Fístula retroauricular complicada asociada a disgenesia mayor del oído externo. Reporte de un caso / Complicated retroauricular fistula with external ear major dysgenesis. A case report

Las anomalías de las hendiduras y arcos branquiales son la segunda causa más común de lesiones congénitas de cabeza y cuello en niños. Representan el 8% de todas las malformaciones congénitas y el 30% de las de cabeza y cuello. Pueden permanecer asintomáticas o manifestarse como una tumefacción y asociarse a infecciones recurrentes supuradas en la región preauricular, subauricular o retroauricular, en las regiones de la parótida y/o del cuello. Fueron reportados casos asociados a malformaciones óticas.La tomografía computada de alta resolución y la resonancia magnética con gadolinio pueden ayudar al diagnóstico. El tratamiento médico antibiótico está indicado ante una infección aguda, además de incisión y drenaje en los procesos abscedados. El tratamiento definitivo es quirúrgico con la extirpación completa de la lesión. Se presenta a un niño de 6 años de edad con infección aguda en la región retroauricular derecha en el oído disgenésico

Branchial cleft anomalies are the second most common congenital head and neck lesions in children. The first congenital head and neck lesion is thyroglossal duct cysts. First branchial cleft anomalies are rare congenital head and neck malformations (8% of branchial cleft anomalies).The initial clinical manifestation was recurrent infections and/or discharge in auricular, periauricular, parotid or upper neck regions. These anomalies are extremely rare and other associated facial malformations were described.The computed tomography and magnetic resonance can help the diagnosis. The antibiotic treatment is indicated in acute infection. Also, incision and drainage are recommended in abscessed processes.In this report, we present a case of retroauricular sinus infection in a 6-year-old child with congenital ear anomalies

Manejo endoscópico de anomalías del cuarto arco branquial: reporte de tres casos / Endoscopic management of anomalies of the fourth branchial arch: report of three cases

Las anomalías de cuarto arco branquial corresponden a una entidad patológica infrecuente. Para su manejo existen distintas alternativas terapéuticas siendo una de ellas la cauterización endoscópica. Reportamos 3 casos de senos de cuarto arco branquial tratados mediante cauterización endoscópica en el Hospital Regional de Concepción luego de una revisión de fichas clínicas de todos los pacientes con diagnóstico de anomalías de cuarto arco branquial. Se identificaron tres casos de senos de cuarto arco branquial. Todos corresponden a pacientes de sexo masculino que presentaron cuadro de absceso cervical, diagnosticándose 2 de ellos al presentar recurrencia. Todos fueron tratados mediante cauterización endoscópica de la apertura fistulosa en seno piriforme. Estas anomalías representan vestigios de un trayecto que se origina desde el vértice del seno piriforme. La cauterización endoscópica presenta una serie de ventajas con tasas de recurrencia similares a la cirugía abierta de cuello, menores tasas de complicaciones y costo económico. Las anomalias de cuarto arco branquial constituyen una patología infrecuente y el diagnóstico requiere alta sospecha clínica. El manejo endoscópico ha demostrado ser una alternativa segura y efectiva con menor tasa de complicaciones.

Anomalies of the fourth branchial arch correspond to an uncommon pathological entity. There are different therapeutic alternatives being one of them the endoscopic cauterization. We report 3 cases of fourth branchial arch anomalies treated by endoscopic cauterization in the Regional Hospital of Concepción. Review of clinical records of all patients with diagnosis of fourth branchial anomalies operated by endoscopic cauterization at the Regional Hospital of Concepción. Cases: Three cases of fourth branchial arch sinus were identified. All of them were male patients who presented with a cervical abscess, diagnosing 2 of them when they recurred. All 3 cases were treated by endoscopic cauterization of the fistulous opening in the piriform sinus. These anomalies represent vestiges of a path that originates from the apex of the piriform sinus. Endoscopic cauterization presents a number of advantages with recurrence rates similar to open neck surgery, with lower complication rates and economic cost. Fourth branchial anomalies constitute an uncommon pathology and the diagnosis requires high clinical suspicion. Endoscopic management has proven to be a safe and effective alternative with a lower rate of complications.

