Clinical characteristics and serious complications of esophageal button battery ingestion in the pediatric on 83 cases / 中华耳鼻咽喉头颈外科杂志

Objective: To analyze the clinical characteristics and complications of esophageal foreign bodies of button battery ingestion in children. Methods: A retrospective descriptive study included 83 children who were hospitalized in our hospital on account of button battery ingestion from January 2011 to December 2021. There were 50 males (60.2%) and 33 females (39.8%). The age ranged from 7.6 months to one month off 10 years, with a median age of 18 months. The data of patient demographics and time from ingestion to admission, location, symptoms, management, complications, and follow-up outcome were recorded. SPSS17.0 software was used for statistical analysis. Results: Seventy-two children (86.7%) were younger than 3 years old. The time from ingestion to admission ranged from 1 h to 2 months, with a median time of 8 h. Among the 63 children who were first diagnosed in our hospital, the most common clinical symptoms were nausea and vomiting (32 cases, 50.8%), dysphagia (31 cases, 49.2%), salivation (11 cases, 17.5%) and fever (10 cases, 15.9%). Seventy-three of 83 cases had complete preoperative diagnostic tests, and 55 cases (75.3%) were diagnosed by X-ray. In 56 cases (76.7%), the foreign badies were impacted in the upper third of esophagus. In 72 cases (86.7%), the foreign badies were removed by rigid esophagoscopy. 23 (27.7%) had serious complications, including tracheoesophageal fistula in 15 cases(TEF;65.2%), vocal cord paralysis (VCP;34.8%) in 8 cases, esophageal perforation in 3 cases (EP;13.0%), hemorrhage in 3 cases(13.0%), mediastinitis in 3 cases (13%), and periesophageal abscess in 1 case (4.3%). There were significant differences in the exposure time of foreign bodies and unwitnessed ingestion by guardians in the complications group (P<0.05). 2 cases died (2.4%)respectively due to arterial esophageal fistula bleeding and respiratory failure caused by stent displacement during the treatment of tracheoesophageal fistula. Conclusion: Accidental button battery ingestion can be life-threatening. and it mostly happens in children under 3 years old. Serious complications may happen cause of non-specific clinical manifestations and unwitnessed ingestions. Anterior and lateral chest X-ray is the first examination choice. Tracheoesophageal fistula is the most common serious complication.

Atresia esofágica tipo III associada a ânus imperfurado: um relato de caso / Esophageal atresia type III associated to imperforate anus: a case report

Introdução: As malformações congênitas são defeitos que ocorrem durante o desenvolvimento embrio-fetal, principalmente entre a terceira e oitava semana de gestação, podendo ser suspeitado no período pré-natal. A atresia de esôfago com ou sem fístula traqueoesofágica pode estar associada a outras anomalias congênitas, como o ânus imperfurado, o qual faz parte uma síndrome denominada malformação anorretal. Objetivo: discutir o caso de um recém-nascido (RN) portador de atresia de esôfago tipo III e ânus imperfurado, bem como associar suas ocorrências simultâneas. Relato de caso: paciente do sexo masculino, advindo de uma gestação gemelar complicada por pré-eclâmpsia, apresentava ao nascimento ausência do reflexo de busca e sucção, bradicardia e cianose central. Foi diagnosticado com ânus imperfurado e atresia esofágica. Foram realizados cirurgia de Colostomia em Duas Bocas e tratamento cirúrgico da atresia de esôfago. O paciente ficou 48 dias em incubadora com intubação orotraqueal e ventilação mecânica por 26 dias. Resultado: a programação pós- alta indicada foi o acompanhamento com cirurgião pediátrico e neurocirurgião, tendo evoluído bem e aguardando anorretoplastia. Conclusão: o rápido diagnóstico é tão necessário, nesses casos, quanto as abordagens cirúrgicas. O apoio psicológico aos familiares é de extrema importância, pois visa a melhor aceitação da família frente ao caso, propiciando dessa forma, melhores prognóstico e tratamento do paciente.

Introduction: Congenital malformations are defects that occur during embryo-fetal development, especially between the third and eighth week of pregnancy, and may be suspected in the prenatal period. Esophageal atresia with or without tracheoesophageal fistula may be associated with other congenital anomalies, such as the imperforate anus, which is part of a syndrome called anorectal malformation. Objective: to discuss the case of a newborn (NB) with type III esophageal atresia and imperforate anus, as well as to associate its simultaneous occurrences. Case report: male patient, resulting from a twin pregnancy complicated by pre-eclampsia, had at birth the absence of the search and suction reflex, bradycardia and central cyanosis. He was diagnosed with imperforate anus and esophageal atresia. Double-barrel colostomy surgery and esophageal atresia surgery were performed. The patient spent 48 days in an incubator with orotracheal intubation and mechanical ventilation for 26 days. Results: the post-discharge schedule indicated was for follow-up with a pediatric surgeon and neurosurgeon. Patient evolved well and awaits anorectomy. Conclusion: rapid diagnosis is as necessary in these cases as surgical approaches. Psychological support to family members is extremely important, as it aims at better family acceptance in the case, thus providing better prognosis and treatment for the patient.

