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Congenital Cystic Diseases of the Lung in Infant and Children: Our Experience with 64 Cases - Abstract

Ahmed H. Al-Salem, Hilal Matta

 

Abstract

Background: Congenital cystic diseases of the lung comprise a group of rare but closely related conditions with variable clinical features. This is a review of our experience with 64 cases outlining aspects of clinical features, diagnosis and management.

Patients and methods: The medical records of all infants and children with the diagnosis of congenital cystic diseases of the lung were retrospectively reviewed for age at diagnosis, sex, clinical features, diagnosis, treatment and outcome. The histology was obtained from the histopathology report.

Results: A total of 64 (39 males and 25 females) infants and children with congenital cystic diseases of the lung were treated. Their age at presentation ranged from 1 day to 15 years (mean 2.1 years). There were 23 cases of congenital lobar emphysema, 3 cases of pulmonary sequestration, 20 bronchogenic cysts and 18 cases of congenital cystic adenomatoid malformation. Twenty three (14 males and 9 females) patients had congenital lobar emphysema. Their age at presentation ranged from 1 day to 8 months (mean 3.1 months). The majority presented with variable degrees of respiratory distress symptoms and some of them were diagnosed because of repeated attacks of chest infection. Nine presented immediately after birth with respiratory distress symptoms, two of them had severe symptoms necessitating surgery within the first week after their delivery. In 19 patients the left upper lobe was affected and in the remaining 4, the right upper lobe was affected. Three patients (2 males: 1 female) had pulmonary sequestration. Two had extralobar pulmonary sequestration and the third had intralobar pulmonary sequestration. Twenty patients had bronchogenic cysts. There were 12 males and 8 females. Their age at diagnosis ranged from 2 months to 15 years (mean 4.2 years). Their clinical presentation was variable ranging from mild respiratory symptoms (2 patients), recurrent chest infection (11 patients) and respiratory distress (2 patients). In 5 patients, the cysts were discovered incidentally on routine chest X-ray. Two patients were born with large bronchogenic cysts and initially had mild respiratory distress but within two months, their symptoms increased necessitating thoracotomy and lobectomy. In 12 patients, the bronchogenic cysts were intralobar (right upper lobe 3, right lower lobe 4, right middle lobe 2, and left upper lobe 3). In 8, bronchogenic cysts were extralobar (right posterior mediastinum 4 and para-tracheal in 4). Eighteen patients had congenital cystic adenomatoid malformation. There were 11 males and 7 females. Their age at diagnosis ranged from 1 week to 12 years (mean 3.2 years). The presentation of these patients was also variable. The majority (14 patients) presented with repeated attacks of chest infection. In two patients, the diagnosis was confused with left congenital diaphragmatic hernia.

Conclusions: Congenital cystic diseases of the lung comprise a unique group of malformations with variable clinical presentation ranging from asymptomatic to those who present early with respiratory distress. Their common presentation is repeated chest infections. Awareness of this is important to obviate associated morbidity and mortality. Asymptomatic or mildly symptomatic congenital lobar emphysema can be treated conservatively, but the majority of patients with congenital cystic diseases of the lung require surgical excision.

Keywords: congenital cystic diseases of the lung, congenital cystic adenomatoid malformation, bronchogenic cyst, broncho-pulmonary sequestration, congenital lobar emphysema

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