Perioperative Anesthesia Management in a Patient with Ehlers-Danlos Syndrome: A Case Report of Scleral Buckling Surgery
Abstract
This case report describes the anesthetic management of a 27-year-old man with hypermobile Ehlers-Danlos syndrome (hEDS) who underwent scleral buckling surgery for retinal detachment. The patient who had joint hypermobility and mild mitral valve prolapse required careful anesthetic management. The surgery, which was performed under general anesthesia with a laryngeal mask airway and EDS-tailored monitoring, was uneventfully accomplished in 85 minutes. The patient was discharged the next day, recovering well, emphasizing the importance of careful preoperative evaluation, correct positioning, and multidisciplinary care for patients with hEDS undergoing complex ophthalmic surgery.
[1] Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, et al. The 2017 international classification of the Ehlers–Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175(1):8-26.
[2] Laserna A, Nishtar M, Vidovich C, Borovcanin Z. Perioperative Management of Ehlers-Danlos Type III Syndrome Associated With Postural Orthostatic Tachycardia in Patients Undergoing General Anesthesia. Cureus. 2021; 13(11):e19311.
[3] Castori M. Surgical recommendations in Ehlers-Danlos syndrome(s) need patient classification: the example of Ehlers-Danlos syndrome hypermobility type (a.k.a. joint hypermobility syndrome). Dig Surg. 2012;29(6):453-5.
[4] Types of EDS - The Ehlers-Danlos Support UK [Internet]. [cited 2025 May 10]. Available from: https://www.ehlers-danlos.org/what-is-eds/information-on-eds/types-of-eds/
[5] Ehlers Danlos Anesthesia Considerations and Risks | The EDS Clinic [Internet]. [cited 2025 May 16]. Available from: https://www.eds.clinic/articles/anesthesia-risks-for-ehlers-danlos-syndrome-patients
[6] Ehlers Danlos syndrome - NYSORA [Internet]. [cited 2025 May 10]. Available from: https://www.nysora.com/anesthesia/ehlers-danlos-syndrome/
[7] Asanad S, Bayomi M, Brown D, Buzzard J, Lai E, Ling C, et al. Ehlers-Danlos syndromes and their manifestations in the visual system. Front Med (Lausanne). 2022; 9:996458.
[8] Ehlers-Danlos syndrome: MedlinePlus Genetics [Internet]. [cited 2025 May 10]. Available from: https://medlineplus.gov/genetics/condition/ehlers-danlos-syndrome/
[9] Im JS, Lim YH, Park JS, Lee SS, Kim KM. Rupture of abdominal aortic aneurysm after spine surgery in the patient with Ehlers-Danlos syndrome - A case report. Korean J Anesthesiol. 2010; 58(6):555-9.
[10] Lane D. Anaesthetic implications of vascular type Ehlers-Danlos syndrome. Anaesth Intensive Care. 2006; 34(4):501-5.
[11] Ohshita N, Kanazumi M, Tsuji K, Yoshida H, Morita S, Momota Y, et al. Anesthetic Management of a Patient With Ehlers-Danlos Syndrome. Anesth Prog. 2016; 63(4):204-207.
[12] Wiesmann T, Castori M, Malfait F, Wulf H. Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet J Rare Dis. 2014; 9:109.
[13] Bascom R, Dhingra R, Francomano CA. Respiratory manifestations in the Ehlers–Danlos syndromes. Am J Med Genet C Semin Med Genet. 2021;187(4):533–48.
[14] Schubart JR, Schaefer E, Janicki P, Adhikary SD, Schilling A, Hakim AJ, et al. Resistance to local anesthesia in people with the Ehlers-Danlos Syndromes presenting for dental surgery. J Dent Anesth Pain Med. 2019;19(5):261-270.
[15] Ohashi N, Furutani K, Ishii H, Baba H. [Perioperative brachial plexus injury caused by hyperabduction of the upper extremity in a patient with Ehlers-Danlos syndrome in the prone position]. Masui. 2012; 61(6):626–8.
[16] Athari M, Tajbakhsh A, Mirbolook A, Beheshtian M, Forghan M, Abtahi D. Management of scoliosis in a pediatric patient with lysyl hydroxylase-3 deficiency: a case report J Med Case Rep. 2024; 18(1):474.
[17] Glard Y, Launay F, Edgard-Rosa G, Collignon P, Jouve JL, Bollini G. Scoliotic curve patterns in patients with Marfan syndrome. J Child Orthop. 2008; 2(3):211-6.
[18] Kroese LF, Mommers EH, Robbens C, Bouvy ND, Lange JF, Berrevoet F. Complications and recurrence rates of patients with Ehlers-Danlos syndrome undergoing ventral hernioplasty: a case series. Hernia. 2018; 22(4):611-616.
