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Congenital lobar emphysema — Clinical Guidelines

Overview

Congenital lobar emphysema (CLE) is a rare congenital disorder characterized by overdistension of one or more lung lobes due to airway obstruction, often presenting in infancy with respiratory distress 1 6 10.

Diagnosis

Key Diagnostic Criteria: Hyperinflation and hyperlucency on chest radiography, typically affecting a single lobe 1 17 18.

Recommended Tests:

- Chest radiography and CT scans for precise lobe identification and severity assessment 17 18. - Bronchoscopy to evaluate airway patency and identify potential obstructions 9 12. - Lung scintigraphy using solubilized xenon 133 for ventilation assessment 14.

Management

First-Line Treatment:

- Surgical lobectomy is the definitive treatment, often performed urgently to relieve respiratory compromise 1 3 15 18. - Sequential lobectomies may be necessary for bilateral cases 3.

Adjunctive Measures:

- Thoracic epidural analgesia for safe anesthesia induction and postoperative pain management 7. - Use of surgical staplers for precise resection, particularly in pediatric patients 13.

Special Populations

Pregnancy: Surgical intervention, including lobectomy, can be safely performed during pregnancy without adverse maternal or fetal outcomes 11.

Pediatrics: Early surgical intervention is crucial; bronchoscopy should precede surgery to rule out treatable intra-luminal abnormalities 12.

Comorbidities: CLE often coexists with congenital heart disease (CHD); aggressive lobectomy may precede CHD repair 1.

Key Recommendations

Surgical Lobectomy is Indicated for Symptomatic Patients: Early surgical lobectomy is recommended for symptomatic congenital lobar emphysema to prevent respiratory failure 1 3 15 (Evidence: Strong).

Bronchoscopy Should Precede Surgery: Perform bronchoscopy to exclude treatable airway abnormalities before surgical intervention 12 (Evidence: Moderate).

Consider Sequential Lobectomies for Bilateral Cases: In cases of bilateral congenital lobar emphysema, staged lobectomies may be necessary 3 (Evidence: Moderate).

Thoracic Epidural Analgesia for Anesthesia: Utilize thoracic epidural analgesia to manage anesthesia induction and postoperative pain in pediatric patients 7 (Evidence: Weak).

Surgical Intervention Feasible During Pregnancy: Surgical treatment, including lobectomy, can be safely performed during pregnancy 11 (Evidence: Moderate).

References

1 Kubo S, Tanaka T, Oshima Y. Surgical management of congenital lobar emphysema associated with CHD. Cardiology in the young 2021. link 2 Blonk M, Van de Maele I, Combes A, Stablay B, De Cock H, Polis I et al.. Congenital lobar emphysema in a kitten. The Journal of small animal practice 2017. link 3 Maiya S, Clarke JR, More B, Desai M, Parikh D. Bilateral congenital lobar emphysema: how should we proceed?. Pediatric surgery international 2005. link 4 Matsumoto H, Kakehata T, Hyodo T, Hanada K, Tsuji Y, Hoshino S et al.. Surgical correction of congenital lobar emphysema in a dog. The Journal of veterinary medical science 2004. link 5 Billet JP, Sharpe A. Surgical treatment of congenital lobar emphysema in a puppy. The Journal of small animal practice 2002. link 6 Roberts PA, Holland AJ, Halliday RJ, Arbuckle SM, Cass DT. Congenital lobar emphysema: Like father, like son. Journal of pediatric surgery 2002. link 7 Raghavendran S, Diwan R, Shah T, Vas L. Continuous caudal epidural analgesia for congenital lobar emphysema: a report of three cases. Anesthesia and analgesia 2001. link 8 Thompson AJ, Reid AJ, Reid M. Congenital lobar emphysema occurring in twins. Journal of perinatal medicine 2000. link 9 Doull IJ, Connett GJ, Warner JO. Bronchoscopic appearances of congenital lobar emphysema. Pediatric pulmonology 1996. link1099-0496(199603)21:3<195::AID-PPUL9>3.0.CO;2-P) 10 Carrol ED, Campbell ME, Shaw BN, Pilling DW. Congenital lobar emphysema in congenital cytomegalovirus infection. Pediatric radiology 1996. link 11 Critchley PS, Forrester-Wood CP, Ridley PD. Adult congenital lobar emphysema in pregnancy. Thorax 1995. link 12 Saim L, Mohamad AS, Ambu VK. Congenital lobar emphysema: a case with bronchial septum. International journal of pediatric otorhinolaryngology 1994. link90018-3) 13 Mattioli G, Cagnazzo A, Martucciello G, Jasouni V. Use of staplers in congenital lobar emphysema. European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie 1993. link 14 Oates E, Sarno RC. Solubilized xenon 133 lung scintigraphy. Journal of pediatric surgery 1988. link80005-8) 15 Ekkelkamp S, Vos A. Successful surgical treatment of a newborn with bilateral congenital lobar emphysema. Journal of pediatric surgery 1987. link80495-5) 16 Martinez-Frontanilla LA, Hernandez J, Haase GM, Burrington JD. Surgery of acquired lobar emphysema in the neonate. Journal of pediatric surgery 1984. link80256-0) 17 Pardes JG, Auh YH, Blomquist K, Kazam E, Magid M. CT diagnosis of congenital lobar emphysema. Journal of computer assisted tomography 1983. link 18 Adeyemo AO, Omole CO, Oyedeji GA. Congenital lobar emphysema of left upper lobe. A case report. Scandinavian journal of thoracic and cardiovascular surgery 1983. link 19 Davis C, Freant LJ, Henderson KC. Congenital lobar emphysema. Southern medical journal 1979. link 20 Powell HC, Elliott ML. Congenital lobar emphysema. Virchows Archiv. A, Pathological anatomy and histology 1977. link 21 Canty TG. Congenital lobar emphysema resulting from bronchial sling around a normal right main pulmonary artery. The Journal of thoracic and cardiovascular surgery 1977. link 22 Urban AE, Stark J, Waterston DJ. Congenital lobar emphysema. Thoraxchirurgie, vaskulare Chirurgie 1975. link

Congenital lobar emphysema