Tratamiento conservador del quilotórax posquirúrgico en paciente pediátrico / Conservative treatment of post-surgical chylothorax in pediatric patient

INTRODUCCIÓN. El quilotórax resulta de un daño al conducto torácico por ruptura, laceración, desgarro o compresión. Es una patología rara de derrame pleural en la edad pediátrica, pero frecuente como complicación posterior a cirugía cardiotorácica. La base del tratamiento conservador se ha fundamentado en: drenaje inicial, modificación de la dieta, uso de somatostatina o análogos sintéticos como octreotide, cirugía, prevención y manejo de complicaciones. Fue preciso describir la experiencia institucional clínica así como su abordaje. CASO CLÍNICO. Paciente masculino de 4 meses de edad, que ingresó a la Unidad Pediátrica Área de Emergencias del Hospital de Especialidades Carlos Andrade Marín, el 13 de septiembre de 2019 con antece-dente quirúrgico de atresia de esófago corregida en etapa neonatal. Acudió con dificultad respiratoria, radiografía de tórax que evidenció derrame pleural derecho, toracentesis diagnóstica con salida de líquido de aspecto turbio y lechoso; se colocó tubo de tórax derecho. Se prescribió ayuno inicial, nutrición parenteral durante 4 semanas hasta comprobar resolución del quilotórax. Fue dado de alta en condición estable tras 43 días de hospitalización. DISCUSIÓN. La evidencia científica registró que el tratamiento conservador del quilotórax se basó en: drenaje, reposo digestivo inicial, nutrición parenteral, modificación cualitativa de la dieta enteral y uso de octreotide; el mismo que fue aplicado al paciente de este caso clínico con evolución favorable. CONCLUSIÓN. El tratamiento conservador y multidisciplinario en el abordaje del qui-lotórax fue exitoso y no necesitó manejo quirúrgico.

INTRODUCTION. Chylothorax results from damage to the thoracic duct by rupture, la-ceration, tear or compression. It is a rare pathology of pleural effusion in pediatric age, but frequent as a complication after cardiothoracic surgery. The basis of conservative treatment has been based on: initial drainage, diet modification, use of somatostatin or synthetic analogues such as octreotide, surgery, prevention and management of complications. It was necessary to describe the clinical institutional experience as well as its approach. CLINICAL CASE. A 4-month-old male patient was admitted to the Emergency Area Pediatric Unit of the Carlos Andrade Marín Specialties Hospital on september 13, 2019 with a surgical history of esophageal atresia corrected in the neonatal stage. He went with respiratory distress, chest X-ray that showed right pleural effusion, diagnostic thoracentesis with outflow of cloudy and milky fluid; a right chest tube was placed. Initial fasting was prescribed, parenteral nutrition for 4 weeks until resolution of the chylothorax was verified. He was discharged in stable condition after 43 days of hospitalization. DISCUSSION. The scientific evidence recorded that the conservative treatment of chylothorax was based on: drainage, initial digestive rest, parenteral nutrition, qualitative modification of enteral diet and use of octreotide; the same that was applied to the patient of this clinical case with favorable evolu-tion. CONCLUSION. Conservative and multidisciplinary treatment in the approach to chylothorax was successful.

Double-lumen Aortic Arch: Persistence of the Fifth Aortic Arch?

Abstract Double-lumen aortic arch is a rare congenital anomaly related to persistence of the fifth aortic arch. It may be found alone or in association with other anatomical changes of the heart. We report a case of double-lumen aortic arch associated with coarctation of the aorta and patent ductus arteriosus in a child with a congenital malformation known as the VACTERL association (vertebral defects, imperforate anus, cardiopathy, tracheoesophageal fistula, renal abnormalities and limb anomalies).

Effect of muscle relaxants on the prognosis of neonates with congenital esophageal atresia-tracheoesophageal fistula after surgery / 中国当代儿科杂志

