Congenital malformation of lung parenchyma: 15 years experience in a thoracic surgical unit.

Objective. To review the surgical management of congenital malformations of lung parenchyma in a thoracic surgery unit over a period of 15 years. Methods. We carried out a retrospective analysis of records of all patients who had surgery for congenital malformations of lung parenchyma between 1995 and 2010. Results. Forty-five patients underwent surgery for congenital lung lesions out of 3735 thoracotomies performed during the study period. The lesions included 29 lung sequestrations, 12 bronchogenic cysts, 3 congenital lobar emphysema and one congenital cystic adenomatoid malformation. Only 26 (26%) cases were diagnosed preoperatively. Twenty-eight (62.2%) patients underwent lobectomy, 5 (11.1%) patients had pneumonectomy, and 10 (22.2%) patients had removal of cyst while 2 (0.45%) patients had lung resection with repair of the oesophageal connection. No mortality was recorded. One patient had post-operative complication of oesophageal fistula which was successfully managed conservatively. The follow-up was between 8 months to 14 years. All patients were asymptomatic and had no physical limitations during the follow-up. Conclusions. Surgery is curative and produces good long-term result in patients with congenital malformations of lung parenchyma. It should be offered to patients as a therapeutic option where indicated and feasible.

Quiste broncogénico de localización poco habitual: reporte de 2 casos y revisión de la literatura / Bronchogenic cyst of unusual location: report of two cases and review of the literature

El quiste broncogénico es un remanente del desarrollo de traquea y bronquios. Alteraciones en la migración de células durante el desarrollo, permite que estos grupos originen quistes revestidos por epitelio respiratorio. La ubicación más habitual es la intratoráxica, dentro de ella, la intrapulmonar es la más frecuente, seguida de la mediastínica. El diagnóstico definitivo se establece mediante la biopsia diferida. Es una patología poco frecuente y las presentaciones extra torácicas son muy infrecuentes, por lo que compartimos dos casos, uno de ubicación sublingual y otro de ubicación subcutánea en zona escapular.

The bronchogenic cyst is a remanent of the development of the tracheobronchial tree. Alterations in cell migration during development cause the formation of cyst lining by respiratory epithelium. They are usually located in the thorax in lung and mediastinum. The definite diagnosis is realized with the pathology report. This is an uncommon pathological condition and the extra- thoracic forms are very unusual. We present two cases, one of which is of sublingual location and the other of subcutaneous location at the scapular site.

Obesidad mórbida asociada a quiste broncogénico y leiomioma subcardial que simula GIST: caso clínico / Morbid obesity, esophageal bronchogenic cyst and subcardial leiomioma: report of one case

Gastric wall diseases are found in approximately of 1 percent of the patients submitted to bariatric surgery. Half of these are leiomiomas. Esophageal bronchogenic cysts are exceptional We report a 57 years old female with morbid obesity, a bronchogenic esophageal cyst and subcardial leiomioma. The preoperative study and intraoperative biopsy, suggested the presence of a gastrointestinal stromal tumor (GIST), but the definitive pathological study did not confirm its presence. A total gastrectomy was performed, with an uneventful postoperative course.

La patología intramural gástrica tiene una frecuencia menor al 1 por ciento, en cirugía gástrica. Alrededor del 50 por ciento corresponden a leiomiomas. Los quistes broncogénicos tienen origen embriológico y son excepcionales, y la mayor parte de los reportes corresponden a pacientes pediátricos. Se presenta un caso clínico de paciente portadora de Obesidad mórbida (IMC = 52), asociado a lesión subcardial que simula GIST, cuyo estudio histopatológico resultó ser un quiste esofágico de origen broncogénico de 40 mm, asociado a leiomioma subcardial de 10 mm. Fue sometida a gastrectomía total más anastomosis esófago-yeyunal en Y de Roux, con asa de 180 cm. No presentó morbi-mortalidad postoperatoria. Se discuten los métodos de estudio preoperatorio y las alternativas terapéuticas. No hemos encontrado en la literatura otro caso de asociación de estas raras patologías.

