dois casos / Surgical Treatment of Primary Pachydermoperiostosis: Report of Two Cases / Surgical Treatment of Primary Pachydermoperiostosis: Report of Two Cases

A paquidermoperiostose primária é uma doença rara, caracterizada por aposição excessiva do periósteo do crânio, coexistindo com espessamento da epiderme e derme (paquidermia), provocando deformidades grosseiras. Devido à diversidade de estruturas acometidas, há várias opções cirúrgicas e métodos complementares que são utilizados no tratamento das alterações faciais desses pacientes. Esse trabalho apresenta o lifting subperiosteal como uma opção de tratamento estético para a face de pacientes portadores dessa síndrome, através do relato de dois casos operados no Hospital das Clínicas da Universidade Federal de Minas Gerais.

Primary pachydermoperiostosis is a rare disease characterized by excessive skull affixing of the periosteum, coexisting with thickening of the epidermis and dermis (pachydermia), thereby causing gross deformities. Owing to the variety of affected structures, there are several surgical options and complementary methods that are used in the treatment of facial alterations in these patients. This report describes the use of subperiosteal detachment as an aesthetic treatment option for the faces of two patients with primary pachydermoperiostosis, operated at the Hospital das Clínicas of the Federal University of Minas Gerais.

Pachydermoperiostosis in an African patient caused by a Chinese/Japanese SLCO2A1 mutation-case report and review of literature.

OBJECTIVES: Pachydermoperiostosis is a rare clinical entity characterized by skin thickening of the forehead, eyelids, and hands, digital clubbing, and periostosis. Two genes have been associated, HPGD and recently SLCO2A1. We present a detailed clinical and genetic description of an African pachydermoperiostosis patient with a SLCO2A1 mutation. METHODS: Standard clinical and laboratory evaluation was carried out. Genetic screening was done with PCR followed by direct sequencing. We discuss the clinical features and known mutations of previously reported cases identified through a PubMed literature review. RESULTS: The clinical findings showed special features, including exuberant knee effusions and an extraordinary good response on surgery of the blepharoptosis. We found a splice site mutation in the SLCO2A1 gene in homozygous form: c.940+1G>A. This mutation was previously reported only in 1 Chinese and 3 Japanese cases and was considered as a founder mutation in Japan. Beside our case, only one other patient in the literature carried this mutation in homozygous condition, but with different main clinical symptoms. CONCLUSIONS: Our case demonstrates phenotypic heterogeneity of PDP even between homozygous carriers of the same mutation, suggesting further modifiers. Besides, it shows that this rare SLCO2A1 mutation is not exclusively present in East-Asia, but can occur in various ethnicities, with different origin, thus the incidence is probably underestimated.

Cardiac metastasis and hypertrophic osteoarthropathy in recurrent infantile fibrosarcoma.

Cardiac metastasis and hypertrophic osteoarthropathy are both quite rare. We describe a patient presenting with hypertrophic osteoarthropathy as the first symptom of recurrent infantile fibrosarcoma (IF). During surgical resection of lung metastasis, the patient suffered sudden cardiac arrest. Autopsy demonstrated a metastatic lesion in the intraventricular septum of the heart, which is previously undescribed in the literature. This case demonstrates that IF can be aggressive despite its more typical benign course.

Artropatía de Charcot secundaria a tabes dorsal: reporte de un caso / Charcot`s arthropathy due to tabes dorsalis: case report

El Tabes Dorsal (TD) es una enfermedad toxico degenerativa donde hay afectación de las raíces, los ganglios y los cordones posteriores de la médula espinal por la espiroqueta de la sífilis, como manifestación tardía de la enfermedad. La TD es muy rara en nuestro medio. El 5 a 10 % de los pacientes presentan una Osteoartropatía Neuropática, que afecta de forma predominante a las articulaciones de las extremidades inferiores. Se presenta el caso de un paciente masculino de 29 años de edad, con diagnóstico clínico y de laboratorio de sífilis, el cual inicia su enfermedad actual en febrero de 2003 con trastornos de la marcha, acompañado de artralgias progresivas y espasticidad en miembros inferiores. Al examen físico se encontró marcha con aumento de la base de sustentación, signo de Romberg positivo, arreflexia rotuliana y aquílea. Los estudios radiológicos de ambas rodillas muestran signos importantes de destrucción articular. Se realizó una Artroplastia Total de Rodilla (ATR) izquierda. La ATR se puede ofrecer a un selecto grupo de pacientes con OAN en estadíos finales

The Tabes Dorsalis (TD) are a degenerative toxic disease where there is roots, ganglia and posterior cords affectation of the spinal marrow by the syphili´s spiroquette, as delayed manifestation of the disease. TD is very rare in our means. 5 to 10% of these patients development a Neuropathic osteoarthropathy (NOA), that affects the joints of the lower extremities. We present the case of a 29 years old masculine patient, with clinical and laboratory diagnosis of syphilis, which initiates his disease in February 2003 with march upheavals, accompanied by progressive arthralgias and lower limbs spasticity. To the initial physical examination, there was march with sustenation base increased, lower extremity osteotendonous areflexia and positive Romberg's sign. The radiological studies of both knees show important signs of articular destruction. A left Total Knee Arthroplasty (TKA) was made. TKA can be offered to a select group of patients with NOA in final stages

Frontal rhytidectomy as surgical treatment for pachydermoperiostosis: a case report.