OBJECTIVE@#To summarize the experience in the application of muscle relaxants in the perioperative period in neonates with congenital esophageal atresia-tracheoesophageal fistula (EA-TEF).@*METHODS@#A retrospective analysis was performed on the medical data of 58 previously untreated neonates with EA-TEF who were treated in the Neonatal Center of Beijing Children's Hospital, Capital Medical University from 2017 to 2019. The incidence rate of anastomotic leak was compared between the neonates receiving muscle relaxants for different durations after surgery (≤ 5 days and > 5 days). The correlation between the duration of postoperative use of muscle relaxants and the duration of mechanical ventilation was evaluated.@*RESULTS@#Among the 58 neonates with EA-TEF, 44 underwent surgery, among whom 35 with type III EA-TEF underwent thoracoscopic surgery. Among these 35 neonates, 30 (86%) received muscle relaxants after surgery, with a median duration of 4.75 days, and 6 (18%) experienced anastomotic leak. There was no significant difference in the incidence rate of anastomosis leak between the ≤ 5 days and > 5 days groups (@*CONCLUSIONS@#Prolonged use of muscle relaxants after surgery cannot significantly reduce the incidence of anastomotic leak, but can prolong the duration of invasive mechanical ventilation in neonates with EA-TEF. Therefore, prolonged use of muscle relaxants is not recommended after surgery.

Características clínico-quirúrgicas de la atresia esofágica en el Instituto Nacional de Salud del Niño San Borja, Lima, Perú. 2015-2017 / Clinical-surgical characteristics of esophageal atresia in the National Institute of Children's Health San Borja, Lima, Peru. 2015-2017

RESUMEN Objetivo : Describir las características clínico-quirúrgicas y sociodemográficas de pacientes con atresia esofágica (AE) del Instituto Nacional de Salud del Niño San Borja (INSN-SB) 2015-2017. Material y métodos : Estudio observacional descriptivo de casos con AE 2015-2017. Los datos se procesaron y analizaron en el programa SPSS v20, las variables cualitativas fueron expresadas en frecuencias absolutas y relativas. Resultados : Se analizaron 74 casos con AE, 55,4% (n=41) fueron de sexo masculino. Según edad gestacional 45 a término, 28 pre-término y un post-término. El 59,5% tuvieron peso adecuado al nacer. 28,4% tuvieron control prenatal (CPN) completo y 68,9% no tuvieron diagnóstico prenatal de AE. La atresia con fistula traqueo-esofágica (FTE) distal fue la más frecuente con 74,3% (n=55). Se presentó sialorrea en 55 pacientes (74,3%) y disnea en 54 (72,9%). Predominaron las malformaciones cardiacas en el 27,1%, seguida por digestivas 20,3% y genitourinarias 17,6%. Solo 26 tuvieron síndromes asociados, VACTERL en 12 casos. La anastomosis termino-terminal y cierre de fistula se realizó en 55 pacientes (74,3%). 48 presentaron complicaciones tempranas, predominando sepsis (20,3%) y 46 complicaciones tardías, siendo reflujo gastroesofágico el más frecuente 29,7%. La mortalidad fue 10,8%. Conclusiones : La mayoría fue de sexo masculino, a término, con adecuado peso al nacer y sin CPN. La AE con FTE distal fue la más frecuente. Sialorrea y disnea fueron los síntomas predominantes y malformaciones cardiacas y digestivas las más asociadas. La anastomosis término-terminal y cierre de fístula fue la técnica más frecuente, como complicaciones sepsis y reflujo gastroesofágico. La mortalidad fue baja.

ABSTRACT Objective : Describe the clinical-surgical and sociodemographic characteristics of patients with esophageal atresia (EA) of the Instituto Nacional de Salud del Niño San Borja (INSN-SB) during 2015-2017. Material and methods : Descriptive observational study of cases with EA 2015-2017. The data was processed and analyzed in the SPSS v20 program, the absolute and relative frequency distributions were expressed in tables. Results : 74 cases with AE were analyzed, 55.4% (n=41) were male. According to gestational age, 45 were at term, 28 pre-term and one post-term. 59.5% had adequate weight at birth. Only 28.4% had complete prenatal care (CPN). 68.9% did not have a prenatal diagnosis of AE. Atresia with distal tracheoesophageal fistula (TEF) was the most frequent in 74.3% (n=55). Hypersalivation occurred in 55 patients (74.3%), followed by dyspnea in 54 (72.9%). Cardiac malformations predominated in 27.1%, followed by digestive 20.3% and genitourinary 17.6%. Only 26 had associated syndromes, VACTERL in 12 cases. End-to-end anastomosis and fistula closure were performed in 55 patients (74.3%). 48 presented early complications, predominating sepsis (20.3%) and 46 late complications, with gastroesophageal reflux being the most frequent 29.7%. Regarding mortality, 8 (10.8%) died. Conclusions : Most of the cases were male, at term, with adequate weight at birth and without CPN. AE with distal TEF was the most frequent. Sialorrhea and dyspnea were the predominant symptoms and cardiac and digestive malformations the most associated. End-to-end anastomosis surgery and fistula closure was the most frequent technique. Complications were sepsis and gastroesophageal reflux. Mortality was low.

