AGS is an autosomal-dominant condition affecting several body systems and characterized by neonatal jaundice along with a paucity of intrahepatic bile ducts on liver histology. It is also known as Alagille-Watson syndrome or arterio-hepatic dysplasia, and is caused by defects in the Notch signaling pathway. The disease affects the liver, heart, skeleton, eyes, kidneys, central nervous system, and is associated with characteristic facial features. (1, 2) The syndrome was first described by MacMahon and Thannhauser in 1949, who also characterized the histological changes observed in affected patients as "hypoplasia of intrahepatic bile ducts," "paucity of intrahepatic bile ducts," and "intrahepatic biliary atresia” to define their findings. (3)
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The patient was intubated with a size 6 endotracheal tube using a McGrath video laryngoscope. Desflurane 2.5-4.3% was used for anesthesia maintenance, supplemented with muscle relaxation with vecuronium. Monitoring included standard anesthesia monitors, bispectral index (BIS), twitch stimulator, invasive central venous (CVP), and arterial pressure monitoring. Intraoperative transesophageal echocardiography (TEE) was considered and made available at the bedside, although it was not utilized due to its interference with the cranial fluoroscopy imaging. Mechanical ventilation using pressure-controlled ventilation (PCV) mode was used throughout the case with the following parameters: peak airway pressure 15 cmH2O, FiO2 0.4-0.6, fresh gas flow 1-1.5 L/min. Normocapnia was maintained throughout the surgery. CVP remained stable at 16-22 mmHg.
Surgery included diagnostic cerebral arteriography with coil embolization of left posterior communicating artery aneurysm, and placement of right external ventricular drain (EVD). The estimated blood loss during the procedure was minimal (less than 50 ml), and the total intraoperative fluid intake was 500 ml of crystalloids. The patient remained hemodynamically stable during anesthesia and did not require any vasopressor
The patient was intubated with a size 6 endotracheal tube using a McGrath video laryngoscope. Desflurane 2.5-4.3% was used for anesthesia maintenance, supplemented with muscle relaxation with vecuronium. Monitoring included standard anesthesia monitors, bispectral index (BIS), twitch stimulator, invasive central venous (CVP), and arterial pressure monitoring. Intraoperative transesophageal echocardiography (TEE) was considered and made available at the bedside, although it was not utilized due to its interference with the cranial fluoroscopy imaging. Mechanical ventilation using pressure-controlled ventilation (PCV) mode was used throughout the case with the following parameters: peak airway pressure 15 cmH2O, FiO2 0.4-0.6, fresh gas flow 1-1.5 L/min. Normocapnia was maintained throughout the surgery. CVP remained stable at 16-22 mmHg.
Surgery included diagnostic cerebral arteriography with coil embolization of left posterior communicating artery aneurysm, and placement of right external ventricular drain (EVD). The estimated blood loss during the procedure was minimal (less than 50 ml), and the total intraoperative fluid intake was 500 ml of crystalloids. The patient remained hemodynamically stable during anesthesia and did not require any vasopressor