Braquio-dorso-mamoplastia: cirurgia do contorno corporal da região superior do tronco após grandes perdas ponderais / Brachial-dorsal mammoplasty: body contour surgery of the upper region of the trunk after major weight loss

INTRODUÇÃO: No Brasil foram realizadas 88 mil cirurgias bariátricas em 2014. Após o emagrecimento, ocorre desinsuflação de todo o tronco, com flacidez e dobra de excesso cutâneo das mamas à região dorsal posterior. Por isso, propomos procedimento cirúrgico que trate a região superior toda do tronco, incluindo mamas, dorso lateral e posterior e braços. OBJETIVO: Mostrar a experiência pessoal em cirurgias de braquio-dorso-mamoplastia. MÉTODO: Foram operados 13 pacientes no período de 2007 a 2014, sendo 11 mulheres e dois homens. RESULTADOS: A média de idade foi de 41,9 anos. A braquio-dorso-mamoplastia foi realizada em todos os pacientes. Em três casos, foi realizada braquio-dorso-mamoplastia em L, devido à menor deformidade latero-posterior. A duração média das cirurgias foi de 4 horas e 30 minutos. O Índice de Massa Corporal (IMC) médio, antes da cirurgia bariátrica, foi de 53,5 kg/m2. As perdas ponderais variaram de 47 a 114 kg. O IMC pré-operatório variou de 23 a 39 kg/m2. Não houve casos de infecção, grandes deiscências ou seroma. Duas pacientes evoluíram com hematoma, necessitando de nova intervenção para drenagem. CONCLUSÃO: As deformidades da região torácica no paciente com perda ponderal maciça são variáveis, sendo necessários diversos tratamentos cirúrgicos que deverão abordar o tórax inteiro como área anatômica única. É importante compreender a deformidade que cada paciente apresenta e adequar a abordagem cirúrgica em conformidade. A indicação principal para uma braquio-dorso-mamoplastia é a queda lateral do sulco inframamário.

INTRODUCTION: In Brazil, 88000 bariatric surgeries were performed in 2014. After weight loss, deflation occurs around the trunk, with sagging and folding of excess skin of the breasts in the posterior dorsal region . We, therefore, propose a surgical procedure to treat upper region of the entire trunk, including breasts, side and rear back, and arms. OBJECTIVE: To present a personal experience in brachial-dorsal mammoplasty surgeries. METHOD: We operated 13 patients from 2007 to 2014, 11 women and two men. RESULTS: The average age was 41.9 years. The brachial-dorsal mammoplasty was performed in all patients. In three cases, L-brachial-dorsal mammoplasty was performed, due to lower latero-posterior deformity. The average surgery duration was 4 hours and 30 minutes. The average Body Mass Index (BMI), before bariatric surgery, was 53.5 kg/m2. Weight loss ranged from 47 to 114 kg. The pre-surgery BMI ranged from 23 to 39 kg/m2. There were no cases of infection, large dehiscence or seroma. Two patients developed hematoma, requiring a new intervention for drainage. CONCLUSION: Deformities of the thoracic region in patients with large weight loss are variable, requiring several surgical treatments that should address the entire chest as a single anatomical area. It is important to understand the deformity that each patient presents and adapt the surgical approach accordingly. The main indication for a brachial-dorsal mammoplasty is lateral drop of the inframammary fold.

Fístula laterocervical izquierda de tercer arco branquial: caso clínico / Third left laterocervical fistula gill arch: case report

En el presente trabajo queremos describir un caso poco común de trayecto fistuloso cervical. Se presenta en un nino y nos parece importante a tener en cuenta como diagnóstico diferencial, dentro de la otorrinolaringología infantil. Las anomalías branquiales pueden presentarse como quistes, trayectos sinusoidales o fístulas. Se explican por un mal desarrollo del arco branquial durante el periodo embriológico. Se presentan principalmente en la infancia entre los 2 a 3 años de edad y a veces también en la juventud. Aparecen igualmente en ambos sexos. Las fistulas del primer arco branquial son raras (1%-8%), las más comunes son las del segundo arco (90%). En nuestra experiencia el método de imagen de elección para evaluar las anomalías de los arcos branquiales es la fistulografia. El tratamiento de todas ellas es la exéresis quirúrgica.

