Malformación congénita de la vía aérea pulmonar. Reporte de un caso / Congenital malformation of the pulmonary airway. Report of a case

RESUMEN El objetivo de este estudio es realizar una revisión de la Malformación Congénita Pulmonar a propósito del caso de un feto de una paciente de 34 años que cursaba su segunda gesta, cuyo diagnóstico se realizó a la semana 30 de gestación. Además se realizó una revisión bibliográfica de la patología y de casos similares.

ABSTRACT The objective of this study is to perform a review of Congenital Pulmonary Malformation regarding a case of a fetus in a 34-year-old woman who was in her second pregnancy, whose diagnosis was made at week 30 of gestation. In addition, a bibliographic review of the pathology and similar cases was carried out

Quiste congénito del conducto nasolacrimal/dacrio-cistocele: causa de dacriocistitis no diagnosticada / Congenital cyst of nasolacrimal duct/dacriocistocele: cause of non-diagnosed dacriocistitis

RESUMEN Se presenta dos casos de pacientes menores de 5 meses los cuales durante las primeras semanas de vida presentan descarga ocular muco-purulenta de manera recurrente. Posteriormente en la endoscopia se encuentra en los dos casos masas quísticas intranasales dando un diagnóstico de dacriocistocele más quiste congénito del conducto nasolagrimal. El dacriocistocele es una causa muy rara de obstrucción del conducto nasal y más raro cuando viene acompañado de un quiste congénito del conducto nasolagrimal. Debido a su rareza y a su sintomatología muchas veces atípica resulta en un diagnóstico complicado para el otorrinolaringólogo y para el oftalmólogo.

ABSTRACT Two cases of patients younger than 5 months are presented, who during the first weeks of life have recurrent muco-purulent ocular dis-charge. Subsequently, in endoscopy, in both cases, intranasal cystic masses were found, resulting in a diagnosis of dacryocystocele plus congenital cyst of the lacrimal duct. Dacryocystocele is an uncommon cause of nasal duct obstruction, but it becomes less common when accompanied by a congenital tear duct cyst. Due to its uncommonness and its often-atypical symptoms, it results in a complicated diagnosis for the ENT and the ophthalmologist.

Type I pleuropulmonary blastoma presenting as congenital pulmonary airway malformation: A report of two cases

Pleuropulmonary blastoma (PPB) is a rare aggressive intrathoracic tumor which is believed to originate from embryonic uncommitted lung mesenchymal cells, which are important for developing the lung. Type I PPB is cystic, type II is cystic and solid, while type III is predominantly solid. Diagnosing type 1 PPB is a challenge for both radiologists as well as pathologists. Owing to its purely cystic nature, type I PPB it is often mistaken for unrelated entities such as congenital pulmonary airway malformation and congenital lobar emphysema which delays surgical intervention. Here, we report two such cases presenting clinically and radiologically as congenital pulmonary airway malformation. On histology, a final diagnosis of type I pleuropulmonay blastoma was made. Thereafter, chemotherapy was administered following complete surgical excision.

Quiste de conducto cístico / Cystic duct cyst

Los quistes congénitos de la vía biliar son infrecuentes y se definen por la dilatación quística del árbol biliar en cualquiera de sus porciones. Los quistes del conducto cístico son aún menos frecuentes. Su etiología permanece incierta y el tratamiento consiste en la resección debido a su potencial desarrollo de malignidad. Presentamos el caso de una paciente en la que se diagnosticó dilatación del conducto cístico y fue tratada por vía laparoscópica.

Congenital biliary duct cysts are rare and are defined as cystic dilatations of the biliary tree in any of its portions. Cystic duct cysts are more uncommon. Their etiology remains uncertain and they should be resected due to the possible development of malignancy. We report the case of a female patient with a diagnosis of dilation of the cystic duct that was treated with laparoscopic surgery.

