Work

Anesthetic management of an infant with arterial tortuosity syndrome undergoing combined laparoscopic procedures: a case report

Year: 2026

Type: article

Abstract: Background Arterial tortuosity syndrome (ATS) is a rare autosomal recessive connective-tissue disorder marked by elongation and tortuosity of major arteries, predisposing affected patients to aneurysm formation, stenosis, and arterial dissection. These vascular abnormalities, together with characteristic craniofacial and connective-tissue features, pose significant anesthetic challenges, particularly in airway management, hemodynamic control, and perioperative cardiovascular risk. The complexity is further heightened in infants undergoing prolonged laparoscopic procedures, in which pneumoperitoneum, reverse-Trendelenburg positioning, and the limited physiological reserve of early infancy may adversely affect ventilation, venous return, and cardiovascular stability. Case presentation We describe the anesthetic management of a 3-month-old, 4.6-kg infant with genetically confirmed ATS, who underwent combined laparoscopic repair of a Morgagni diaphragmatic hernia and a para-esophageal hiatal hernia. Preoperative evaluation demonstrated tortuosity of the aorta, head and neck vessels, and pulmonary arteries, without pulmonary hypertension or ventricular dysfunction. Inhalational induction with sevoflurane was performed while preserving spontaneous ventilation because of the anticipated difficult airway and the presence of a Morgagni diaphragmatic hernia, where positive-pressure ventilation could result in gastric insufflation and further distension of herniated abdominal contents. Tracheal intubation was successfully achieved with a 3.0-mm cuffed endotracheal tube. Anesthesia was maintained with sevoflurane and opioid supplementation. Carbon dioxide pneumoperitoneum was introduced gradually, with intra-abdominal pressure maintained below 10 mmHg to limit hemodynamic and ventilatory disturbances. The intraoperative course remained stable, and the patient was extubated uneventfully before transfer to the pediatric intensive care unit. Conclusion This case underscores essential anesthetic considerations for infants with ATS undergoing prolonged laparoscopic procedures, emphasizing anticipatory airway planning, vascular-protective strategies, controlled pneumoperitoneum, vigilant cardiovascular management, and coordinated multidisciplinary care.

Source: Frontiers in Pediatrics

DOI: 10.3389/fped.2026.1929151

Author: Zainab Abdulaziz Alzayer

Institutions: King Fahd Hospital of the University, Imam Abdulrahman Bin Faisal University

Language: English

Topic: Anesthesia and Sedative Agents

Subfield: Anesthesiology and Pain Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $3,801