The thickened folded skin of Touraine-Solente-Golé syndrome can result in cosmetic and functional deformities. The treatment of pachydermoperiostosis is usually centered around improving the cosmetic appearance through plastic surgery. We describe the case of a 27-year-old male who had pachydermoperiostosis with a leonine facies that was managed with frontal rhytidectomy. A greatly improved cosmetic appearance was achieved with this procedure.

A case of pachydermoperiostosis treated by oral administration of a bisphosphonate and arthroscopic synovectomy.

Pachydermoperiostosis (PDP) is a rare hereditary disorder characterized by pachydermia, digital clubbing, and periosteal hypertrophy. Here, we report a case of PDP showing symptoms consistent with arthritis, which was treated by oral administration of risedronate sodium and arthroscopic synovectomy.

[Correction of finger clubbing in primary hypertrophic osteoarthropathy (Touraine-Solente-Gole syndrome)]. / Korrektur der Trommelschlegelfinger unter ästhetischen Aspekten bei hereditärer hypertropher Osteoarthropathie (Touraine-Solente-Gole-Syndrom).

Finger clubbing can be a single physical finding. In Touraine-Solente-Gole syndrome, the primary form of hypertrophic osteoarthropathy, it is mostly associated with bone pain, hyperhydrosis, pachydermy and wrinkling of the forehead. In other cases, the presence of clubbing is associated with neoplastic, pulmonary, cardiac or other diseases and is then called Bamberger-Pierre-Marie syndrome, the secondary type of hypertrophic osteoarthropathy. The patient's history and careful physical examination, sometimes accompanied by laboratory and imaging studies, leads to the diagnosis. A patient with hereditary hypertrophic osteoarthropathy and its clinical symptoms is presented. Surgical correction of the clubbing fingers is demonstrated in the paper with bilateral resection and shortening of the nail bed, nail matrix and resection of soft tissue. Clubbing fingers are rare, but they might be part of a syndrome or a symptom of other diseases. Reconstructive surgery for aesthetic reasons can be performed.

Severe complete pachydermoperiostosis (Touraine-Solente-Golé syndrome).

BACKGROUND: The thickened, folded skin of Touraine-Solente-Golé syndrome (pachydermoperiostosis) can result in cosmetic and fuctional deformities. OBJECTIVE: To illustrate that simple surgical procedures can improve cosmetic and functional status. METHODS: Case report of a 33-year-old male with bilateral eyelid ptosis, enlargement of the eyelids, and a leonine facies owing to deep skin furrows managed with bilateral blepharoplasties and tarsal wedge resections, along with excision of multiple skin furrows. RESULTS: A greatly improved cosmetic appearance was achieved with these standard procedures. CONCLUSIONS: The appearance of patients with pachydermoperiostosis can be greatly improved with standard surgical procedures.

[Treatment and pathologic change of pachydermoperiostosis].

OBJECTIVE: To investigate the treatment and possible pathogenesis of pachydermoperiostosis. METHODS: Facelift was performed on five patients with pachydermoperiostosis. Histological studies were performed to check the affected skin for possible pathogenesis. RESULTS: After the surgery of facelift, appearances of the patients were markedly improved. Histological studies revealed significant changes in the skin, characterized by hyperplasia and thickening of the subcutaneous collagen, as well as an increase of PAS-positive substance. CONCLUSION: Alterations of fibroblast bioactivity might be responsible for the pathogenesis of pachydermoperiostosis. There have been no satisfying treatments to the disease. Facelift is a feasible method to improve the aging appearance of the patients.

Síndrome de Touraine Solente Goulé Paquidermoperiostosis primaria: breve revisión y reporte del primer caso en Costa Rica / Syndrome of Tourine Solente Goulé Pachydermoperiostosis primary: brief revision and reports of the first case in Costa Rica

La paquidermoperiostosis es una enfermedad poco frecuente que se caracteriza por un engrosamiento de la piel en el cuero cabelludo, frente y párpados, también ocurre en la piel de manos y pies, con un engrosamiento concomitante de las estructuras óseas de las extremidades. El crecimiento acral hace sospechar acromegalia. Su presentación puede ser idiopática, con inicio en la adolescencia y estabilización en la tercera y cuarta década, es de origen genético, de transmisión autosómico dominante y penetranica variable, o secundaria a enfermedades tumorales, principalmente pulmonares o intratorácicas de inicio en la cuarta y quinta décadas de la vida. El tratamiento para sus dos variedades es quirúrgico, ya sea reconstructivo tratándose de la primaria, con mejoría estética; o excisional en el caso de la secundaria, con regresión total o parcial a la normalidad. Se presenta el primer caso conocido en el ámbito de la medicina costarricense, a fin de dar a conocer esta patología al cuerpo científico y facilitar la detección de otros posibles casos. Esta es una enfermedad poco frecuente en el mundo y hasta ahora desconocida en nuestro país.

The disease known as pachydermoperiostosis is of rare occurrence and is characterized by thickening of the skin folds on the forehead and eyelids and in the extremities. It is also accompanied by a gross thickening of the underlying the bony structures of hands and feet, raising the diagnostic possibility of acromegaly. It has two presentations, idiopathic, which is transmitted in an autosomic and dominant fashion with variability in its penetrance and starts in the teen years and halts progression in the third and fourth decades of life, the secondary type starts in the fourth and fifth decade and its secondary to a pulmonary or intrathoracic tumoral lesion. Treatment consists in either reconstructive surgery for the primary type, with cosmetic acceptable results and surgical excision in the secondary type with partial or complete regression of the disease. We present to our knowledge what is the first reported case in Costa Rica with the purpose of informing the scientific body in our country of the existence of the disease and to help detect any other possible cases.