Herlyn-Werner-Wunderlich syndrome: A fertility-sparing approach to a rare mullerian anomaly.

The Herlyn-Werner-Wunderlich syndrome (HWWS) is characterized by the triad of uterus didelphys, obstructed hemivagina, and renal agenesis. The typical clinical presentation involves chronic pelvic pain, dysmenorrhea, and palpable abdominal mass, related to hematocolpos/hematometra. It is a rare disease, with a challenging clinical and radiological diagnosis. Surgery is the definitive treatment. Complications such as endometriosis, infertility and chronic pelvic pain occur more frequently and severely when diagnosis and treatment are delayed. This is a case report of a twelve-year-old patient admitted to the Gynecology Department of the Federal University of Rio de Janeiro's General Hospital (HUCFF/UFRJ), in March 2021, with progressive symptoms of dysmenorrhea and abdominal distention due to palpable abdominal mass. She had a previous history of congenital solitary kidney. Magnetic Resonance Imaging (MRI) showed a double uterus with hematometra and hematocolpos on the left side, pelvic endometriosis and left renal agenesis. Conservative clinical treatment with inhibition of the hypothalamic-pituitary-ovarian (H-P-O) axis was initiated while a definitive surgical approach was being defined. In June 2022, the patient underwent left hemi-hysterectomy and salpingectomy, achieving full remission of symptoms. Given the rarity of this syndrome and its potential complications, our report aims to familiarize clinicians with it, mostly those who work with children and adolescents, so that more patients have access to early diagnosis and adequate treatment. Consequently, future fertility can be effectively preserved.

Plastic surgery in chest wall reconstruction: relevant aspects - case series.

OBJECTIVE: to discuss the participation of Plastic Surgery in the reconstruction of the chest wall, highlighting relevant aspects of interdisciplinaryness. METHODS: we analyzed charts from 20 patients who underwent extensive resection of the thoracic integument, between 2000 and 2014, recording the indication of resection, the extent and depth of the raw areas, types of reconstructions performed and complications. RESULTS: among the 20 patients, averaging 55 years old, five were males and 15 females. They resections were: one squamous cell carcinoma, two basal cell carcinomas, five chondrosarcomas and 12 breast tumors. The extent of the bloody areas ranged from 4x9 cm to 25x40 cm. In 12 patients the resection included the muscular plane. In the remaining eight, the tumor removal achieved a total wall thickness. For reconstruction we used: one muscular flap associated with skin grafting, nine flaps and ten regional fasciocutaneous flaps. Two patients undergoing reconstruction with fasciocutaneous flaps had partially suffering of the flap, solved with employment of a myocutaneous flap. The other patients displayed no complications with the techniques used, requiring only one surgery. CONCLUSION: the proper assessment of local tissues and flaps available for reconstruction, in addition to the successful integration of Plastic Surgery with the specialties involved in the treatment, enable extensive resections of the chest wall and reconstructions that provide patient recovery.

Plastic surgery in chest wall reconstruction: relevant aspects - case series / A cirurgia plástica na reconstrução da parede torácica: aspectos relevantes - série de casos

Objective: to discuss the participation of Plastic Surgery in the reconstruction of the chest wall, highlighting relevant aspects of interdisciplinaryness. Methods: we analyzed charts from 20 patients who underwent extensive resection of the thoracic integument, between 2000 and 2014, recording the indication of resection, the extent and depth of the raw areas, types of reconstructions performed and complications. Results: among the 20 patients, averaging 55 years old, five were males and 15 females. They resections were: one squamous cell carcinoma, two basal cell carcinomas, five chondrosarcomas and 12 breast tumors. The extent of the bloody areas ranged from 4x9 cm to 25x40 cm. In 12 patients the resection included the muscular plane. In the remaining eight, the tumor removal achieved a total wall thickness. For reconstruction we used: one muscular flap associated with skin grafting, nine flaps and ten regional fasciocutaneous flaps. Two patients undergoing reconstruction with fasciocutaneous flaps had partially suffering of the flap, solved with employment of a myocutaneous flap. The other patients displayed no complications with the techniques used, requiring only one surgery. Conclusion: the proper assessment of local tissues and flaps available for reconstruction, in addition to the successful integration of Plastic Surgery with the specialties involved in the treatment, enable extensive resections of the chest wall and reconstructions that provide patient recovery.