Fístula traqueoesofágica en H en el período neonatal: dificultades en el diagnóstico y diferentes abordajes terapéuticos. Serie de casos / H-type tracheoesophageal fistula in neonatal period: Difficulties in diagnosis and different treatment approaches. A case series

La fístula traqueoesofágica congénita sin atresia esofágica asociada, conocida como tipo H, es una anomalía infrecuente. Se manifiesta con episodios de tos, ahogo y cianosis durante la alimentación y/o neumonía recurrente.Si bien los síntomas están usualmente presentes desde el nacimiento, el diagnóstico es difícil. La rareza de esta patología, los síntomas no específicos y las limitaciones en la demostración radiológica y endoscópica de la fístula contribuyen, a menudo, a la demora entre la presentación y la confirmación del diagnóstico.Se describen las manifestaciones clínicas, los métodos de evaluación y el tratamiento de 3 neonatos con esta patología, y se presentan recomendaciones para el diagnóstico a fin de evitar demoras innecesarias en el manejo de las fístulas en H.

Congenital tracheoesophageal fistula not associated with esophageal atresia, known as H-type fistula, is an uncommon anomaly. It presents with cough, choking, and cyanosis during feeding and/or recurrent pneumonia. Although symptoms are usually present from birth, diagnosis is difficult. The rarity of this disease, non-specific symptoms, and the limitations of radiological and endoscopic confirmation of the fistula often result in a delay between presentation and diagnosis confirmation. Here we describe the clinical manifestations, assessment methods, and management of 3 newborn infants with H-type tracheoesophageal fistula, together with diagnosis recommendations to prevent unnecessary delays in the management of this condition.

Communicating Bronchopulmonary Foregut Malformation Type III with Pulmonary Sequestration Diagnosed in a Newborn: A Case Report

Communicating bronchopulmonary foregut malformation (CBPFM) is a communication between the respiratory and gastrointestinal tracts that can be difficult to differentiate from pulmonary sequestration or H-type tracheoesophageal fistula (TEF) because of the similarities in clinical features. A female neonate born at full term had been experiencing respiratory difficulty during feeding from the third day of life. The esophagography performed to rule out H-type TEF revealed that the esophageal bronchus directly communicated with the left lower lobe (LLL) of the lung. Lobectomy of the LLL, fistulectomy of the esophagobronchial fistula, and primary repair of the esophagus were performed. Finally, CBPFM type III with pulmonary sequestration was confirmed on the basis of the postoperative histopathological finding. We report the first newborn case of CBPFM type III with pulmonary sequestration in Korea.

Factores que influyen en los resultados de fístula traqueoinnominada I / Influential factors in the results of traqueoinnonate fistula I

Introducción: La fístula traqueoinnominada, aunque poco frecuente, constituye una amenaza potencial para la vida, si no es reconocida y tratada oportunamente. Objetivo: Describir los resultados obtenidos e identificar las acciones que permitan identificarlos. Métodos: Se realizó el estudio retrospectivo, sobre una base de datos prospectiva de pacientes con fístulas traqueonominadas tratados entre 1991 y 2013. Los enfermos se clasificaron según la operación previa: traqueostomía, uso de tubo en T en reintervenciones con intensión de resecar el segmento traqueal estenótico. La revisión de la patogenia y de los métodos diagnósticos permitirán una mejor comprensión para enfrentar esta temible complicación. Se analizan los factores que pudieron influenciar los resultados. Resultados: Cuatro pacientes pertenecían al sexo femenino. Dos tenían una traqueostomía, dos fueron reoperados después de tratamiento con tubo en T; a uno se le realizó resección y anastomosis traqueal y en el otro no fue posible porque se produjo lesión puntiforme traqueal durante la disección mediastinal. Al último se le había colocado un tubo en T, mediante traqueofisura. En dos de tres pacientes, el sangrado centinela se interpretó incorrectamente. En el primero, se consideró que era la consecuencia del tratamiento anticoagulante y en el segundo no se dio valor a la pequeña cantidad de sangre en los esputos. Conclusiones: Un alto índice de sospecha, el diagnóstico temprano y las medidas al pie de la cama: compresión digital y colocación de un tubo ET o una cánula de traqueostomía con el manguito hiperinsuflado, constituyen la única esperanza para estos pacientes(AU)

Introduction: The tracheoinnominate fistula, though a rare entity, is a potential life threat if it is not promptly recognized and treated. Objective: To analyze the influential factors in the results of tracheoinnominate fistula I. Methods: Retrospective study supported on the prospective databases from patients with tracheoinnominate fistulae, who had been treated from 1991 to 2013. They were classified according to their previous surgery: tracheostomy, use of a T-tube or re-interventions aimed at resecting the stenotic tracheal segment. The review of pathogeny and of diagnostic methods allowed a better understanding to face this severe complication. The factors that could have an impact on the results were also analyzed. Results: Four patients were women. Two patients had undergone tracheostomy; two had been reoperated after treatment with T-tube; resection and tracheal anastomosis were performed in one of them but these procedures were not possible to be applied to the other because of a punctiform tracheal lesion during the mediastinal dissection. A T-tube had been placed in this last patient through tracheofissure. The sentinel bleeding in two of three patients was not properly evaluated. In one of them, the bleeding was considered to be the result of anticoagulant treatment whereas in the other, the small amount of blood in the sputum was underassessed. Conclusions: High amount of suspicion, early diagnosis and application of bedside measures such as digital compression, placement of a T-tube or a tracheostomy cannula with overinflated cuff are the only hopeful options for these patients(AU)