Sternal bronchogenic cyst: a rare condition

Bronchogenic cyst is a congenital developmental abnormality of the embryonic foregut usually formed as a result of an accessory lung bud becoming isolated from the rest of the tracheobroncheal tree. Usually a solitary extra pulmonary cyst, it's a benign condition, found most commonly in the mediastinum with rare occurrence on the skin or subcutaneous tissues.We report a case of cutaneous bronchogenic cyst that occurred in the skin over the manubrium sterni of four years old boy. The diagnosis was made by the histopathological findings which revealed ciliated and mucin-producing pseudo stratified columnar epithelium of respiratory type

Seven years experience of bronchogenic cysts

To reveal the clinical, radiological, and surgical results of bronchogenic cysts. Patients that underwent surgical procedure between January 2000 and June 2007, at Izmir Dr. Suat Seren Chest Disease and Thoracic Surgery Training Hospital, Izmir, Turkey with a radiological diagnosis of bronchogenic cyst were assessed retrospectively. Patients with confirmed histopathologically bronchogenic cyst [n=28] were evaluated for age, gender, symptoms, clinical and radiological signs, procedure of surgical treatment, and post-operative complications. There were 12 females, 16 males, and the mean age was 45.3 [25-73] years. Cysts were located at the pulmonary parenchyma in 53.5%, at the mediastinum in 43%, and at the intrathoracic extrapulmonary in 3.5%. There was no relation between localization and gender [p=0.276], and localization and the presence of symptoms [p=0.409]. Frequently seen symptoms were dyspnea and chest pain. Cysts were infected in 11%, and intact in 89%. The average diameter of the cysts was 6.18 cm [2-12]. Surgical complete resection was performed via thoracotomy in all patients. Mean follow-up time was 36 months, and there was no death. Minor postoperative complications occurred in 3 patients. Radiology alone may not be enough for diagnosis of bronchogenic cysts in all patients. Early surgical intervention is suggested for the exact diagnosis and prevention of operative difficulties and complications

Tratamento operatório de 60 pacientes com malformações pulmonares: o que aprendemos? / Surgical treatment of 60 patients with pulmonary malformations: what have we learned?

OBJETIVO: Analisar retrospectivamente os prontuários de pacientes com malformações pulmonares submetidos a tratamento operatório e verificar a evolução clínica até o diagnóstico definitivo. MÉTODOS:Analisamos os prontuários dos pacientes com malformações pulmonares operados no Hospital São Paulo-Universidade Federal de São Paulo/Escola Paulista de Medicina-de 1969 a 2004. Cada prontuário foi analisado quanto aos seguintes aspectos: quadro clínico, diagnóstico, tratamento prévio, tratamento operatório e complicações hospitalares. Os critérios de inclusão foram os seguintes: ter diagnóstico de malformação pulmonar, ter sido submetido à ressecção pulmonar e ter prontuário com dados completos. RESULTADOS: A análise dos prontuários revelou que 60 pacientes com diagnóstico de malformações pulmonares foram operados-27 casos de cisto broncogênico, 14 de ensifema lobar congênito, 10 de seqüestro pulmonar e 9 de malformação adenomatóide cística. A idade variou de 4 dias a 62 anos (média de 17,9 anos). Houve predominância do sexo masculino (55 por cento). Noventa e dois por cento dos pacientes apresentavam sintomas (média de duração, 15,37 meses). Dos 60 pacientes operados, 27 (45 por cento) receberam tratamento domiciliar ou hospitalar com antibiótico antes da operação. Quanto às complicações, observamos morbidade de 23 por cento e mortalidade de 3,3 por cento. A duração dos procedimentos operatórios realizados em nossos pacientes variou de 1 a 8 h (média, 3,2 h). CONCLUSÕES: A falha ou atraso no diagnóstico das malformações pulmonares resultou em tratamentos e hospitalizações desnecessárias e em complicações infecciosas recorrentes e freqüentes. Acreditamos que o tratamento definitivo é a operação, a qual é curativa e tem baixa morbidade e mortalidade.

OBJECTIVE: To retrospectively analyze the medical charts of patients with pulmonary malformations submitted to surgical treatment and to investigate the clinical evolution prior to the definitive diagnosis. METHODS: We analyzed the medical charts of patients with pulmonary malformations operated on at the São Paulo Hospital-Federal University of São Paulo/Paulista School of Medicine-from 1969 to 2004. Each medical chart was analyzed as to the following aspects: clinical profile; diagnosis; previous treatment; surgical treatment; and nosocomial complications. The inclusion criteria were having received a diagnosis of pulmonary malformation, having undergone pulmonary resection, and chart data being complete. RESULTS: The analysis of the medical charts revealed that 60 patients diagnosed with pulmonary malformations-27 cases of bronchogenic cyst, 14 cases of congenital lobar emphysema, 10 cases of pulmonary sequestration, and 9 cases of cystic adenomatoid malformation-underwent surgery. Ages ranged from 4 days to 62 years (mean, 17.9 years). There was a predominance of males (55 percent). Ninety-two percent of the patients presented symptoms (mean duration: 15.37 months). Of the 60 patients undergoing surgery, 27 (45 percent) received preoperative home or hospital treatment with antibiotics. Regarding complications, we observed that morbidity was 23 percent, and mortality was 3.3 percent. Surgical times ranged from 1 to 8 h (mean, 3.2 h). CONCLUSIONS: Misdiagnosis or delayed diagnosis of pulmonary malformations resulted in unnecessary treatments and hospitalizations, as well as in frequent, recurrent infectious complications. We believe that the definitive treatment is surgery, which is curative and has low morbidity and mortality rates.

