Ratzlaff, Robert A.

Acute porphyria is a group of rare disorders in the biosynthesis pathway of heme that can result in severe neurovisceral attacks leading to morbidity and mortality. Perioperative complications have been largely prevented due to avoidance of precipitants and early treatment of symptoms. However, these measures may not always be successful, because not all physiological stressors can be evaded. This case illustrates a porphyria attack precipitated by prolonged cardiopulmonary bypass that manifested as postoperative delayed emergence, failure to wean from mechanical ventilation, autonomic insufficiency requiring significant vasoactive agents, and, ultimately, failure to thrive. The patient passed after withdrawal of care.

Although no consensus exists on whether extracorporeal membrane oxygenation (ECMO) support is an indication for solid-organ transplantation other than heart and lung, this practice continues to be limited. We present a case of a 55-year-old man who was placed on venoarterial ECMO (VA-ECMO) during orthotopic heart transplantation (OHT) because of acute graft failure. Twenty-four hours later, the patient underwent deceased-donor renal transplantation (DDRT) while on VA-ECMO and had no complications. On post-DDRT day 2 (post-OHT day 3), the patient was successfully decannulated from VA-ECMO and ultimately discharged home. This case highlights the potential successful use of ECMO support during solid-organ transplantation other than heart and lung and the importance of trained providers and tailored anticoagulation. To the authors’ knowledge, this is the first report of perioperative ECMO use during kidney transplantation after recent heart transplantation.

Ornithine transcarbamylase (OTC) deficiency is well known for its diagnosis in the neonatal period. Presentation often occurs after protein feeding and manifests as poor oral intake, vomiting, lethargy progressing to seizure, respiratory difficulty, and eventually coma. Presentation at adulthood is rare (and likely underdiagnosed); however, OTC deficiency can be life-threatening and requires prompt investigation and treatment. Reports and guidelines are scarce due to its rarity. Here, we present a 59-year-old woman with a past history of irritable bowel syndrome who underwent a reparative operation for rectal prolapse and enterocele. Her postoperative course was complicated by a bowel perforation (which was repaired), prolonged mechanical ventilation, tracheostomy, critical illness myopathy, protein-caloric malnutrition, and altered mental status. After standard therapy for delirium failed, further investigation showed hyperammonemia and increased urine orotic acid, ultimately leading to the diagnosis of OTC deficiency. This case highlights the importance of considering OTC deficiency in hospitalized adults, especially during the diagnostic evaluation for altered mental status.