[19] Wakabayashi R, Tanaka S, Tsuchiyama K, Yamamoto K, Maruyama Y, Numata K, et al. Anesthetic management of a patient with musculocontractural Ehlers-Danlos syndrome undergoing scoliosis surgery. JA Clin Rep. 2020; 6(1):46.
[2] Laserna A, Nishtar M, Vidovich C, Borovcanin Z. Perioperative Management of Ehlers-Danlos Type III Syndrome Associated With Postural Orthostatic Tachycardia in Patients Undergoing General Anesthesia. Cureus. 2021; 13(11):e19311.
[3] Castori M. Surgical recommendations in Ehlers-Danlos syndrome(s) need patient classification: the example of Ehlers-Danlos syndrome hypermobility type (a.k.a. joint hypermobility syndrome). Dig Surg. 2012;29(6):453-5.
[4] Types of EDS - The Ehlers-Danlos Support UK [Internet]. [cited 2025 May 10]. Available from: https://www.ehlers-danlos.org/what-is-eds/information-on-eds/types-of-eds/
[5] Ehlers Danlos Anesthesia Considerations and Risks | The EDS Clinic [Internet]. [cited 2025 May 16]. Available from: https://www.eds.clinic/articles/anesthesia-risks-for-ehlers-danlos-syndrome-patients
[6] Ehlers Danlos syndrome - NYSORA [Internet]. [cited 2025 May 10]. Available from: https://www.nysora.com/anesthesia/ehlers-danlos-syndrome/
[7] Asanad S, Bayomi M, Brown D, Buzzard J, Lai E, Ling C, et al. Ehlers-Danlos syndromes and their manifestations in the visual system. Front Med (Lausanne). 2022; 9:996458.
[8] Ehlers-Danlos syndrome: MedlinePlus Genetics [Internet]. [cited 2025 May 10]. Available from: https://medlineplus.gov/genetics/condition/ehlers-danlos-syndrome/
[9] Im JS, Lim YH, Park JS, Lee SS, Kim KM. Rupture of abdominal aortic aneurysm after spine surgery in the patient with Ehlers-Danlos syndrome - A case report. Korean J Anesthesiol. 2010; 58(6):555-9.
[10] Lane D. Anaesthetic implications of vascular type Ehlers-Danlos syndrome. Anaesth Intensive Care. 2006; 34(4):501-5.
[11] Ohshita N, Kanazumi M, Tsuji K, Yoshida H, Morita S, Momota Y, et al. Anesthetic Management of a Patient With Ehlers-Danlos Syndrome. Anesth Prog. 2016; 63(4):204-207.
[12] Wiesmann T, Castori M, Malfait F, Wulf H. Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet J Rare Dis. 2014; 9:109.
[13] Bascom R, Dhingra R, Francomano CA. Respiratory manifestations in the Ehlers–Danlos syndromes. Am J Med Genet C Semin Med Genet. 2021;187(4):533–48.
[14] Schubart JR, Schaefer E, Janicki P, Adhikary SD, Schilling A, Hakim AJ, et al. Resistance to local anesthesia in people with the Ehlers-Danlos Syndromes presenting for dental surgery. J Dent Anesth Pain Med. 2019;19(5):261-270.
[15] Ohashi N, Furutani K, Ishii H, Baba H. [Perioperative brachial plexus injury caused by hyperabduction of the upper extremity in a patient with Ehlers-Danlos syndrome in the prone position]. Masui. 2012; 61(6):626–8.
[16] Athari M, Tajbakhsh A, Mirbolook A, Beheshtian M, Forghan M, Abtahi D. Management of scoliosis in a pediatric patient with lysyl hydroxylase-3 deficiency: a case report J Med Case Rep. 2024; 18(1):474.
[17] Glard Y, Launay F, Edgard-Rosa G, Collignon P, Jouve JL, Bollini G. Scoliotic curve patterns in patients with Marfan syndrome. J Child Orthop. 2008; 2(3):211-6.
[18] Kroese LF, Mommers EH, Robbens C, Bouvy ND, Lange JF, Berrevoet F. Complications and recurrence rates of patients with Ehlers-Danlos syndrome undergoing ventral hernioplasty: a case series. Hernia. 2018; 22(4):611-616.
[19] Wakabayashi R, Tanaka S, Tsuchiyama K, Yamamoto K, Maruyama Y, Numata K, et al. Anesthetic management of a patient with musculocontractural Ehlers-Danlos syndrome undergoing scoliosis surgery. JA Clin Rep. 2020; 6(1):46.
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| Section | Case Report(s) | |
| Keywords | ||
| Anesthesia Ehlers-Danlos Syndrome Scleral Buckling. | ||
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How to Cite
1.
Daneshvar H, Abtahi D. Perioperative Anesthesia Management in a Patient with Ehlers-Danlos Syndrome: A Case Report of Scleral Buckling Surgery. Arch Anesth & Crit Care. 2025;.