Perinatal management of common neonatal thoracic lesions.

Esophageal atresia, congenital diaphragmatic hernia, bronchopulmonary malformations and cystic lung diseases are the common neonatal thoracic surgical lesions encountered in practice. The availability of antenatal ultrasonography has lead to these lesions being detected before birth. Antenatal diagnosis can be made with a fair degree of accuracy in tertiary fetal medicine centres. Antenatal intervention is limited in a very few centres in the western world and not being done in India at present. The outcome of these babies with antenatal diagnosis of thoracic lesions has changed in the last decade. Earlier intervention is now possible in cystic lung disease before infectious complication has set in. All these lesions are managed exclusively in well developed neonatal surgery units with excellent outcome in the western world. The present study reviews the antenatal detection, clinical presentation, interventional/surgical procedures [antenatally and postnatally] and outcome of these common neonatal thoracic surgical lesions.

Quiste broncogénico intratiroideo: pesentación de un caso / Intrathyroid bronchogenic chyst: presentation of a case

Paciente masculino, blanco, de 30 años de edad, que acude al médico por presentar nódulo en región lateral derecha del cuello, se indica ultrasonido y biopsia aspirativa de la lesión. El primero informa imagen eco lúcida unilocular del lóbulo derecho del tiroides y la segunda compatible con quiste branquial, posteriormente se realiza intervención quirúrgica consistente en hemitiroidectomia derecha. El resultado de la biopsia (B-07-1513) fue el de quiste broncogénico intratiroideo. Se realiza revisión bibliográfica y solamente se encuentra reporte de tres casos de quiste broncogénico intratiroideo, por lo que decidimos realizar esta presentación.

A 30-years-old, white, male patient attended the hospital because he presented a nodule on the right side of the neck. An ultrasound and an aspirative byopsy of the lession were indicated. The first one showed a unilocular echolucid image of the right lobule of the thyroid and the second one, an image compatible with a bronchial chyst. Then, we carried out a surgical intervention: a right hemithyiroidectomy. The result of the biopsy (B-07_1513) was an intrathyroid bronchogenic chyst. We made a bibliographic review and found only three reports of intrathyroid bronchogenic chyst, thus deciding to present this case.

Quiste broncogénico cervical: reporte de un caso / Cervical bronchogenic cyst: report of one case

Los quistes broncogénicos son malformaciones congénitas del árbol traqueobronquial que se ubican preferentemente en pulmones y mediastino, siendo excepcional su aparición en el cuello. Esta situación conlleva que ante una masa cervical de esta naturaleza se postulen diferentes diagnósticos clínicos de lesiones cervicales que son más corrientes, tales como quiste tiroideo, tirogloso, branquial y tímico, entre otros. La imagenología informa sobre el carácter quístico de la lesión pero no proporciona hallazgos específicos. Durante la intervención quirúrgica tampoco es posible establecer el diagnóstico, puesto que la masa simula los quistes cervicales congénitos más frecuentes. El diagnóstico definitivo se establece mediante la biopsia al constatar en la pared del quiste epitelio respiratorio. Comunicamos el caso de una mujer que presentó un quiste broncogénico de ubicación cervical y discutimos algunos aspectos de esta interesante condición.

Bronchogenic cysts are congenital malformations of the tracheobronchial tree that are mainly located in the lungs and mediastinum. Occasionally, they can appear in the neck, generating a cervical mass. Pathology gives the definitive diagnosis. We report a 19 years old female that consulted for an asymptomatic central cervical mass. On examination, a 4 cm diameter painless suprasternal nodule was palpated. Cervical ultrasound and CAT scan showed a cystic lesion. The cyst was completely excised surgically and the pathological report disclosed a bronchogenic cyst.