Cuidados de las fístulas traqueoesofágicas postintubación / Care of the post-intubation tracheoesophageal fistulae

RESUMEN Introducción: Las fístulas traqueoesofágicas posintubación son lesiones graves que presentan alta frecuencia de complicaciones y mortalidad. Objetivo: Actualizar el diagnóstico y tratamiento de pacientes con esta afección. Método: Se revisó la literatura en PUBMED/Medline e Infomed con las palabras clave: fístula, traqueoesofágica, fístula traqueoesofágica, postintubación y las correspondientes en inglés. Se revisaron las referencias bibliográficas de los artículos en busca de publicaciones relevantes. Se escogieron, preferentemente, trabajos prospectivos. Los informes de casos sólo se tomaron en cuenta cuando hacían referencia a alguna técnica novedosa. Desarrollo: Se encontraron 4260 artículos en Medline/Pubmed y 17 en Infomed. No se encontraron ensayos clínicos, metanálisis, ni revisiones sistemáticas. La mayoría de los artículos revisados hacen referencia a presentación de casos y algunos fueron estudios retrospectivos. Las presentaciones de casos se usaron solo si describían una técnica novedosa. Conclusiones: La intubación endotraqueal prolongada es la causa principal de FTEs. La patogenia se asocia con isquemia secundaria a compresión de las paredes traqueal posterior yesofágica anterior, entre el manguito insuflado del tubo endotraqueal y la sonda nasogástrica. El tratamiento conservador, generalmente, no cura la fístula, pero puede limitar la contaminación del árbol traqueobronquial y garantiza la nutrición. La derivación esofágica raramente está indicada, excepto cuando persiste entrada a la VR del contenido gastrointestinal. Actualmente, el tratamiento definitivo es la sutura esofágica y, en la mayoría de los casos, la resección y anastomosis traqueal(AU)

ABSTRACT Introduction: Post-intubation tracheoesophageal fistulae are severe lesions with high frequency of complication and high rate of mortality. Objective: To update the knowledge about the diagnosis and treatment of patients with this disease. Method: Literature review made in PUBMEDF/Medline and Infomed using the keywords: fistula, traqueoesophageal, tracheoesophageal fistula, and post-intubation in Spanish and English. References of articles were also reviewed to find out relevant publications. Prospective papers were particularly chosen. The case reports were only taken into account when they made reference to some novel technique. Results: In this search, 4260 articles were found in Medline/Pubmed and 17 in Infomed databases. No clinical assays, meta-analysis or systematic reviews were found. Most of the articles made reference to case reports and some to retrospective studies. The case reports were only used if they described a novel technique. Conclusions: Prolonged endotracheal intubation is the main cause of traqueoesophageal fistula. The pathogeny is associated to ischemia secondary to compression of posterior tracheal wall and anterior esophageal wall between the inflated cuff of the endotracheal tube and the nasogastric probe. In general, the conservative treatment does not cure the fistula but may restrict contamination of the tracheobronchial tree and guarantee nutrition. The esophageal shunt is rarely indicated, except when the gastrointestinal content continues entering into the VR. At present, the definitive treatment is the esophageal suturing and in most of cases, the resection and tracheal anastomosis(AU)

A successful pregnancy outcome in a case of maternal VACTERL / Philippine Journal of Obstetrics and Gynecology

@#Vertebral anomalies-anal atresia-cardiac abnormalities-tracheoesophageal fistula-renal agenesis-limb (VACTERL) defects association is a rare congenital disease. While most scientific literature focus on the clinical presentation and management of pediatric patients with this condition, this paper focuses on the challenges faced by a 22-year-old primigravid, who was able to carry a pregnancy to term, despite the many anomalies associated with being afflicted with VACTERL.

