Opções terapêuticas no manejo do nevus congênito gigante, experiência do Serviço de Cirurgia Plástica e Reaparadora HC-UFPR / Therapeutic options in the management of giant congenital nevus: Experience of the Plastic and Reconstructing Service at Hospital de Clínicas, Federal University of Paraná

INTRODUÇÃO: O nevus congênito gigante (NCG) possui diversas modalidades de tratamento. A abordagem cirúrgica inclui a ressecção parcial seriada ou excisão total. Objetivo: Demonstrar as principais modalidades terapêuticas utilizadas para o tratamento do NCG e avaliar a incidência da localização, idade e tamanho destas lesões no Serviço de Cirurgia Plástica e Reparadora do Hospital de Clínicas da Universidade Federal do Paraná. MÉTODOS: Estudo retrospectivo dos pacientes submetidos a tratamento cirúrgico do NCG no período de janeiro de 2004 a janeiro de 2010. Foram coletados dados como: idade, gênero, tratamento realizado, número de cirurgias realizadas, evolução e complicações. RESULTADOS: Foram avaliados 11 pacientes, sendo 8 mulheres e 3 homens. A média de idade foi de 12,4 anos (3 a 25 anos). O subtipo mais encontrado foi o nevus melanocítico intradérmico correspondendo a 90,9% dos casos e 9,1% com nevus melanocítico composto. Não foram identificados casos de melanoma. A localização mais comum foi a região da cabeça e pescoço. O diâmetro médio das lesões foi de 9,1 cm.As técnicas utilizadas para reconstrução foram: sutura primária, retalhos locais, enxerto de pele e uso de expansores. No seguimento, 63,6% dos pacientes apresentavam ainda nevus residual, 27,3% apresentaram ressecção completa e 9,1% perderam seguimento. CONCLUSÃO: Foi observada maior incidência de NCG na faixa etária de 3 a 25 anos, sendo a localização mais comum na face e com tamanho médio de 9,1cm. O principal tratamento instituído para os pacientes com NCG foi a ressecção parcelada, com bons resultados.

INTRODUCTION: Several modalities are available for the treatment of giant congenital nevus (GCN). The surgical approach includes partial serial resection or total excision. Objective: To demonstrate the main therapeutic modalities in the treatment of GCN and to assess the incidence of location, age, and size of this lesion at the Plastic and Reconstructive Surgery Service of the Hospital de Clínicas, Federal University of Paraná. METHODS: This retrospective study included patients who had undergone surgical treatment for GCN between January 2004 and January 2010. We collected data such as age, sex, treatment performed, number of surgeries carried out, evolution, and complications. RESULTS: We evaluated 11 patients (8 female and 3 male). The average age was 12.4 years (range, 3-25 years). The GCN subtype most commonly found was intradermal melanocytic nevus, which accounted for 90.9% of cases, with melanocytic nevus accounting for the remaining 9.1%. No cases of melanoma were identified. The most common location was the head and neck. The average diameter of the lesions was 9.1 cm. The techniques used for the reconstruction included primary suture, local flaps, skin graft, and the use of expanders. In the follow-up period, 63.6% of the patients still presented a residual nevus, 27.3% underwent complete resection, and 9.1% were not monitored. CONCLUSION: A higher incidence of GCN was observed in patients aged 3-25 years. The most common location was the face, and the average size was 9.1 cm. The main treatment of patients with GCN was splitting resection, which resulted in satisfactory outcomes.

Total forehead flap: hemicraniofacial giant congenital melanocytic nevus.

Being non-hair-bearing and relatively thin and having similar color and texture of the skin of the forehead provide an excellent characteristic not only for nasal reconstruction but also in other facial areas. A 28-year-old man presented in the Department of Plastic Surgery, Argerich Hospital, Buenos Aires, Argentina, with a giant congenital melanocytic nevus extended on complete left cheek, total nose, both upper and lower lids, ocular globe, and temporoparietal region. Tumor resection and reconstructive aspect were planned in 3 stages. (1) Excision of the tumor located in the cheek, nose, and both upper and lower left lids. The reconstruction of the cheek was made with a total forehead flap. The nose was resurfaced by means of a total skin graft. (2) resection of the temporoparietal nevus. (3) Treatment of the tumor involving the ocular structures. Total forehead flap was outlined, permitting carryout of skin of the total forehead area, and the blood supply was originated from the frontal and parietal branches of the temporal superficial artery and secondarily by anastomosis with the branches arising from both homolateral auricular and occipital vessels. Postoperative control 1 year after surgery showed an excellent aesthetic and functional result not only in the recipient area but also in the donor area.

Prenatal diagnosis of a large fetal cervical teratoma by three-dimensional ultrasonography: a case report.

CONTEXT: The cervical teratomas are rare, benign tumors, they are formed by the three embryonic layers, and they represent only 6% of teratomas. The prognosis depends mainly on the size and location of the lesion, on the tumor growth rate, and on the level of tracheal compression. Prenatal diagnosis is usually reached with the aid of a two-dimensional ultrasonography (2DUS) after the 15th week of gestation, which shows a large heterogeneous mass in the cervical region, plus a polyhydramnios, on the Doppler mode which also shows the vascularization of the tumor. CASE REPORT: We report a case of cervical teratoma diagnosed on the 31st week of gestation with the aid of a 2DUS and color Doppler mode. We focus on the main findings obtained with the three-dimensional ultrasonography in surface mode, and we highlight the importance of this methodology for the indirect evaluation of the neonatal prognosis.

