Body Image Disorders and Surgical Timing in Patients Affected by Poland Syndrome: Data Analysis of 58 Case Studies.

BACKGROUND: Poland syndrome is a congenital anomaly of pectoralis muscles, breast, chest, and upper arm. Several studies have reported that patients affected by chest wall deformities often experience body image disorders and decreased quality of life. Cosmetic corrective surgery is generally postponed until physical development is achieved, and latissimus dorsi flap surgery is usually suggested. This study aims to propose a new surgical timing for these patients. METHODS: Patients affected by Poland syndrome (n = 58) and control patients (n = 50) were included in the study, and their body-related psychopathology was evaluated through the Body Uneasiness Test, a valuable multidimensional tool for the clinical assessment of body uneasiness. The Global Severity Index and several subscales were considered. Age and surgical status were taken into account. RESULTS: Significant differences were detected between Poland syndrome patients and controls with respect to all Body Uneasiness Test subscales. Among subjects aged younger than 20 years, the group of patients not operated on was the most affected, whereas the group of peers after surgery showed scores similar to those detected in the control group. CONCLUSIONS: Surgical planning for patients with Poland syndrome should start in the period of growth to allow proper body image stabilization. Current surgical options allow for reduction of the use of invasive interventions such as the latissimus dorsi flap, in favor of less invasive surgical techniques such as expanders, implants, and autologous fat grafting. This new surgical timing will help to ameliorate problems with physical and mental development. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Síndrome de Poland. A propósito de un caso y revisión de la literatura / Poland syndrome. Case report and review of the literature

El síndrome de Poland es una deformidad congénita poco frecuente, esporádica y de patogenia incierta. Se caracteriza por ausencia/hipoplasia del pectoral mayor, alteraciones de la mano y de la glándula mamaria ipsolateral. Se presenta un caso de un varón con clínica sugestiva de síndrome de Poland en el hemitórax izquierdo, sin alteración de la extremidad superior, aunque asociaba dextrocardia y herniación pulmonar, una relación poco frecuente. Se hace hincapié en la necesidad de contar con un equipo multidisciplinario para su manejo inicial y tratamiento a largo plazo (AU)

The Poland syndrome is a rare, sporadic and congenital deformity with uncertain pathogenesis. It is characterized by absence or hypoplasia of the pectoralis major muscle, malformations of the hand and involvement of the ipsilateral mammary gland. A case of a newborn with clinical manifestations suggestive of Poland syndrome on the left hemithorax, associated dextrocardia and defect pulmonary despite being a rare association. Emphasis on the need for a multidisciplinary team in the initial management and long-term treatment (AU)

Exploring quality of life in Italian patients with rare disease: a computer-aided content analysis of illness stories.

The present study used a narrative-based approach to identify common themes that characterized the illness experience and quality of life of patients affected by rare disease (RD). Textual data were comprised of illness stories written by 32 adult Italian patients (eight men and 24 women), with the following RD diagnoses: amyotrophic lateral sclerosis (n = 12), anorectal atresia (n = 4), Poland syndrome (n = 4), and idiopathic pulmonary hypertension (n = 12). Computer-aided content analysis was performed to detect the main themes (cluster analysis) and latent factors (correspondence analysis) emerging in patients' narratives, and to test their association with gender and diagnosis. Four thematic domains were detected in the textual corpus, which are respectively referred to as: hopelessness (12.74%), need for autonomy (38.43%), search for normalcy (11.89%), and expectations of recovery (36.94%). Three latent factors explained the overall data variance: the relationship with social and medical healthcare providers (F1), adjustment processes to disease and social limitations (F2), and self-beliefs and coping (F3). Some differences were revealed with respect to patient gender and diagnosis. Illness stories highlight the significant relationship of RD patients with healthcare services and their need for a holistic approach because of the lack of effective treatment. Physical limitation and emotional distress do not necessarily seem to overlap for adjustment and quality of life (QoL). Overall, the perception of illness chronicity is likely to affect patients' self-beliefs and coping with more than their feeling of abnormalcy, that is the less salient theme.

Women's quality of life and surgical long-term outcome after breast reconstruction in Poland syndrome patients.

BACKGROUND: In the last decades, several surgical approaches have been used to improve the appearance and quality of life of female Poland syndrome patients. The aim of this study was to analyze the women's quality of life and long-term outcome after breast reconstruction. METHODS: Forty-nine women with Poland syndrome who were treated surgically between 1974 and 2007 received standardized questionnaires to evaluate their quality of life and satisfaction after surgical treatment. RESULTS: Patient response was 65%, with 32 completed questionnaires by 16 women who had pedicled latissimus dorsi myocutaneous (LDM) flaps, 12 with tissue expander or silicone implants, and 4 who had free transverse rectus abdominis myocutaneous (TRAM) flaps. Of these patients, 16 were satisfied or highly satisfied with their postoperative appearance (13 with LDM, 2 with prosthesis, and 1 with TRAM), 16 patients would recommend the same surgery to others under similar circumstances (10 with LDM, 5 with prosthesis, and 1 with TRAM), and 18 patients would choose the same method again (14 with LDM, 3 with prosthesis, and 1 with TRAM). CONCLUSIONS: In our study, we found that satisfactory outcome in breast reconstruction was achieved particularly when using the LDM flap, which remains the only method that recreates the anterior axillary fold. Future studies on reconstruction methods with autogenous tissue (e.g., TRAM, deep inferior epigastric perforator [DIEP] flap) are needed, however, to analyze quality of life and long-term outcome in patients with Poland syndrome.

Unilateral hypoplastic breast in a male-to-female transsexual with Poland syndrome after gender reassignment--reconstructive considerations.

Gender reassignment procedures are performed more frequently nowadays due to a multidisciplinary approach and improved techniques and selection process. Many male-to-female patients require bilateral breast augmentation as part of the transformation following the gender reassignment if they fail to develop female breast features after hormonal treatment. We report on a very rare incidence of male-to-female gender reassignment in a patient with Poland syndrome. A male-to-female transsexual on hormonal therapy for gender reassignment developed one normal female-shaped breast whereas the other breast remained hypoplastic. As a male, he was not aware of his chest wall deformity but it became a major issue after successful gender reassignment surgery. Our experience with the specific reconstructive considerations and recommendations regarding our surgical approach to this complex reconstructive problem are discussed.