Esophageal Reconstruction with Gastric Pull-up in a Premature Infant with Type B Esophageal Atresia

Esophageal atresia (EA) with proximal tracheoesophageal fistula (TEF; gross type B) is a rare defect. Although most patients have long-gap EA, there are still no established surgical guidelines. A premature male infant with symmetric intrauterine growth retardation (birth weight, 1,616 g) was born at 35 weeks and 5 days of gestation. The initial diagnosis was pure EA (gross type A) based on failure to pass an orogastric tube and the absence of stomach gas. A “feed and grow” approach was implemented, with gastrostomy performed on postnatal day 2. A fistula was detected during bronchoscopy for recurrent pneumonia; thus, we confirmed type B EA and performed TEF excision and cervical end esophagostomy. As the infant's stomach volume was insufficient for bolus feeding after reaching a body weight of 2.5 kg, continuous tube feeding was provided through a gastrojejunal tube. On the basis of these findings, esophageal reconstruction with gastric pull-up was performed on postnatal day 141 (infant weight, 4.7 kg), and he was discharged 21 days postoperatively. At 12 months after birth, there was no catch-up growth; however, he is currently receiving a baby food diet without any complications. In patients with EA, bronchoscopy is useful for confirming TEF, whereas for those with long-gap EA with a small stomach volume, esophageal reconstruction with gastric pull-up after continuous feeding through a gastrojejunal tube is worth considering.

Successful Management of Acquired Tracheomalacia of Patients With Amyotrophic Lateral Sclerosis: A Report of Three Cases

Tracheomalacia is characterized by weakness of the tracheal walls and supporting cartilage. It results in dynamic compression of the airway, where the cross-sectional area of the trachea is reduced by expiratory compression. Acquired tracheomalacia results from complications associated with the use of endotracheal or tracheostomy tubes. In this report, we present three cases of patients with amyotrophic lateral sclerosis (ALS) successfully treated for tracheomalacia, including one case where the patient underwent surgery for combined tracheoesophageal fistula. We discuss the appropriate management strategies for tracheomalacia in patients with ALS. Through these case reports, we note the results of ALS patients who will have tracheostomy, and who are therefore at risk of sustaining a long term high cuff pressure, this study provides an evaluation for tracheomalacia and therapeutic management which should be considered for improving patient care outcomes.

Treatment of large persistent tracheoesophageal peristomal fistulas using silicon rings / Tratamento de fístula periestomal traqueoesofágica grande e persistente com anéis de silicone

Abstract Introduction: Tracheoesophageal peristomal fistulae can often be solved by reducing the size of the fistula or replacing the prosthesis; however, even with conservative techniques, leakage around the fistula may continue in total laryngectomy patients. Also, several techniques have been developed to overcome this problem, including injections around the fistula, fistula closure with local flaps, myofascial flaps, or free flaps and fistula closure using a septal perforation silicon button. Objective: To present the results of the application of silicon ring expanding the voice prosthesis in patients with large and persistent peri-prosthetic fistula. Methods: A voice prosthesis was fitted to 42 patients after total laryngectomy. Leakage was detected around the prosthesis in 18 of these 42 patients. Four patients demonstrated improvement with conservative methods. Eight of 18 patients who couldn't be cured with conservative methods were treated by using primary suture closure and 4 patients were treated with local flaps. As silicon ring was applied as a primary treatment in the 2 remaining patients and also, applied to 2 patients who had recurrence after suture repair and to 2 patients who had recurrence after local flap implementation. Silicon rings were used in a total of 6 patients due to the secondary trachea-esophageal fistula. Patients were treated with provox-1 initially and later with provox-2. At the time of leakage around the fistula, 6 patients had provox-2. Results: Fistulae were treated successfully in 6 patients, and effective speech of patients was preserved. Patients experienced no adaptation problem. Prosthesis changing time was not different between silicon rings expanded and normal prosthesis applied patients. Silicon ring combined voice prosthesis was used 26 times; there was no recurrence in fistula complication during 29 ± 6 months follow up. Conclusion: Silicon rings for modified expanded voice prosthesis seems to be an effective treatment for persistent peri-prosthetic leakage, for both, fistula closure and preserving the patients speech.