Anomalías congénitas del aparato branquial: estudio de imágenes / Congenital anomalies of the branchial apparatus: imaging study

: The branchial apparatus consists of six arches that are separated from each other by branchial clefts and pharyngeal pouches. Its development is complex and multiple congenital defects can occur; including branchial cleft cysts, sinuses and fistulas, thymic cysts, aberrant thymic tissue and parathyroid cysts. The majority arises from the second branchial arch and they correspond to vestigial remnants from incomplete obliteration of the branchial apparatus or buried epithelial cell rests. Imaging studies are very helpful in the diagnosis and characterization of these anomalies, and require a good understanding of the embryogenesis. Considering the anatomical location and the radiological appearance the diagnosis could be precised in most cases. The purpose of this presentation is to show the appearance of some of the most frequent congenital anomalies of the branchial apparatus using different imaging modalities and emphasizing the normal embryologic development.

El aparato branquial, formado por seis arcos separados por hendiduras extemas y bolsas faríngeas en su parte interna, tiene un complejo desarrollo del cual pueden derivar anomalías como senos, fístulas o quistes y alteraciones del timo o glándula paratiroi-des. La mayoría de las malformaciones derivan del segundo componente branquial. Los estudios de imagen son útiles para el diagnóstico y caracterización de estas lesiones que será más exacta con un adecuado conocimiento del desarrollo embriológico. El objetivo de esta presentación es revisar la embriología y el aspecto más común en imágenes de algunas de estas anomalías.

Innovation and surgical techniques: endoscopic resection of cervical branchiogenic cysts.

The recent advent of endoscopic procedures has compelled both plastic and neck and head surgeons to reconsider the conventional methods by which the excision of cervical congenital cystic is classically achieved.An endoscopic approach for excision of the cervical congenital cystic is described. This procedure is anatomically safe and can be made with minimal morbidity through a small transcervical incision. Both specific instruments and solid anatomical knowledge are necessary to perform a safe and efficient cystic endoscopic excision. The essential surgical steps are as follows: 1. Minimal incision placed in natural cervical wrinkle over the dome of the cyst; 2. Intracystic or extracystic dissection; 3. Identification and protection of the sternocleidomastoid muscle, spinal nerve, hypoglossi nerve, and posterior belly of digastric muscle; 4. Careful dissection of the posterior surface of the cyst, avoiding injury on the carotid vessels and internal jugular vein. Eight patients were operated on with this technique and they were very pleased with postoperative comfort and aesthetic results. Inconspicuous scars and no complications were registered. With advanced endoscopic instruments and the development of new surgical technique and surgeon experience, the endoscopic surgery can be the method of choice in cervical excision of branchiogenic cysts.

Lesiones quísticas del cuello: correlación clínica y de imagen / Cystic lesions of the neck image and clinical correlation

Se hace una revisión retro y prospectiva en 14 pacientes (8 mujeres, 6 hombres) con lesiones quísticas congénitas a nivel del cuello. Los autores analizan las lesiones más frecuentes encontradas a nivel del cuello supra e infrahioideo y que incluyen quiste del conducto tirogloso, quiste del segundo arco branquial, linfangioma o higroma quístico, sus manifestaciones clínicas correlacionadas con las características observadas a través de los métodos de imagen

[Cervico-facial heterotopic brain tissue with a mandibular deformity]. / Tejido cerebral heterotópico cervico-facial con deformidad mandibular.

Case report of a premature baby girl who presented with a right cervico-facial mass which caused severe respiratory difficulty. The patient underwent subtotal resection of the mass whose pathologic specimen report revealed heterotopic cerebral tissue compatible with encephalocele. A head and neck CT Scan performed could not evidence a connection between the mass and the cranial cavity, reason for which the possibility of encephalocele was disregarded. After subtotal resection of the mass, the tumor began to grow from soft palate and the patient died from respiratory complications at four months of age. Autopsy reported heterotopic cerebral tissue from neck and soft palate; right lung pneumonia and infection by CMV. Although the heterotopic cerebral tissue was reported as benign, the clinical characterization of this mass is compatible with a malignant behavior due to the aggressiveness of its growth.

Tejido cerebral heterotópico cervico-facial con deformidad mandibular / Cervico-facial heterotopic brain tissue with a mandibular deformity

Este es el caso de una paciente prematura que presentó una masa faciocervical derecha que le provocó dificultad respiratoria severa. La bebé fue sometida a excisión subtotal de la masa de cuyo tejido se realizó un estudio patológico donde se informó tejido cerebral heterotópico compatible con encefalocele. Por tomografía computadorizada de cabeza no se evidenció conección de la masa con la cavidad craneal, por lo que se descarta encefalocele. Falleció a los 4 meses con dificultad respiratoria. En la autopsia realizada se informó tejido cerebral heterotópico en cuello y paladar; neumonía pulmón derecho e infección por citomegalovirus. El tejido cerebral heterotópico a pesar de que se informa como benigno, en este caso se comportó como maligno por lo agresivo de su crecimiento