# Life-Threatening Haemodynamic Instability During General Anaesthesia in a Child With Renal Tubular Dysgenesis: A Case Report

## TL;DR

A child with a rare kidney disorder faced life-threatening low blood pressure during anesthesia, but careful planning helped manage it safely later.

## Contribution

Highlights safe anesthesia strategies for children with renal tubular dysgenesis based on a clinical case.

## Key findings

- A child with RTD developed life-threatening hypotension during anesthesia.
- Stable hemodynamics were achieved with fluids and vasopressors in a subsequent surgery.
- Detailed preoperative planning is crucial for anesthesia in RTD patients.

## Abstract

Autosomal recessive renal tubular dysgenesis (RTD) is attributed to a rare genetic mutation affecting the renin-angiotensin system, leading to reduced production and activity of angiotensin II. Because only a small number of patients survive beyond the neonatal period, information on safe anaesthetic management remains scarce. This report describes the case of a seven-year-old girl with autosomal recessive RTD who developed life-threatening hypotension requiring cardiopulmonary resuscitation immediately after induction of anaesthesia for surgery. When she was rescheduled for surgery a year later, we maintained a stable haemodynamic status by perioperative administration of fluids and careful administration of anaesthetics and vasopressors during general anaesthesia. Anaesthetists should be aware of the potential for life-threatening hypotension during general anaesthesia in children with RTD. General anaesthesia in these children requires detailed preoperative planning with adequate peri-anaesthetic fluid administration, careful titration of anaesthetics, and the use of vasopressors to prevent lethal hypotension.