Autologous Fat Grafting with Percutaneous Fasciotomy and Reduction of the Nipple-Areolar Complex for the Correction of Tuberous Breast Deformity in Teenagers.

BACKGROUND: Tuberous breast deformity in the adolescent can be a source of anxiety and social isolation. Current techniques of implant placement and flap reconstruction are not always feasible in this population. OBJECTIVES: The authors evaluated the use of autologous fat grafting with percutaneous fasciotomy and reduction in the nipple-areolar complex for correction of tuberous breast deformity in teenagers. METHODS: A retrospective chart review was conducted for nine teenaged patients with tuberous breast deformity who received autologous fat grafting between January 2016 and December 2018. The recipient site was prepared with the use of percutaneous fasciotomies to release the constricted lower pole of the breast, lowering of the inframammary crease, and reduction in the nipple-areolar complex. Fat was harvested by conventional liposuction prior to injection through three designated sites located at the inframammary fold, anterior axillary line, and upper pole. Complications were recorded. RESULTS: Patients had an average age of 14.9 years at the time of surgery. An average of 1.8 filling sessions were required with a mean of 220 cc of fat injected per breast. Patients were followed for an average of 21 months postoperatively. No serious complications were noted. All patients reported satisfaction with their final outcomes. CONCLUSIONS: Autologous fat grafting in conjunction with percutaneous fasciotomy and reduction in the nipple-areolar complex is a safe and effective treatment of the tuberous breast deformity in teenage patients. It provides an esthetic result with minimal scarring and high satisfaction rates while eliminating the need for flaps or implants. 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.

Long-term follow-up of myelomeningocele defect closure with dorsal intercostal artery perforator propeller flaps.

BACKGROUND: Closure of the skin defect in myelomeningocele repair determines the quality of the surgical result. In large myelomeningoceles, the direct closure or by skin undermining may not be possible. In these cases, the skin defect must be closed using flaps to provide a tension-free and successful closure. METHODS: In this procedure, after neurosurgical repair and closure of the placode, the defect surgically becomes an ovoid in shape. Then, this defect is closed by transposition of two dorsal intercostal artery perforator propeller flaps. The defect size was > 5 cm in diameter. The localization of the lesion was lumbosacral. RESULT: A successful tension-free one-stage closure was obtained, without necrosis. Healing was uneventful without any complications and the coverage remained stable over 9 years with the child growth. CONCLUSION: The dorsal intercostal artery perforator propeller flap enables the surgeon to achieve a tension-free defect closure of considerably large myelomeningocele defects. This type of closure provides a durable coverage and a soft tissue padding over the neural tissues that persist with the child growth. The dorsal intercostal artery perforator propeller flap seems to be a useful closure of large myelomeningocele defects.

Síndrome GAPO, a propósito de un caso / GAPO syndrome: case report

El síndrome GAPO es una rara enfermedad autosómica recesiva caracterizada por retraso en el crecimiento, alopecia, pseudoanodoncia y atrofia óptica. Se han descrito mutaciones en el gen ANTXR1 como origen etiológico. Presenta afectación de múltiples aparatos, por lo que requiere un manejo multidisciplinar para lograr su adecuado tratamiento.

GAPO syndrome is a rare autosomal recessive disease characterized by growth retardation, alopecia, pseudoanodontia and optic atrophy. Gene alterations in the ANTXR1 gene have been reported to be causative of this disorder. Abnormalities of diverse organs and systems have been described. A multidisciplinary management to achieve an adequate treatment is required.

Dermatofibrosarcoma protuberans en pacientes pediátricos / Dermatofribosarcoma protuberans in pediatric patients

Antecedentes y Objetivos. Presentamos 3 casos de dermatofibrosarcoma protuberans (DFSP) en pacientes pediátricos para documentar la presentación clínica poco frecuente de esta patología. Pacientes y Método. Realizamos un análisis descriptivo retrospectivo de los 3 últimos casos de DFSP en nuestro centro durante los años 2010 - 2013. Resultados. Una niña de 12 años remitida desde otra institución por DFSP en parte distal del muslo, en la que ampliamos bordes quirúrgicos de resección en 3 cm y practicamos cobertura con dermis artificial e injerto autólogo; tras 1 año de observación sin recidiva, realizamos reconstrucción del defecto mediante injertos autólogos de grasa. Otra niña de 12 años diagnosticada de Síndrome de Hamartomatosis Múltiple asociado al gen PTEN, que presentó DFSP en labio mayor derecho y en mama derecha, recidivante en esta última localización. Por último, un DFSP congénito en una paciente remitida a los 3 meses de vida con tumoración gigante de pared abdominal. El diagnóstico orientativo inicial con resonancia magnética con gadolino, solo se confirmó finalmente por los hallazgos histopatológicos de la pieza de resección; ni la punción aspiración ni la biopsia cutánea lograron confirmar el diagnóstico. Conclusiones. El DFSP es un tipo de sarcoma raro de la piel del niño y del adulto, localmente agresivo y con una alta tasa de recidiva. La escisión local amplia puede provocar mutilaciones y desfigurar al paciente; por ello la cirugía micrográfica de Mohs permite reducir los márgenes quirúrgicos (AU)

Background and Objectives. We present 3 cases of dermato fibro sarcoma protuberans (DFSP) to document the unusual clinical presentation of this condition in pediatric patients. Patients and Methods. We conduct a retrospective descriptive analysis of the last 3 cases of DFSP treated in our institution during years 2010-2013. Results. A 12-year-old girl referred from another institution for DFSP in distal thigh; we practised surgical margins extended 3 cm resection and cover with artificial dermis and autologous graft; after 1 year follow up without recurrence, reconstruction of the defect was performed using autologous fat grafts. Another 12-year-old girl diagnosed of Multiple Hamartoma Syndrome associated with PTEN gene, who presented DFSP at the right labia major and at the right breast, being recurrent in this last location. Finally, a congenital DFSP in a patient referred at 3 months with giant tumour of abdominal wall. The initial diagnosis was made by MRI with gadolinium. The final diagnosis of DFSP was made based on histopathological findings of the piece following surgical resection, because percutaneous biopsy or skin biopsy couldn´t confirm the diagnostic. Conclusions. DFSP is a rare type of sarcoma of the skin in children and adults, locally aggressive, with a high rate of recurrence. Wide local excision may cause maiming and disfiguring to the patient, thereby Mohs micrographic surgery (CMM) enables to reduce surgical margins (AU)