Isolated oesophageal cyst connected with the bronchus

We report an unusual case of a patient with an oesophageal cyst connected to the bronchus. A 24-year old male with a two-year history of repeated attacks of chest infection and haemoptysis was found to have a cyst of 4 x 4 cm affecting the anterior and apical segments of the right upper lobe. The cyst was excised in its entirety and the histopathological study of the cyst showed stratified squamous epithelium with submucosal and muscular layer but no cartilage. The pathological diagnosis was an oesophageal cyst. No previous case of isolated oesophageal cyst connected to the bronchus has been reported according to the available literature.

Quiste broncogénico retroperitoneal: Caso clínico / Retroperitoneal bronchogenic cyst: Report of one case

Retroperitoneal cystic tumors are uncommon. More than two thirds are malignant. Benign lesions include lipomas and íeiomyomas, among others. Bronchogenic cysts are congenital anomalies that result from an abnormal budding of the primitive foregut, and are most commonly found in the mediastinum. Occasionally they can be seen in the skin, subcutaneous tissue, in the pre-sternal andpericardial areas, but those located under the diaphragm are extremely rare. We report a 24 year-old female who consulted for abdominal pain. An abdominal ultrasound disclosed a retroperitoneal cystic lesion, that was confirmed with a CAT scan. The patient was operated, finding a cyst that was adhered to the pancreas. A distal pancreatectomy and cyst excision were done. The pathological examination of the lesion showed a bronchogenic cyst. The patient was discharged ten days after surgery.

Dois casos de cisto broncogênico do mediastino em lactentes sintomáticos / Two cases of mediastinal bronchogenic cyst in symptomatic infants

Os cistos broncogênicos são malformações relativamente infreqüentes, que causam 10% das massas e 60% dos cistos mediastinais. No entanto, uma intervenção cirúrgica, geralmente, é necessária para a confirmação do diagnóstico, para a exclusão de malignidade e prevenção de complicações, tais como hemorragia e infecção. Descrevemos dois casos de cisto broncogênico do mediastino, em lactentes com menos de 1 ano de vida. Os diagnósticos foram suspeitados devido à evolução com sintomas digestivos, associados à compressão esofagiana, demonstrada na radiografia de tórax e na seriografia esôfago-gástrica com bário. A tomografia computadorizada de tórax reforçou a hipótese diagnóstica e ambos os pacientes foram submetidos à cirurgia, sendo o diagnóstico confirmado com a histopatologia das lesões. Os dois casos obtiveram total sucesso terapêutico, tornando-se assintomáticos após a cirurgia. Este trabalho reforça a importância de se incluir a possibilidade do diagnóstico de cisto broncogênico mediastinal, em lactentes com sintomas digestivos de refluxo gastro-esofagiano que não melhoram com o tratamento clínico adequado.

Resultados del tratamiento quirurgico del quiste broncogeno / Results of the surgical treatment of the bronchogenic cyst

El quiste broncogeno es una malformacion congenita poco frecuente, que generalmente requiere tratamiento quirurgico. Con el objetivo de evaluar los resultados obtenidos en pacientes intervenidos quirurgicamente por quiste broncogeno, se realizo un estudio descriptivo de 9 pacientes consecutivos operados entre julio de 1995 y julio de 2004. Se evaluo edad, sexo, manifestaciones clinicas y tecnicas quirurgicas empleadas. Los resultados se determinaron en funcion de las complicaciones y la mortalidad. En 5 casos la lesion era de localizacion intraparenquimatosa ¨Den uno de ellos se ubicaba en la cisura interlobar izquierda¨D y en 4 se localizaba en el mediastino medio. Se practicaron 9 operaciones primarias y 3 reoperaciones. En un caso se dejo un fragmento de la pared del quiste que no fue posible resecar. Se realizo lobectomia inferior a 3 de los pacientes con quistes intraparenquimatosos (dos derechas y una izquierda). A los otros dos se extirpo el quiste, que en uno habia sustituido practicamente al lobulo superior izquierdo hipoplasico. En 5 pacientes el acceso se realizo mediante videotoracoscopia (una conversion) y en 4 mediante toracotomia convencional. Se produjo apertura bronquial durante la extirpacion del quiste mediante videotoracoscopia en un paciente. Hubo 3 reoperaciones (en 2 pacientes): por sangrado (1) y por atelectasia con infeccion pulmonar intratable y hematoma abscedado (1). Otras complicaciones fueron la sepsis de la herida (2) y el derrame pleural (1). No se ha documentado ninguna recidiva del quiste ni fallecidos. El tratamiento quirurgico de los quistes broncogenos no est¨¢ exento de complicaciones, pero produce buenos resultados a largo plazo(AU)