Diagnóstico ecográfico prenatal y resultados neonatales de la malformación adenomatoidea quística pulmonar / Antenatal ultrasound diagnosis and neonatal results of the congenital cystic adenomatoid malformation of the lung

INTRODUCCIÓN: La malformación adenomatoidea quística (MAQ) es una patología congénita del pulmón poco frecuente y su diagnóstico prenatal es factible en la mayoría de los casos. Existen discrepancias en cuanto al manejo prenatal y tratamiento postparto. OBJETIVO: Analizar una serie de casos de MAQ diagnosticados prenatalmente, evaluando los hallazgos ecográficos y la evolución fetal y post natal. PACIENTES Y MÉTODO: Estudio retrospectivo de todos los casos diagnosticados prenatalmente por ecografìa entre 2005 y 2016 en dos hospitales de referencia. Los exámenes de ultrasonido fueron realizados utilizando ecógrafos de alta resolución, Toshiba Xario y Voluson 730 Expert Pro, con seguimiento desde el diagnóstico hasta el parto. Las variables analizadas incluyeron la edad gestacional en el momento del diagnóstico, las características de la lesión pulmonar, las malformaciones asocia das, el estudio citogenético, la evolución del embarazo, el tipo de parto, presencia de distrés respira torio, necesidad de pruebas de imagen complementarias, evolución clínica pediátrica y tratamientos postnatales necesarios. Se consideró resolución la desaparición total de la lesión ecográfica prenatal o que la radiografía torácica postnatal no mostrara lesión alguna. RESULTADOS: Se identificaron pre natalmente 17 casos. La evolución varía desde la resolución prenatal de la lesión hasta la persistencia de la misma tras el nacimiento. Tres pacientes decidieron abortar voluntariamente por hallazgos ecográficos de mal pronóstico. De los catorce casos restantes no hubo ningún caso de muerte fetal ni neonatal, un caso requirió cirugía tras el nacimiento y cuatro pacientes presentaron sintomatología leve durante el primer año de vida. Se ha reportado un caso de falso negativo con muerte neonatal, que la necropsia informó como MAQ tipo 0. CONCLUSIONES: Esta malformación pulmonar presenta buen pronóstico, excluyendo los casos con hidrops fetal. La ecografía bidimensional suele ser suficiente para el diagnóstico y el seguimiento. La tomografía computarizada es la técnica de elección para confirmar la resolución de las lesiones tras el nacimiento. El tratamiento quirúrgico es preferible sobre el manejo conservador, aunque se desconoce si las complicaciones potenciales de esta patología, aun siendo asintomática, justifican la morbilidad quirúrgica.

INTRODUCTION: Congenital cystic adenomatoid malformation (CCAM) is a rare congenital lung di sease, and in the most of cases, prenatal diagnosis is feasible. There are discrepancies regarding pre natal management and postpartum treatment. OBJECTIVE: To analyze prenatally diagnosed CCAM in our hospitals, in order to evaluate ultrasound findings with fetal and postnatal evolution. PATIENTS AND METHOD: Retrospective study of all cases diagnosed prenatally by ultrasound between 2005 and 2016 in two reference hospitals. The ultrasounds were performed using high-resolution ultrasound scanners, Toshiba Xario and Voluson 730 Expert Pro, with follow-up from diagnosis to delivery. The variables analyzed included gestational age at diagnosis, the characteristics of the lung lesion, associated malformations, cytogenetic study, the evolution of pregnancy, type of delivery, presence of respiratory distress, need for complementary imaging tests, pediatric clinical course, and necessary postnatal treatments. It was considered a resolution the total disappearance of the lesion in the pre natal ultrasound or that the postnatal chest X-ray showed no lesion. RESULTS: 17 cases were prenatally diagnosed. The evolution ranges from the prenatal resolution of the lesion to the persistence after bir th. Three patients voluntarily decided to have an abortion due to ultrasound findings of poor progno sis. Of the fourteen remaining cases there were no cases of fetal or neonatal deaths, one case required surgery after birth and four patients had mild symptoms during the first year of life. One case of false negative with neonatal death has been reported which necropsy reported as CCAM type 0. CONCLUSIONS: This pulmonary malformation presents good prognosis, excluding cases with fetal hydrops. Two-dimensional ultrasound is usually enough for diagnosis and follow-up. Computed tomography is the technique of choice to confirm the resolution of lesions after birth. Surgical treatment is pre ferable over conservative management, although it is unknown if the potential complications of this disease, even when asymptomatic, justify surgical morbidity.