In this paper we describe a rare case of cervical fistula tract. It comes in a child and it seems important to consider in the differential diagnosis, in ORL child. Branchial anomalies can present as cysts or fistulas sinusoidal paths. Explained by poor development of gill arch during embryologic. They occur mainly in children between 2-3 years of age and sometimes in youth. Appear equally in both sexes. The first brachial arch fistulas are rare (1%-8%>), the most common are the second arch (90%%). In our experience the imaging method of choice to evaluate abnormalities of the brachial arches is the sinogram. Treatment is surgical removal.

Fissura congênita cervical da linha média / Congenital midline cervical left", "_e": "n

Existe uma deformidade cervical da linha média que tem sido descrita com vários nomes. Embora sua causa embriológica continue sendo controversa, a etiologia mais aceita até hoje é a falha da fusão na linha média dos arcos branquiais distais. A característica principal é uma lesão protuberante na linha média da região anterior do pescoço, entre o mento e a fúrcula esternal. Recomenda-se a ressecção cirúrgica completa da lesão, e a reconstrução do defeito com w-plastia ou zetaplastia. Neste artigo, é relatado o caso de uma paciente de 38 anos, com fissura congênita cervical da linha média. Após exérese da lesão, foi realizada zetaplastia simples, obtendo-se bom resultado tanto estético como funcional. As fissuras congênitas cervicais da linha média são alterações raras na população mundial, porém seu diagnóstico precoce e tratamento cirúrgico adequado são necessários para prevenir alterações no crescimento facial e restrição na extensão do pescoço.

Congenital midline cervical cleft is a deformity known by several names. Although its embryologic origin has not been clearly established, the most widely accepted etiology is impaired midline fusion of the distal branchial arches. The main characteristic of this malformation is a protruding lesion at the midline of the anterior neck, between the chin and the suprasternal notch. Complete surgical resection of the lesion is recommended, with repair of the defect using W-plasty or Z-plasty. This article reports the case of a 38-year-old patient with congenital midline cervical cleft. A simple Z-plasty was carried out after excision of the lesion, and a satisfactory aesthetic and functional result was achieved. Congenital midline cervical cleft is a rare abnormality; however, early diagnosis and appropriate surgical treatment are necessary to avoid changes in the development of the face and limitation of neck extension.

Síndrome brânquio-otorrenal: um importante alerta / Branchio-oto-renal syndrome: an important warning

A síndrome brânquio-otorrenal (SBOR) é uma doença autossômica dominante, provocada por mutações em genes da organogênese humana, principalmente o gene EYA 1. Sua incidência é de 1 em 40.000 recém-nascidos vivos. As manifestações clínicas da SBOR decorrem de transtorno genético e envolvem perda auditiva de condução, neurossensorial ou mista, malformações da orelha externa, média e interna, cistos ou fístulas branquiais e anomalias renais que podem variar de hipoplasia a agenesia renal. O diagnóstico clínico da SBOR se baseia na presença de critérios clínicos. A genética molecular é de grande valor para a confirmação do diagnóstico e para estabelecer o diagnóstico pré-natal. Testes audiométricos completos e exames renais apurados são indicados a fim de mensurar com precisão a extensão dos danos causados pela doença. Sua ampla variabilidade clínica sugere, na literatura, que os diferentes fenótipos dessa síndrome representam, na realidade, grupos heterogêneos de doenças, que fazem diagnóstico diferencial, principalmente, com a síndrome brânquio-otorrenal. O tratamento se limita a reparar os danos causados pela doença, podendo estar indicado o uso de próteses auriculares ou intervenções cirúrgicas otológicas e renais, a fim de melhorar a qualidade de vida dos pacientes. O aconselhamento genético é a melhor forma de prevenção, sendo fundamental no tratamento.