The cyst broncogeno is a not very frequent congenital malformacion that generally requires surgical treatment. With the objective of evaluating the results obtained in patients intervened surgically by cyst broncogeno, one carries out a descriptive study of 9 serial patients operated between July of 1995 and July of 2004. It is evaluated age, sex, manifestations clinical and technical surgical employees. The results were determined in function of the complications and the mortality. In 5 cases the lesion era of localization intraparenquimatosa ¨Den one of them was located in the incision interlobar izquierda¨D and in 4 it was located in the half mediastino. They were practiced 9 primary operations and 3 reoperaciones. In a case you accent a fragment of the wall of the cyst that was not possible to dry up. One carries out inferior lobectomia at 3 of the patients with cysts intraparenquimatosos (two rights and a left). TO the other ones two you extirpates the cyst that had substituted practically to the lobe superior left hipoplasico in one. In 5 patients the access one carries out by means of videotoracoscopia (a conversion) and in 4 by means of conventional toracotomia. Bronchial opening took place during the extirpation of the cyst by means of videotoracoscopia in a patient. There was 3 reoperaciones (in 2 patients): had bled (1) and for atelectasia with unsociable lung infection and hematoma abscedado (1). Other complications were the sepsis of the wound (2) and the spill pleural (1). it has not been documented any relapse of the cyst neither deceaseds. The surgical treatment of the cysts broncogenos non est¨ exempt¢of complications, but it produces long term good results(AU)

Cisto broncogênico simulando asma brônquica em criança / Brochongenic cyst mimicking asthma in an infant

O cisto broncogênico é a lesão cística mais comum do mediastino. Algumas de suas manifestações clínicas ocorrem por compressão das vias aéreas. Uma forma incomum de apresentação é o caráter recorrente dos sintomas obstrutivos, assemelhando-se a asma brônquica. Os autores relatam e discutem caso de cisto broncogênico simulando asma em uma criança, onde a retirada da lesão possibilitou a remissão completa dos sintomas

Cisto broncogênico complicado por mediastinite e empiema contra-lateral / Bronchogenic cyst complicated by mediastinitis and contralateral empyema

O cisto broncogênico, apesar da aparência pouco ominosa como opacidade de contornos precisos no mediastino, tem potencial não desprezível para complicar. Relatamos um caso de complicação grave em um paciente de 28 anos com queixa de dor epigástrica irradiada para o dorso, e radiografia de tórax demonstrando massa bem delimitada no mediastino posterior e inferior à direita. Em cinco dias evoluiu para sepse decorrente de mediastinite e empiema pleural à esquerda. O paciente necessitou ser submetido a toracotomia esquerda para descorticação pulmonar precoce e desbridamento do mediastino e, num segundo tempo com intervalo de uma semana, a toracotomia direita para ressecção do cisto mediastinal infectado. Este caso enfatiza a indicação sensata de ressecção dos cistos mediastinais mesmo na apresentação assintomática, face ao risco inerente de complicações.

Cisto broncogêncio simulando asma bronquica em criança / Brinchogenic cyst resembling asthma in children

O cisto broncogênico é a lesão cística mais comum do mediastino, Algumas de suas manifestações clínicas ocorrem por compressão das vias aéreas, Uma forma incomum de apresentação é o caráter recorrente dos sintomas obstrutivos, assemelhando-se a asma brônquica, Os autores relatam e discutem caso de cisto broncogênico simulando asma em uma criança, onde a retirada da lesão possibilitou a remissão completa dos sintomas

Intradiaphragmatic bronchogenic cyst

The bronchogenic cyst is a common congenital malformation, generally located in the mediastinum; however, it can develop in other areas, such as the diaphragm. A rare case of intradiaphragmatic bronchogenic cyst is described here, discovered in a 32 year-old patient, who experienced thoracic pain and dyspnea following thoracic trauma. The pre-operative exams were compatible with left diaphragmatic hernia. The patient was submitted to a left post-lateral thoracotomy with the operative discovery of a cystic lesion enveloped by the diaphragm with mucinous content, and a partial resection of the diaphragm was performed. The histological findings of the operated portion revealed ciliated cylindrical epithelium, compatible with bronchogenic cysts. The post-operative outcome was excellent.

Cisto broncogênico com abordagem videotoracoscópica / Bronchogenic cyst with videothoracoscopic approach

Cistos broncogênicos são a causa mais comum de massa mediastinal cística e o tratamento preconizado compreende a ressecção completa da lesão. Os autores relatam um caso de cisto broncogênico com tratamento efetivo por cirurgia torácica videoassistida (CTVA). Enfatizam os benefícios da CTVA no manejo dessa patologia em relação à toracotomia convencional