Objetivo: discorrer sobre a participação da Cirurgia Plástica na reconstrução da parede torácica, ressaltando os aspectos relevantes das associações interdisciplinares. Métodos: foram analisados prontuários de 20 pacientes submetidos a extensas ressecções do tegumento torácico, no período entre 2000 e 2014, quanto à indicação das ressecções, à extensão e à profundidade das áreas cruentas, aos tipos de reconstruções realizadas e às complicações. Resultados: entre os 20 pacientes, com média de 55 anos de idade, cinco eram do sexo masculino e 15 do feminino. Foram ressecados: um carcinoma espinocelular, dois carcinomas basocelulares, cinco condrossarcomas e 12 tumores de mama. A extensão das áreas cruentas variou de 4x9 cm até 25x40 cm. Em 12 pacientes as ressecções abrangeram o plano muscular. Nos oito restantes, a retirada do tumor atingiu a espessura total da parede. Para reconstrução foram utilizados: um retalho muscular associado à enxertia de pele, nove retalhos miocutâneos e dez retalhos fasciocutâneos da região. Em dois pacientes submetidos à reconstrução com retalhos fasciocutâneos houve sofrimento parcial do retalho, resolvido com o emprego de retalho miocutâneo. Nos outros pacientes não houve intercorrências com as técnicas empregadas, sendo necessária somente uma cirurgia. Conclusão: a adequada avaliação dos tecidos locais e dos retalhos disponíveis para a reconstrução, além da boa integração da Cirurgia Plástica com as especialidades envolvidas no tratamento, possibilitam extensas ressecções da parede torácica e reconstruções que propiciam a recuperação do paciente.

Sentinel lymph node identification in breast cancer: a comparison study of deep versus superficial injection of radiopharmaceutical.

OBJECTIVES: Sentinel lymph node biopsy (SLNB) has been performed for purposes of disease staging. SLN is usually located in the axillary region and internal mammary chain (IMC). Metastasis in internal mammary nodes can be an important prognostic factor and an indication for systemic treatment in patients with small carcinomas. The SLNB technique continues to evolve and the proper radiopharmaceutical injection route remains under discussion. This study evaluated the success rate of deep injection to identify axillary and extra-axillary SLNs and compared the results with superficial injection technique. METHODS: Forty-six patients diagnosed with breast cancer (stages I and II) were submitted to radiopharmaceutical injection. Deep injection of technetium-99m-dextran 500 was carried out in 20 patients (group A) and periareolar injection of technetium-99m-phytate was carried out in 26 patients (group B). All SLNs were studied by imprint cytology and hematoxylin and eosin staining. RESULTS: SLN identification rate was 76.1% (35 of 46). The SLN identification rate was 75% (15 of 20) for group A and 76.9% (20 of 26) for group B. Axillary SLNs were identified in 65% (13 of 20) of group A and 76.9% (20 of 26) of group B, with no statistical difference (P = 0.75). Extra-axillary SLNs were only identified in group A, and IMC was the principal extra-axillary location. CONCLUSION: Deep injection of radiopharmaceutical achieved a good SLN identification rate in axillary and extra-axillary locations and it is an important method for detecting IMC sentinel nodes.

Educação a distância mediada pela internet: "Linfonodo sentinela, prevenção, diagnóstico precoce e biópsia - nova técnica de abordagem do câncer de mama" / Internet-conducted distance education: sentinel lymph node, prevention, early diagnosis and biopsy – a new technique for approaching breast cancer

OBJETIVO: O objetivo deste estudo é o desenvolvimento e a aplicação de um curso na modalidade "Educação a distância mediada pela internet". MATERIAIS E MÉTODOS: Foi utilizado o curso "Linfonodo sentinela, prevenção, diagnóstico precoce e biópsia - nova técnica de abordagem do câncer de mama" como modelo de aplicação. O material didático para a modalidade "Educação a distância" foi elaborado visando a um público composto por médicos envolvidos com o tratamento do câncer de mama. O curso foi estruturado em ambiente virtual de aprendizagem, um espaço virtual que permitiu a interação entre os participantes. RESULTADOS: A duração do curso foi de 12 semanas. Iniciou-se com nove participantes, médicos ginecologistas com pelo menos oito anos de experiência profissional. Todos os alunos participaram de alguma forma, dois realizaram exercícios e interagiram. O alcance do curso pelo método atingiu quatro estados e oito municípios. Não ocorreu adesão integral dos alunos, apesar de a maioria permanecer até o fim do curso. Possivelmente, não houve motivação suficiente para participação nas atividades propostas. CONCLUSÃO: Os resultados mostram que é necessário quebrar as barreiras da falta de cultura relacionada a esta forma de aprendizagem. É fundamental a participação facilitadora do coordenador para integração e mobilização dos participantes.

OBJECTIVE: The present study was aimed at developing and delivering a course in the modality "Internet-conducted distance education". MATERIALS AND METHODS: The theme "Sentinel lymph node, prevention, early diagnosis and biopsy - a new technique for approaching breast cancer" was utilized as an application model, and the didactic material for distance-learning was targeted to a selected audience of physicians involved in the treatment of breast cancer. The course was structured in a virtual learning environment, allowing interaction among participating students. RESULTS: The coursework took place over a 12-week period. Nine gynecologists with at least eight years of professional experience participated in some way in the course, but only two of them accomplished exercises and interacted. In the distance-education modality, the course reached eight cities in four Brazilian states. A full engagement did not occur, although most of participants have remained until the end of the course. Possibly, there was a lack of motivation. CONCLUSION: The results demonstrate that it is necessary to break the barriers to the culture related to this form of learning. A pro-active participation of the coordinator is essential for integrating and mobilizing the participants.

