Case Report

Anaesthetic Management in a Child with Cutis Laxa for Bilateral Ureteric Reimplantation


Cutis laxa is a rare congenital multisystem connective tissue disorder. Patients with cutis laxa have facial features, pulmonary emphysema and right-sided heart failure. Anaesthetic management is important because of difficult airway and respiratory/cardiac abnormalities. We have reported the preoperative evaluation and anaesthetic management of a child with Cutis laxa with right cleft lip and palate.

[1] Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller A, Leffell DJ. Heritable disorders of connective tissue with skin changes. In: Fitzpatrick’s dermatology in general medicine. 7th. ed. New York: Mc Graw Hill; 2008:1308-10
[2] Guia Torrent JM, Castro Gracia F, Cuenca Gornez M et al. Cardiovascular changes in the cutis laxa congenital syndrome. Rev Esp Cardiol. 1999; 52(3): 204–6.
[3] Andiran N, Sarikayalar F, Saraçlar M, Çaglar M. Autossomal recessive form of congenital cutis laxa: more than the clinical appearance. Pediatr Dermatol. 2002; 19(5):412-4.
[4] Maleki A, Ebrahim Soltani A, Goudarzi M, Yaghooti AA, Ostad Alipour A, Espahbodi E. Anesthesia in Rare Case: Cutis Laxa Syndrome. Arch Anesth & Crit Care. 2015; 1(4):139-40.
[5] Pandey R, Garg R, Manikandan R, Punj J, Darlong V, Singh SA. Perianesthetic management of generalized congenital cutis laxa syndrome associated with pulmonary stenosis undergoing inguinal hernia repair. Paediatr Anaesth. 2008; 18(9):907-9.
[6] Hajjar WM, Alrajeh AS, Alturki LS, Al-Nassar SA, Hajjar AW. Near-fatal presentation of bilateral pneumothorax in cutis laxa patient: Case report, and review of the literature. Ann Thorac Med [serial online] 2018; 13(4):254-6
[7] Hugh M Smith, Lindsay Warner Anaesthetic recommendations for patients suffering from De Barsy syndrome. Orphan anasethesia. Available at ome_US_en_ANS_ORPHA2962.pdf.
[8] Warner LL, Olsen DA, Smith HM. Clinical implications of de Barsy syndrome. Paediatr Anaesth. 2018; 28(1):59-62.
[9] Davies SJ, Huges HE. Costello syndrome: natural history and differential diagnosis of cutis laxa. J Med Genet. 1994; 31:486-9.
[10] Aponte EP, Smith HM, Wanek BJ, Rettke SR. Anesthesia considerations for patients with de Barsy syndrome. J Clin Anesth. 2010; 22(7):499-504.
[11] Dearlove O, Harper N. Costello syndrome. Paediatr Anaesth. 1997; 7:476-477.
[12] Furderer S, Stanek A, Karbowski A and Eckardt A. Intraoperative hyperpyrexia in patients with osteogenesis imperfecta. Z Orthop Ihre Grenzgeb. 2000; 138:136-139.
IssueVol 7 No 3 (2021): Summer QRcode
SectionCase Report(s)
Paediatric anaesthesia cutis laxa difficult airway

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
Ramachandran H, Bhagya D. Anaesthetic Management in a Child with Cutis Laxa for Bilateral Ureteric Reimplantation. Arch Anesth & Crit Care. 2021;7(3):176-179.