Resumo Introdução: Fístulas traqueoesofágicas persistentes podem ser resolvidas através da redução do tamanho da fístula ou substituição da prótese; no entanto, mesmo com técnicas conservadoras, o pertuito em torno da fístula pode continuar em pacientes com laringectomia total. Além disso, várias técnicas têm sido desenvolvidas para superar esse problema, inclusive injeções ao redor da fístula, fechamento da fístula com retalhos locais, retalhos miofasciais ou retalhos livres e fechamento da fístula com um botão septal de silicone. Objetivo: Apresentar os resultados da aplicação de anel de silicone para expansão da prótese vocal em pacientes com grandes fístulas periprotéticas persistentes. Método: Prótese vocal foi colocada em 42 pacientes após laringectomia total, e fístula foi detectada ao redor da prótese em 18 desses 42 pacientes. Quatro pacientes obtiveram melhora com métodos conservadores. Oito dos 18 pacientes que não obtiveram sucesso com métodos conservadores foram tratados usando sutura primária e quatro pacientes foram tratados com retalhos locais. Um anel de silicone foi aplicado inicialmente nos dois pacientes restantes e, também, aplicado a dois pacientes que tiveram recorrência após a técnica de sutura e a dois pacientes que tiveram recorrência após a utilização de retalho local. No total, seis pacientes receberam anéis de silicone em decorrência da fístula traqueoesofágica secundária. Os pacientes haviam sido tratados com provox-1 inicialmente e posteriormente com provox-2. No momento da detecção da fístula em torno do estoma, seis pacientes haviam recebido provox-2. Resultados: A fístula foi tratada com sucesso em seis pacientes. Além disso, após o tratamento a fala foi mantida de forma eficaz. Não houve problema de adaptação. O tempo de troca da prótese expandida com os anéis de silicone não foi diferente do tempo que se leva para a colocação da prótese normal. O anel de silicone combinado com a prótese vocal foi usado 26 vezes em pacientes na época da troca de prótese e não houve recorrência da fístula durante os 29 ± 6 meses de acompanhamento. Conclusão: Os resultados sugerem que em casos de grandes fístulas peri-prostéticas persistentes, anéis expandidos de silicone e prótese vocal modificada são eficazes tanto para o fechamento da fístula como para a manutenção da fala do paciente.

Relato de um caso de associação de VACTERL e discussão acerca de seus aspectos prognósticos / Case report of VACTERL association and discussion about its prognostic aspects

Associação de VACTERL corresponde à combinação de três ou mais das seguintes malformações: Vertebrais, atresia Anal, alterações Cardíacas, fístula Traqueo-esofágica com atresia de Esôfago, anomalias Renais e de membros (Limbs). Sua etiologia ainda é desconhecida, mas acredita-se que seja multifatorial, associada a mutações em genes como FOXF1 e ZIC3 e a fatores de risco maternos. No presente relato, descreve-se um caso de associação de VACTERL diagnosticado no Hospital Universitário Regional de Maringá (HURM). Paciente L.P.N., sexo feminino, um dia de vida, foi referenciada para UTI neonatal devido ao quadro de desconforto respiratório, cianose e salivação intensa na sala de parto, sendo diagnosticada com atresia de esôfago. No 9º dia de vida realizou-se correção cirúrgica da atresia esofá- gica e fístula traqueo-esofágica. Outras malformações foram observadas, detectando-se comunicação interventricular perimembranosa moderada e rim direito ausente, sugerindo diagnóstico de VACTERL. Com três anos e nove meses, apresenta-se eutrófica, saudável e em acompanhamento multiprofissional. Apesar de ser uma afecção rara, esta associação necessita de expertise dos profissionais de saúde, para que haja detecção precoce e tratamento de suas alterações e complicações. Além disso, se manejada da forma correta, a criança apresenta boa evolução e perspectivas de uma vida normal. O prognóstico depende da extensão e da gravidade das anomalias associadas, bem como da capacitação dos pais e equipe profissional (AU)

VACTERL Association corresponds to the combination of three or more of the following defects: Vertebral, Anal atresia, Cardiac changes, Tracheo-esophageal fistula, Esophagus atresia, Renal anomalies and Limbs. Its etiology is still unknown, but believed to be multifactorial, associated with mutations in genes as FOXF1 and ZIC3 and maternal risk factors. This report describes a case of VACTERL diagnosed in the Regional University Hospital of Maringá (HURM). L.P.N. female patient, 1 day in life, was referred to neonatal ICU due to respiratory discomfort, cyanosis and salivation in the delivery room, and diagnosed with esophageal atresia. On the 9th day of life surgical repair of esophageal atresia and tracheoesophageal fistula was performed. Other malformations were observed by detecting moderate perimembranous ventricular septal defect and absent right kidney, suggesting perimembranous ventricular septal defect and absent right kidney, suggesting VACTERL diagnostic. At the age of 3 years and 9 months, presents eutrophic, healthy, and in multiprofessional follow up. Despite being a rare disease, this association requires expertise of health professionals, in order to be early detected and to treat its alterations and complications. Also, if handled correctly, the child shows good performance and prospects of a normal life. The prognosis depends on the extent and severity of associated anomalies, as well as the training of parents and professional staff (AU)

Clinical Study of Congenital Esophageal Stenosis: Comparison according to Association of Esophageal Atresia and Tracheoesophageal Fistula / 대한소아소화기영양학회지

