ABSTRACT

Introduction: Osteogenesis imperfecta is a group of heterogenous abnormalities of the collagen synthesis, characterized by multiple fractures and deformities. Thoracic surgery in patients with osteogenesis imperfecta is not common. Clinical case: We present a case of a 30 years old male with a previous diagnosis of osteogenesis imperfecta who was referred to our center for presenting a loculated empyema on his right hemithorax. A right posterolateral thoracotomy was performed. In the middle of the surgery we visualized an anatomical abnormality that coincides with an accessory fissure of the superior lobe. Conclusion: Osteogenesis imperfecta is a rare clinical entity, however the surgeon must take into consideration the physiopathological implications during the surgery.

KEYWORDS

Osteogenesis imperfecta, accessory fissure, empyema.

REFERENCES

1. Forlino A, Marini JC. Osteogenesis imperfecta. Lancet 2016;387(10028):1657-1671. https://doi.org/10.1016/s0140-6736(15)00728-x.

2. Tournis S, Dede AD. Osteogenesis imperfect. A clinical update. Metabolism 2018;80:27-37. https://doi.org/10.1016/j.metabol.2017.06.001.

3. Marini J, Cabral W. Osteogenesis imperfecta. In: Thakker RV, Whyte MP, Eisman JA, Igarashi T, editors. Genetics of bone biology and skeletal disease. 2nd. ed. London Wall: Academic Press, Elsevier; 2018. p. 397-414. doi:10.1016/b978-0-12-804182-6.00023-x.

4. Renaud A, Aucourt J, Weill J, et al. Radiographic features of osteogenesis imperfecta. Insights Imaging 2013;4(4):417-429. https://doi.org/10.1007/s13244-013-0258-4.

5. Morello R. Osteogenesis imperfecta and therapeutics. Matrix Biol 2018;71-72:294-312. https://doi.org/10.1016/j.matbio.2018.03.010-

6. Van Dijk FS, Sillence DO. Osteogenesis imperfecta: clinical diagnosis, nomenclature and severity assessment. Am J Med Genet A 2014;164A(6):1470-1481. https://doi.org/10.1002/ajmg.a.36545.

7. Lafage-Proust MH, Courtois I. The management of osteogenesis imperfecta in adults: State of the art. Joint Bone Spine 2019;86(5):589-593. https://doi.org/10.1016/j.jbspin.2019.02.001.

8. Stynowick GA, Tobias JD. Perioperative care of the patient with osteogenesis imperfecta. Orthopedics 2007;30(12):1043-1049. https://doi.org/10.3928/01477447-20071201-12.

9. Erdoğan MA, Sanli M, Ersoy MO. Anesthesia management in a child with osteogenesis imperfecta and epidural hemorrhage. Braz J Anesthesiol 2013;63(4):366-368. https://doi.org/10.1016/j.bjan.2012.07.007.

10. Keegan MT, Whatcott BD, Harrison BA. Osteogenesis imperfecta, perioperative bleeding, and desmopressin. Anesthesiology 2002;97(4):1011-1013. doi: 10.1097/00000542-200210000-00039.

11. Libman RH. Anesthetic considerations for the patient with osteogenesis imperfecta. Clin Orthop Relat Res 1981;(159):123-125.

12. LoMauro A, Pochintesta S, Romei M, et al. Rib cage deformities alter respiratory muscle action and chest wall function in patients with severe osteogenesis imperfecta. PLoS One 2012;7(4):e35965. https://doi.org/10.1371/journal.pone.0035965.

13. Muttikkal T, Deng C. Non-azygos accessory fissure in right upper lobe associated with superior and inferior accessory fissures in right lower Lobe. J Clin Imaging Sci 2012;2:79. https://doi.org/10.4103/2156-7514.105133.