Duplicaciones de las vías digestivas / Duplications of digestive tract

Las duplicaciones de las vías digestivas son anomalías congénitas infrecuentes, que se pueden localizar en cualquier parte del tubo digestivo, y son más comunes en los hombres. Hasta en un tercio de los pacientes, se acompañan de otras anomalías congénitas. Se han postulado tres teorías para explicar la causa de las duplicaciones: la de la recanalización de la luz intestinal, la del accidente vascular intrauterino y la de la unión o "hermanamiento" incompleta. Se clasifican en duplicaciones quísticas, cuando se comunican con la luz intestinal normal, y en duplicaciones tubulares, cuando no lo hacen; según algunas series, las primeras corresponden de 70 a 75 % de los casos informados y, las segundas, del 25 al 30 %. Poseen tres características esenciales, que son: estar unidas a cualquier segmento del tubo digestivo y con una irrigación sanguínea común; tener una capa muscular, y poseer un revestimiento epitelial que, generalmente, corresponde a la mucosa del mismo trayecto. Los síntomas más comunes son dolor abdominal inespecífico, náuseas, vómitos, hemorragia gastrointestinal, invaginación, obstrucción, ictericia y pancreatitis. El diagnóstico se basa, especialmente, en estudios imagenológicos, desde la radiografía simple hasta la resonancia magnética, siendo de gran importancia la ecografía prenatal. El tratamiento quirúrgico es la principal opción terapéutica, en la cual se usa cada vez más la vía laparoscópica

Alimentary tract duplications are rare congenital lesions that can occur anywhere in the gastrointestinal tract. Three hypotheses about their etiology had been proposed, The luminal recanalization theory, the intrauterine vascular accident theory, and the abortive twinning theory. According to some series, three quarters of gastrointestinal duplications are cystic with no communication to the adjacent alimentary tract, while the remaining are tubular and may communicate with the intestinal lumen.They have three essential characteristics: they are attached to any segment of the gastrointestinal tract and with the same blood supply, having a muscular layer and possessing an epithelial lining that corresponds generally to the mucosa of the same path. The most common symptoms are nonspecific abdominal pain, nausea, vomiting, gastrointestinal hemorrhage, invagination, obstruction, jaundice and pancreatitis. The diagnosis is based mainly on imaging studies, ranging from simple radiography to nuclear resonance, with prenatal ultrasound being of great importance. Surgical treatment is the main therapeutic option, being increasingly used the laparoscopic route

A Case of Ciliated Foregut Cyst of the Gallbladder / 대한소화기학회지

Congenital cysts of the gallbladder are extremely rare, hence only a few ciliated foregut cysts of gallbladder have been reported. We report a case of a 20-year-old woman presenting with mild right upper quadrant abdominal discomfort, with normal levels of serum bilirubin and liver function tests. Abdominal ultrasonography revealed a well-defined cystic mass measured about 2 cm attached to the neck of gallbladder, with internal echogenic debris suggesting a complicated cyst, such as a hemorrhagic cyst. Abdominal computed tomography and magnetic resonance cholangiopancreatography revealed similar findings. Laparoscopic cholecystectomy showed a slightly distended gallbladder. The size of cyst on the neck was 1.6x1.2 cm, and it contained mucosa lined by ciliated pseudostratified columnar epithelium and underlying smooth muscle layers. Histopathology identified a ciliated foregut cyst of gallbladder.

A Case of Intrauterine Lingual Cyst with Successful Delivery Assisting Ex Utero Intrapartum Treatment Procedure / 대한주산의학회잡지

Congenital lingual cyst is rare and there has been no prior case report about prenatally detected lingual cyst in Korea. When a huge oral cyst is observed at prenatal period and can cause life-threatening airway obstruction at birth, ex utero intrapartum treatment (EXIT) procedure is needed to secure the airway. Herein we present a baby with a prenatally detected huge oral cyst. He was delivered safely assisting EXIT procedure and underwent an operation for resection of the cyst from his tongue. The oral cyst was diagnosed as a lingual cyst with rare histologic type consisting mixed gastrointestinal and respiratory epithelium.