Branchial fistula: a rare presentation

Remains of cervical sinus of His may persist as a branchial cyst. A branchial sinus is formed when 2nd branchial arch fails to meet the 5th pharyngeal arch. Peak age for presentation of cysts is in the third decade and that of the congenital sinuses and fistulae is at birth. Rarely a branchial sinus is found to have an internal opening as well, thus forming a true branchial fistula. We present a case of true branchial fistula with external opening on the lateral side of neck and internal opening near the lower pole of tonsil

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.

Preservation of facial nerve in surgery of first branchial cleft anomaly: five cases of duplicated external ear canal

We report five cases of duplicated external ear canal with especial attention to its histology and surgical care of facial nerve for their complete resection. These five patients were selected from 50 cases of first bronchial cleft anomalies including atretic external ear canals, preauricular fistula, cyst and sinuses. The characteristic histopathology and clinical features such as external opening around the auricle and upper neck above the level of the hyoid bone were not observed in other patients. There were no other abnormalities in the real external ear canal [EEC] and auricle. Three of them were connected to the natural EEC with an opening, hence producing ear discharges. Histologic appearances of well formed canal included a circumferential epithelial lined up and canal consisting of stratified squamous epithelium with skin adnexes. A remarkable cartilaginous skeleton [type 2 of first branchial cleft anomaly according to Work's classifications] was also observed. All of them were treated surgically, with preservation of the facial nerve and excising the superficial parotid gland along with the ductal opening and the tract

Imaging of congenital anomalies of the branchial apparatus with surgical and pathological correlation

One of the most challenging topics for surgeons, pathologists and radiologists is congenital anomalies of the branchial apparatus that include fistulas, sinuses, cysts, and temporal bone anomalies. The aim of the study was to describe primarily the CT features of different abnormalities that are secondary to anomalous embryogenesis of the branchial apparatus with surgical and pathological correlation. This study included 30 patients with branchial apparatus anomalies, all evaluated clinically and radiologically with correlation of the imaging findings with the surgical and pathological results. Thirteen patients had first branchial apparatus anomalies; namely five classic first branchial apparatus anomalies, and eight temporal bone anomalies. Classic first branchial apparatus anomalies included two intra-parotid cysts, one intra-parotid sinus and two anterior triangle cysts. Temporal bone anomalies included three isolated external aural atresias, one isolated middle ear cleft anomaly, and four combined external auditory canal and middle ear cleft anomalies. Fifteen patients had second branchial apparatus anomalies; namely eight cysts, four sinuses and three fistulas. Only two third branchial apparatus anomalies were encountered including one cyst and one sinus. The anatomic location of different branchial anomalies was described in reference to their embryonic origin. Of the 13 branchial cysts included in this study, nine displayed well defined borders with fluid attenuation, and four displayed ill defined borders with heterogeneous mixed attenuation; correlated with infective and haemorrhagic changes. Three infected cysts displayed marginal enhancement. Magnetic resonance imaging was performed for eight branchial cysts with post-Gd acquisitions obtained for five of the cysts; MR imaging features matched the nature of the cysts. CT and MR imaging are crucial in the assessment of branchial apparatus anomalies to provide the surgeon with a detailed "road map" stressing the relationship of the lesion to critical neck structures

Pyriform sinus fistula.

Pyriform sinus fistulae/sinuses are rare causes of recurrent cervical abscess, especially on the left side. They can also present as acute thyroiditis. Treatment in the form of simple incision and drainage is invariably unsuccessful, and the entity may be confused with the residual tract of a second branchial arch anomaly. We report a case of pyriform sinus fistula, and believe that this is only the second case report in India. We feel that greater awareness can lead to proper and appropriate diagnosis of this anomaly.