Identificação do linfonodo sentinela no câncer de mama com injeção subdérmica periareolar em quatro pontos do radiofármaco / Sentinel lymph node identification in breast cancer using periareolar and subdermal injection of the radiopharmaceutical in four points

Este estudo visa identificar o linfonodo sentinela por meio da injeção exclusiva de radiofármaco periareolar subdérmico em quatro pontos, independente da topografia do tumor. A biópsia do linfonodo sentinela diminui a morbidade no estadiamento da axila. Foram realizadas 57 biópsias do linfonodo sentinela, em pacientes com câncer de mama, prospectivamente, em dois grupos: grupo A (25 pacientes) e grupo B (32 pacientes). Realizamos a injeção do radiofármaco peritumoral no grupo A, e nova técnica periareolar em quatro pontos no grupo B. A biópsia do linfonodo sentinela foi estudada por "imprint" citológico e hematoxilina e eosina, seguida de linfadenectomia axilar no grupo A e nos casos positivos do grupo B. No grupo A foram identificados 88 por cento (22/25) de linfonodos sentinelas, não houve falso-negativo, com sensibilidade e especificidade de 100 por cento; no grupo B foram identificados 96 por cento (31/32) de linfonodos sentinelas e valor preditivo positivo de 100 por cento. O número de linfonodos sentinelas variou de 1 a 7, moda de 1 e média de 2,7, a área de maior captação variou de 10 a 100 vezes. A injeção periareolar em quatro pontos se apresenta como bom método no mapeamento linfático para identificação do linfonodo sentinela. A padronização deste sítio pode ser o de escolha para identificação do linfonodo sentinela, sendo necessário maior número de casos para confirmação destes achados.

The aim of this study was to identify the sentinel node by periareolar injection of the radiopharmaceutical in four points, regardless of tumor topography. The sentinel node biopsy reduces morbidity in axillary staging. Fifty-seven sentinel node biopsies were prospectively performed in two groups: group A (25 patients) and group B (32 patients). The peritumoral injection technique was used in group A and the new injection technique in four points was used in group B. The sentinel node biopsies were studied by imprint cytology and hematoxilin and eosin staining followed by axillary lymph node dissection in all patients of group A and only in the positive cases of group B. In group A, 88% (22/25) of the sentinel nodes were identified. There was no false negative case; the sensibility and specificity were of 100%. In group B, 96% (31/32) of sentinel nodes were identified and the status of the axillary lymph nodes showed a predictive positive value of 100%. The number of sentinel nodes varied from 1 to 7, mode of 1 and median of 2.7. The hotspot area was 10 to 100 times the background radiation. The periareolar injection in four points seems to be a good lymphatic mapping method for identification of the sentinel node. We suggest the standardization of this site for injections to identify the sentinel node, although further studies to confirm these findings are necessary.

Mastopatia diabética / Diabetic mastopaty

A mastopatia diabética é uma doença rara fibroinflamatória da mama, decorrente da reação auto-imune de acúmulo anormal de proteínas de matriz, causada pela hiperglicemia. Ocorre em pacientes diabéticas de longa data, principalmente as do tipo 1 e que se encontram na pré-menopausa. Aqui se relata um caso de mastopatia diabética em uma mulher de 71 anos de idade, com história de diabetes melito tipo 2, insulino-dependente, de 20 anos de duração, que tinha sido submetida a uma cirurgia plástica mamária aos 35 anos de idade. O exame físico mostrava uma massa endurecida, pouco definida e móvel no quadrante inferior externo, medindo 2 cm. Não possuía expressão mamográfica ou ultra-sonográfica. A biópsia excisional do nódulo e subseqüente exame histopatológico revelaram lobulite linfocítica esclerosante ou mastopatia diabética. O exame clínico pode mimetizar neoplasia maligna de mama e o diagnóstico histopatológico deve ser obrigatório para diagnóstico diferencial.

Diabetic mastopathy is a rare fibroinflammatory breast disease decurrent of autoimmune reaction to the accumulation of abnormal matrix proteins, caused by hyperglycemia. It occurs in long-standing insulin dependent, mainly with type I diabetes, and pre-menopause patients. We present a case of diabetic mastopathy in a 71-year-old woman with a history of insulin-dependent diabetes mellitus type 11 of 20 years duration who has done a mammary plastic surgery. Physical examination showed a stony-hard, ill-defined but freely movable mass at lower outer quadrant measuring 2 cm. It didn't have mammographic netheir ultrassonographic findings. Excisional biopsy of the nodule was performed and the histopathological examination of the lesion revealed sclerosing lymphocitic lobulitis or diabetic mastopathy. Clinical and ultrassonographic exams mimic breast cancer and for this reason the histological diagnosis must be useful for deferential diagnosis.