Quiste congénito de la valécula con alteraciones del peso: Caso clínico / Congenital vallecular cyst with weight disorders: Case report

Los quistes de la valécula son un trastorno raro en los niños; el estridor es el síntoma más frecuente y la alteración del crecimiento, el más infrecuente. Se presenta el caso de un niño de 2 meses referido por la escasa ganancia ponderal desde su nacimiento, con antecedentes de atragantamiento al alimentarse. No presentaba dificultad respiratoria, pero llamaba la atención la respiración bucal y la hiperextensión del cuello con lateralización hacia la derecha. El esofagograma mostró una masa hipofaríngea que desplazaba la laringe y reflujo del medio de contraste hacia el árbol bronquial. Por laringoscopia directa se resecó un quiste con técnica de marsupialización. La evolución fue favorable, sin recidivas, hasta el cuarto mes de seguimiento.

Vallecula cysts are a rare condition in children, with stridor being the most common symptom and growth alterations the more uncommon. We present a 2 months-old child referred by low weight gain from birth, with a history of choking when feeding. He had no respiratory distress but struck mouth breathing and neck hyperextension with lateralization to the right. The esophagogram showed a mass displacing hypopharynx and larynx reflux of contrast into the bronchial tree. The cyst was resected by direct laryngoscopy with marsupialization technique. The outcome was favorable, without recurrence until the fourth month of monitoring.

Progress in diagnosis and treatment of neonatal upper airway obstruction / 国际儿科学杂志

The most common etiology of neonatal upper airway obstruction is congenital abnormalities,including choanal atresia,Pierre Robin sequence,occupational lesions,laryngomalacia,and so on.Some patients are after birth with dyspnea,severe cases with suffocation,and even death.Due to lack of specific clinical manifestations in early neonatal periode,it is usually misdiagnosed as other respiratory diseases.Therefore,early identification of the etiology,as soon as possible to relieve the obstruction,is particularly critical in the prevention of suffocation for the upper airway obstruction in the neonatal period.

Comportamiento del quiste branquial en pacientes del Hospital General Docente Ciro Redondo García, Artemisa, 1993-2009 / Behavior of branchial cyst in patients admitted in the \"Ciro Redondo García\", General Teaching Hospital, Artemisa municipality, 1993-2009

El quiste branquial de origen congénito que aparece en la región lateral del cuello es, con frecuencia, motivo de consulta. Se tuvo como objetivo determinar el comportamiento de los quistes branquiales en el Hospital Ciro Redondo García, de Artemisa en el periodo de 1993 al 2009. Se realizó un estudio descriptivo transversal de los quistes branquiales diagnosticados en pacientes intervenidos quirúrgicamente en el Servicio de Cirugía Maxilofacial. Se estudiaron la edad, el sexo, el color de la piel, el lado del cuello afectado, el resultado histopatológico y los medios auxiliares empleados. Se encontraron 12 quistes branquiales que afectaron el 50 por ciento de ambos sexos, un 75 por ciento a pacientes entre 15 y 30 años y un 75 por ciento de la piel blanca, un 66,7 por ciento afectó el lado derecho del cuello y en un 100 por ciento de los casos se utilizó la biopsia y el ultrasonido como medios auxiliares para su diagnóstico. Se encontró epitelio escamoso estratificado en un 100 por ciento de los quistes y en un 91,6 por ciento el tejido linfoide. No se encontró predilección por el sexo, la mayoría de los pacientes eran menores de 30 años, de piel blanca y presentaban los quistes en el lado derecho del cuello. En todos los pacientes se empleó la biopsia y el ultrasonido como medios auxiliares de diagnóstico. El epitelio escamoso estratificado y el tejido linfoide fueron los hallazgos histopatológicos más relevantes(AU)