PURPOSE: Congenital esophageal atresia (CES) is a rare congenital disease. The severity of symptoms is variable; thus, diagnosis is difficult and tends to be delayed. CES is frequently accompanied by esophageal atresia (EA) with/without tracheoesophageal fistula (TEF). We investigated the characteristics of CES by reviewing our experience with CES patients and researched the differences between CES with EA-TEF and isolated CES. METHODS: A total of 31 patients underwent operations for CES were reviewed retrospectively. The patients were divided into two groups according to the association with EA-TEF, and compared the differences. RESULTS: Sixteen boys and 15 girls were included. The mean age at symptom onset was 8 months old, and the mean age at diagnosis was 21 months old. Nine patients with EA-TEF were included group A, whereas the other 22 patients were assigned to group B. There were no differences in sex, gestational age, associated anomalies and pathologic results between the groups. In group A, the age at diagnosis and age at surgery were younger than in group B despite the age at symptom occurrence being similar. Postoperative complications occurred only in group A. CONCLUSION: In this study, symptoms occurred during the weaning period, and vomiting was the most frequent symptom. CES patients with EA-TEF tended to be diagnosed and treated earlier despite the age at symptom occurrence being similar. CES patients with EA-TEF had more postoperative complications; therefore, greater attention should be paid during the postoperative period.

Clinical Review of Spontaneous Neonatal Gastric Perforation / 소아외과

PURPOSE: Spontaneous neonatal gastric perforation is a rare but fatal disease with unclear etiology. In this study, we reviewed its clinical manifestations, outcomes, and discussed the etiology and prognostic factors. METHODS: There were 12 neonates with gastric perforation in our hospital from 1989 to 2015. Their medical records were reviewed retrospectively including birth record, associated disease, site and size of perforation, type of surgical management, clinical outcome. Also, the prognostic factors were analyzed. RESULTS: The median gestational age and birth weight was 32 weeks (range, 26-43 weeks; preterm birth rate, 66.7%) and 1,883 g (range, 470-4,400 g), respectively. Five patients had associated gastrointestinal anomalies including esophageal atresia and tracheoesophageal fistula (two patients), midgut volvulus, non-rotation and microcolon, and meconium plug syndrome. The median age at surgery was six days after birth (range, 2-13 days), and the median weight at surgery was 1,620 g (range, 510-3,240 g). Upper third part of stomach was the most frequently involved location of perforation. The size of perforation varied from pin point to involving the whole greater curvature. Primary repairs were done in seven cases, and in five cases, resections of necrotic portion were needed. Mortality rate was 33.3% (n=4), morbidity (re-operation) rate was 16.7% (n=2). The causes of death were sepsis (n=3), and heart failure from Ebstein anomaly (n=1). The median hospital stay was 92.5 days (range, 1-176 days). The factors mentioned as prognostic factors in previous studies showed no significant relations to the mortality and morbidity in our study. CONCLUSION: There were improvements of outcomes in patients with large size perforation. As previous studies, we assume these improvements were possible due to the improvements of critical care medicine. Given that rare incidence, a multi-center study can help us get a better understanding of this disease, and a better outcome.

Esophageal Atresia with Bronchogenic Cyst / 소아외과

A baby was diagnosed with esophageal atresia (EA) with tracheoesophageal fistula (TEF) on the next day after birth, and end-to-end anastomosis of esophagus with TEF ligation was performed. The distance between proximal and distal esophageal pouch was checked as 3 vertebral body lengths and a 1 cm-sized bronchogenic cyst (BC) was identified near carina on the right side, just below the proximal esophageal pouch. This case report described the baby who have a BC was located between the both esophageal pouch and a longer esophageal gap than usual EA with distal TEF.

Síndrome de Klippel-Feil con fistula traqueoesofagica, pulgar bifido y angiolipoma cerebral / Klippel-Feil syndrome with tracheoesophageal fistula, bifid thumb and cerebral angiolipoma

El síndrome de Klippel-Feil es una malformación congénita de la charnela cráneo-cervical compleja que involucra vértebras y visceras, caracterizada por la tríada clásica de cuello corto, limitación de movimientos de la cabeza por la fusión de vértebras cervicales e implantación baja del cabello en la región occipital. Se presenta por falla de segmentación en el esqueleto axial del embrión. Su incidencia se estima en 1/40 000-42 000nacimientos y predomina en el sexo femenino. El objetivo del presente trabajo es describir el cuadro clínico de un paciente con síndrome de Klippel-Feil y múltiples malformaciones asociadas, entre ellas, fístula traqueoesofágica, pulgar bífido y lipomas/angiolipomas intracraneales, las cuales, hasta ahora, no han sido descritas en el síndrome, por lo que se considera un hallazgo excepcional.

The Klippel-Feil syndrome is a congenital malformation of the skull flap involving complex cervical vertebrae and organs, characterized by a classic triad: short neck, limitation of movement of the head due to cervical spine fusion and low hairline in occipital region. It results from an error in the axial skeleton segmentation of the embryo; its incidence is estimated at 1/40,000-42,000 births and predominates in females. The aim of this paper is to describe the clinical picture of a patient with Klippel-Feil syndrome and multiple malformations, including tracheoesophageal fistula, bifid thumb and intracranial lipomas/angiolipomas,that have not been previously described in the syndrome, so it is considered an exceptional finding.