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Congenital lobar emphysema: a review of 10 cases
B Bappal1, S A Ghani, R Chaudhary
1Department of Child Health and of Pediatric Surgery, Royal Hospital, Sultanate of Oman.
Insights
Congenital lobar emphysema (CLE) diagnosis is often delayed, despite clear radiographic signs. Early surgical intervention or close monitoring is crucial for managing this rare lung condition in infants.
Area of Science:
- Pediatric Pulmonology
- Thoracic Surgery
- Medical Imaging
Background:
- Congenital lobar emphysema (CLE) is a rare congenital lung malformation.
- Early diagnosis and intervention are critical for favorable outcomes in infants with CLE.
Observation:
- A review of 10 patients with CLE treated between 1988 and 1995 revealed symptom onset from birth to six months.
- Radiographic diagnosis of CLE was possible in all cases, yet only 40% were diagnosed pre-referral.
- Seven patients underwent lobectomy, one had a vascular ring resected, and three were managed conservatively initially.
Findings:
- Delayed diagnosis of CLE is common, potentially hindering timely treatment.
- Surgical intervention (lobectomy) is the primary treatment for symptomatic CLE.
- Conservative management can be an option for select cases, but may require delayed surgery if complications arise.
Implications:
- Improved awareness and interpretation of chest radiographs can expedite CLE diagnosis.
- Optimizing the timing of surgical intervention is key to managing CLE.
- Long-term outcomes for conservatively managed CLE patients warrant further investigation.
Abstract:
We reviewed the clinical features and management of 10 patients with congenital lobar emphysema (CLE) treated in our institution over a period of seven years, from September 1988 to September 1995. The age of onset of symptoms ranged from few days from birth to six months. Correct diagnosis was made in only 4 patients before referral, even though diagnosis could have been arrived at from the chest radiograph in all of them. Lobectomy was done in seven patients, while one patient had vascular ring resected. Of the three patients managed conservatively, in one lobectomy was performed at the end of 5 months conservative course as her clinical condition deteriorated during an intercurrent chest infection. Of the remaining two, one has mild episodic reactive air way disease and the other patient is asymptomatic.