Anaesthetic Management of a Patient with Marfan Syndrome Undergoing Elective Ventral Hernia Repair

Sep 30, 2026

Uploaded by Rowan

Marfan syndrome, an autosomal dominant connective-tissue disorder, involves multiple organ systems, with cardiovascular complications driving major perioperative risk. With increasing longevity and evolving care, anaesthetic management requires careful adaptation to changing clinical profiles. This case report details perioperative anaesthetic planning for elective open ventral hernia repair in a 37-year-old male, emphasizing positioning, haemodynamic monitoring, airway difficulty preparation, titrated drug dosing, ventilation adjustment, and postoperative management of complications.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages10

Reading Time25 min

AccessFree to download

Abstract

1. Introduction

2. Case Report

Case Report Anaesthetic Management of a Patient with Marfan Syndrome Undergoing Elective Ventral Hernia Repair Aurelijus Pkas *, Deimant Stankut and Jrat Gudaityt Department of Anesthesiology, Lithuanian University of Health Sciences, 44307 Kaunas, Lithuania; [deimante.stankute2@stud.lsmu.lt](deimante.stankute2@stud.lsmu.lt) (D.S.); [jurate.gudaityte@lsmu.lt](jurate.gudaityte@lsmu.lt) (J.G.) * Correspondence: [aurelijus.pukas@stud.lsmu.lt](aurelijus.pukas@stud.lsmu.lt) Abstract Background: Marfan syndrome is an autosomal dominant connective tissue disorder that affects multiple organ systems, with cardiovascular complications posing a major risk. With advancements in medical care and the increasing lifespan of patients with Marfan syndrome, the spectrum of medical issues has evolved. This case report highlights the complex anaesthetic management of a patient with Marfan syndrome during elective ventral hernia repair. Case presentation: A 37-year-old male with Marfan syndrome was admitted for elective open ventral hernia repair. Challenges included severe arterial hypertension, prior aortic valve replacement, scoliosis, and an anticipated difficult airway, as the patient presented with restricted mouth opening due to craniofacial abnormalities consistent with difficult laryngoscopy. Preoperative assessments included routine tests, echocardiography and chest X-ray. The anaesthetic management focused on specific patient positioning with head-up tilt, maintenance of haemodynamic stability with the insertion of an arterial line before the induction of anaesthesia and neuromuscular block (NMB) monitoring, followed by titrated doses of all medications. Lung ventilation strategies were specifically adjusted to address the patient’s underlying comorbidities. The patient was extubated and transferred to the recovery unit. The intraoperative and immediate postoperative periods were relatively uneventful. Dyspnea due to external pressure on the abdominal wall caused by a specific binder was treated with the release of pressure. Later postoperative recovery was complicated by hydrothorax and pneumonia, both treated successfully. Conclusions: This case emphasises the importance of multidisciplinary approaches and vigilant monitoring in the management of a patient with Marfan syndrome perioperatively, even for seemingly low-risk operations. Appropriate anaesthetic management helped to avoid major perioperative complications. Keywords: anaesthesia; Marfan syndrome; hernioplasty Academic Editor: Andre van Zundert Received: 7 November 2025 Revised: 11 December 2025 Accepted: 18 December 2025 Published: 23 December 2025 Copyright: © 2025 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license. 1. Introduction Marfan Syndrome is an autosomal dominant disorder caused by a mutation in the FBN1 (fibrillin-1) gene on chromosome 15, which encodes the fibrillin protein [1] . This condition affects approximately 1 in 5000 individuals [2] . The FBN1 gene defect affects multiple organs and systems, most commonly presenting with musculoskeletal, cardiovascular, and ophthalmologic disorders [1] . The most life-threatening complications are cardiovascular, including mitral valve prolapse, aortic insufficiency, aortic root dilation, and aortic dissection [3] . With advancements in medical care and the increasing lifespan of patients with Marfan syndrome, the spectrum of medical issues has evolved. We present a clinical case to highlight the challenges and perioperative course in a patient with atypical Marfan syndrome, focusing on the specific considerations in anaesthesia management. 2. Case Report The patient provided written informed consent for the preparation and publication of this case report, including the use of anonymized clinical data and imaging findings. A 37-year-old male was admit

FAQs

What major perioperative risks in Marfan syndrome motivated the anaesthetic approach in this case?

Cardiovascular complications were central, alongside anticipated airway difficulty from craniofacial abnormalities and coexisting conditions such as severe arterial hypertension and prior aortic valve replacement.

How was the difficult airway risk anticipated and addressed preoperatively?

The patient’s restricted mouth opening and nasopharyngeal anatomy supported anticipation of a difficult laryngoscopy, and preoperative assessment guided the perioperative airway strategy.

What intraoperative and immediate postoperative measures were used to maintain safety?

The anaesthetic plan emphasized head-up positioning, arterial line insertion before induction with neuromuscular block monitoring, titrated dosing of medications, and ventilation adjustments tailored to comorbidities.

Sep 30, 2026

Uploaded by Rowan