Congenital Right Lung Agenesis With Dysplastic Sternum - A Constellation Of Two Rare Cases

Congenital lung agenesis is a rare disease spectrum which includes complete agenesis, lung aplasia (with rudimentary bronchus)and partial agenesis. It is embryological anomaly arising from failure of unilateral lung bud to develop. Sternal dysplasias are veryrare entities with only few reported cases[1] . We present case report of a 1 year old female with complete right lung agenesisassociated with dysplastic sternum and vertebral segmentation anomaly

Asociación LACHT con hipoplasia de la vía aérea superior. Caso clínico / LACHT association with hypoplasia of the upper airway. Clinical case

Resumen Introducción: La asociación Mardini-Nyhan o LACHT es una condición clínica de baja prevalência que cursa con alteraciones pulmonares, cardíacas y de las extremidades. Su etiología no está clara mente documentada hasta la fecha. Objetivo: Describir el caso de una niña de 4 meses y realizar una revisión de la literatura de los casos reportados sobre esta asociación, con el propósito de exponer las alteraciones encontradas y de esta forma orientar el diagnóstico temprano de esta entidad. Caso clínico: Niña de 4 meses de edad que ingresa a cuidado intensivo en insuficiencia respiratoria mixta, con alteraciones pulmonares, cardíacas y de extremidades que cumplen criterios para la asociación LACTH, se documenta adicionalmente hipoplasia de la vía aérea superior, que empeora la evolución, aumenta la dificultad en la ventilación mecánica y favorece el desenlace fatal a los 7 días de hospitali zación. Conclusiones: La asociación LACTH es una patología poco prevalente en la cual los hallazgos clínicos permiten sospechar el diagnóstico. Es caso clínico sería el primer caso diagnosticado en el continente americano y el caso número 11 descrito en la literatura mundial, aportando como nuevo hallazgo la asociación con hipoplasia de la vía aérea superior.

Abstract Introduction: The Mardini-Nyhan or LACHT association is a clinical condition of low prevalence that presents with pulmonary, cardiac and limb abnormalities, in which genetic etiology is not clearly documented to date. Objective: To describe the case of a 4-month-old child and the literature review of cases reported on this association, with the purpose of exposing the alterations found and thus gui de the early diagnosis of this entity. Clinical case: 4 months old girl, who admitted to intensive care in mixed respiratory failure, with pulmonary, cardiac and limb disorders that meet criteria for LACHT association, additionally documents hypoplasia of the upper airway, which worsens the evolution, increases the difficulty in mechanical ventilation and favors the fatal outcome at 7 days of hospita lization. Conclusions: LACTH association is a rare pathology in which the clinical findings make it possible to suspect the diagnosis, this is the first case diagnosed in the Americas and the number 11 case in the world literature and brings as a new finding the association with hypoplasia of the upper airway.

Congenital lung abnormalities / 中华实用儿科临床杂志

Congenital lung anomalies (CLA) are a group of malformations that include bronchopulmonary anomalies,vascular anomalies,and combined lung and vascular anomalies.With the advances in fetal ultrasonography and magnetic resonance imaging,and multi-detector computer tomography,CLA are increasingly being detected during both antenatal and postnatal periods,in turn influence patient counselling and management stratification.This paper focus on congenital lung abnormalities and congenital lobar emphysema.

Diaphragm Translocation as Surgical Treatment for Agenesis of the Right Lung and Secondary Tracheal Compression

A 12-month-old boy was diagnosed with agenesis of the right lung. Mediastinal deviation progressed to the diseased side as the patient matured; therefore, tracheal distortion developed. As a result, tracheal compression developed between the vertebral body and aorta. The patient was repeatedly admitted to the hospital because of recurrent pulmonary infection and combined severe respiratory distress. Diaphragm translocation was performed to treat the patient. The postoperative course was favorable, and computed tomography scan findings and symptoms had improved at 1 year after surgery.

A rare case of left lung agenesis in a female teenager.

Pulmonary agenesis is a rare congenital anomaly. History of recurrent chest infection in first year of life is the presenting symptom although patient may be asymptomatic and detected on routine chest x-ray. Our patient presented with recurrent chest infections since childhood. CT scan thorax revealed absence of lung tissue on left side with mediastinal shifting and herniation of right lung to left side. Pulmonary angiography confirmed the diagnosis of left lung agenesis.

A case of agenesis of the right lung

No abstract available.

A case of right lung agenesis

No abstract available.