Anomalias branquiais: revisão de 23 casos / Branchial anomalies: a review of 23 cases

A anomalia branquial é a doença congênita mais comum vista pelo cirurgião de cabeça e pescoço. Vinte e três pacientes portadores de anomalias branquiais tratados no período de 1994 a 1997 foram revisados. O diagnóstico clínico foi documentado por ultra-sonografia e comprovado pelo exame histopatológico. Encontramos seis anomalias de primeiro arco (26 por cento); 16 do segundo arco (69,5 por cento); e uma do terceiro (4,5 por cento). Não houve anomalia do quarto arco. Após um seguimento que variou de três a 43 meses, não houve caso de recidiva. A prevalência de anomalias do segundo arco é compatível com a literatura, porém, foi alta a incidência do primeiro arco. O emprego da técnica cirúrgica adequada, com a ressecção de todo o trajeto fistuloso, mostrou-se eficaz

Afeccoes congenitas cervicais / Congenital neck affections

Os aspectos diagnosticos e terapeuticos das malformacoes congenitas cervicais sao discutidos com base na experiencia recente da Disciplina de Cirurgia de Cabeca e Pescoco da Faculdade de Medicina da Universidade de Sao Paulo. De 1993 a 1997, 100 pacientes foram submetidos a tratamento por afeccoes congenitas. A maioria foi composta de anomalias relacionadas ao ducto tireoglosso (54 por cento). Carcinoma papilifero em ducto tireoglosso, embora raro, foi encontrado em 2 casos. Os cistos e fistulas branquiais constituiram 19 por cento dos casos. O conhecimento dos fundamentos embriologicos determina a realizacao de tratamento adequado para essas afeccoes, com menor risco de recidiva

Aplicaciones de la tomografía helicoidal en el estudio de un paciente con síndrome de Goldenhar / Use of the helicoidal CT for the study of a patient with Goldenhar syndrome

Estudiamos a una niña de cinco años de edad con síndrome de Goldenhar, una rara alteración congénita resultante de un trastorno en el desarrollo embriológico del primer y segundo arco branquial. Sus manifestaciones craneales y extracraneales, son muy diferentes entre un paciente y otro. Para valorar este amplio espectro de alteraciones la TC helicoidal constituye un método de evaluación complementario de gran utilidad. Las principales manifestaciones del síndrome de Goldenhar asientan a nivel cráneofacial y en algunos casos también pueden existir anomalías extracraneales. Los hallazgos se compararon con los de la literatura internacional actualizada. El pleomorfismo y el amplio espectro de manifestaciones que provoca este trastorno del desarrollo hacen necesaria una detallada valoración en cada paciente en particular. Para cumplir con dicho objetivo la TC helicoidal es el método de elección por su capacidad de obtener imágenes multiplanares que puede evitar los riesgos de una anestesia

Branchial cleft anomalies--a study of 20 cases.

Preauricular sinus, branchial cyst, branchial sinus and branchial fistula are the result of incomplete obliteration of branchial clefts. Classically these lesions have particular sites and nature. The aim of this three years retrospective study was to see the prevalence of these conditions as well as the outcome of the conventional surgery. Twenty cases of branchial cleft anomalies were reported, of which 60% had with preauricular sinus. One case with preauricular sinus had recurrence during postoperative follow-up.

Trago accesorio: estudio clínico-patológico de 16 casos / Accesory tragus: clinical-pathology study of 16 cases

Se presentan 16 casos de trago accesorio unilateral y solitario, con predominio en el sexo femenino y edad variable de aparición. Esta entidad poco sospechosa puede con su localización preauricular plantear numerosos diagnmósticos diferenciales. Se describen sus características histopatológicas y tratamiento

Anomalia da primeira fenda branquial: fístula cervical associada a supuraçäo do ouvido: apresentaçäo de um caso / Abnormality of the first branquial cleft: cervical fistula associated to ear supuration: report of a case

Os autores relatam um raro caso de criança portadora de anomalia originada da primeira fenda cervial cervical. Ela apresentava otorréia no ouvido esquerdo, em concomitância com a presença de um sínus servical. Após drenagem cirúrgica para tratamento do abscesso no triángulo anterior do pescoço, surgiu fístula que se manteve e colocou em comunicaçäo a pele com o assoalho do conduto auditovo externo. Comentam ainda a etiologia, incidência e tratamento cirúrgico