The congenital branchial cyst appearaing in the lateral region of the neck is frequently consultation reason. The aim was to determine the behavior of the branchial cysts in the patients admitted in the Ciro Redondo García General Teaching Hospital of Artemisa municipality from 1993 to 2009. A cross-sectional and descriptive study was conducted of the branchial cysts diagnosed in patients operated on in the Maxillofacial Surgery Service. Study variables were: age, sex, the skin color, neck involved side, histopathological result and the auxiliary means used. There appear 12 branchial cysts involving the 50 per cent of both sexes, a 75 per cent aged between 15 and 30 and a 75 per cent of white skin, a 66,7 per cent involved the neck right side and in a 100 per cent of cases authors used biopsy and ultrasound as auxiliary means for its diagnosis. There was a stratified squamous epithelium in a 100 per cent of cysts and in a 91,6 per cent the lymphoid tissue. There was not predominance of sex, most of patient were aged under 30, white skin and had cysts in the neck right side. In all patients authors used the biopsy and ultrasound as diagnostic auxiliary means. The stratified squamous epithelium and the lymphoid tissue were the more relevant histopathological findings(AU)

Hepatic biloma in the elderly: our experience on 6 patients / 中华肝胆外科杂志

Objective To retrospectively review our experience of treating hepatic biloma in the elderly.Methods Before cyst fenestration,the fistulas between the bile duct and biloma were ligated in the 6 patients.Result All the 6 patients were cured.On follow-up,there was no recurrence.Conclusion The procedure is feasible and safe in the treatment of biloma in the elderly.

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.

Experience of therapy for 86 patients with symptomatic congenital cyst of liver / 重庆医科大学学报

Objective:To investigate the therapy and efficacy in patients with symptomatic congenital cyst of liver.Methods: Eighty-six cases of congenital cyst of liver admitted from Jan. 2000 to Dec. 2006 were reviewed retrospectively.Results: Injection of ethanol(17 cases):with 2 cases of recurrence(11%).open surgery(48cases):with 4 cases of recurrence(8.3%). And laparoscopic surgery(21cases):with 1 case of recurrence(4.8%),We are found as the therapies. Conclusions:Methods of therapy for congenital cyst of liver include:injection of ethanol into the cyst cavity,open surgery,laparoscopic surgery.Laparoscopic fenestration is of minor invasion,faster recovery and shorter hospital stay.

A Case report of congenital uterine cyst / 대한산부인과학회지

Cysts of the uterus are classified into two groups, acquired and congenital. The congenital cysts which are thought to arise from Mullerian or Wolffian remnants, are exceedingly rare. The acquired uterine cysts are by far the more common of the two groups. This paper describes a case of a congenital uterine cyst, with a discussion of the differential diagnosis on clinical and pathologic findings and histogenetic origin with brief review of literature.

Cysts of the pancreas: a report of two cases

A case of pseudosyst of the pancreas is reported, demonstrating the successful use of marsupialization. Because of the relative rarity of this condition, special attention is called to the ease with which it can be mistaken for other more common causes of encysted fluids, and to the special means for differentiating it.

Tailgut Cyst A case report

Tailgut cyst is a rare congenital lesion in retrorectal space. The clinical significance of tailgut cyst presents its morbidity that occurs in the unrecognized and incompletely treated lesion. A forty four year-old female patient visited with lower abdominal pain during defecation. Preoperative abdominopelvic MRI and endorectal ultrasonography revealed a retrorectal mass suggestive of leiomyoma, dermoid cyst, teratoma, or duplication cyst of rectum. She underwent complete resection of retrorectal mass by transsphincteric approach. The mass was multilocular cyst lined by multiple types of epithelium. It was histologically confirmed as a tailgut cyst. She recovered uneventfully. This report includes the case and a brief review of tailgut cyst.

Congenital Uterine Cyst: A case report

I experienced a case of a congenital intramural cyst of the uterine fundus. On examination by light and electron microscope it was composed of a single layer of thin atrophied lining epithelium without secretory activity and was found to be derived from the paramesonephric duct. This case is presented with a brief review of the literature.