Anesthesia and organic aciduria: is the use of lactated Ringer's solution absolutely contraindicated?

Růžková, Kateřina; Weingarten, Toby N.; Larson, Kelly J.; Friedhoff, Robert J.; Gavrilov, Dimitar; Šprung, Juraj

doi:10.1111/pan.12673

Cited by 7 publications

(10 citation statements)

References 26 publications

(53 reference statements)

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“…Following transplant, markers of metabolic control in our patients were improved in both the perioperative and postoperative periods. The importance of appropriate metabolic support in the setting of PA and MMA is well recognized, and recent reports have highlighted the preoperative and anesthetic considerations that complicate the transplant operation . As with the management of the disease prior to transplant, it is imperative that the immediate postoperative treatment minimizes catabolism to avoid metabolic decompensation.…”

Section: Discussionmentioning

confidence: 99%

Liver Transplantation for Propionic Acidemia and Methylmalonic Acidemia: Perioperative Management and Clinical Outcomes

et al. 2018

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Propionic acidemia (PA) and methylmalonic acidemia (MMA) comprise the most common organic acidemias and account for profound morbidity in affected individuals. Although liver transplantation (LT) has emerged as a bulk enzyme-replacement strategy to stabilize metabolically fragile patients, it is not a metabolic cure because patients remain at risk for disease-related complications. We retrospectively studied LT and/or liver-kidney transplant in 9 patients with PA or MMA with additional focus on the optimization of metabolic control and management in the perioperative period. Metabolic crises were common before transplant. By implementing a strategy of carbohydrate minimization with gradual but early lipid and protein introduction, lactate levels significantly improved over the perioperative period (P < 0.001). Posttransplant metabolic improvement is demonstrated by improvements in serum glycine levels (for PA; P < 0.001 × 10 ), methylmalonic acid levels (for MMA; P < 0.001), and ammonia levels (for PA and MMA; P < 0.001). Dietary restriction remained after transplant. However, no further metabolic crises have occurred. Other disease-specific comorbidities such as renal dysfunction and cardiomyopathy stabilized and improved. In conclusion, transplant can provide a strategy for altering the natural history of PA and MMA providing stability to a rare but metabolically brittle population. Nutritional management is critical to optimize patient outcomes.

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Section: Discussionmentioning

confidence: 99%

Liver Transplantation for Propionic Acidemia and Methylmalonic Acidemia: Perioperative Management and Clinical Outcomes

et al. 2018

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“…Case reports have offered insight into the perioperative management of specific OADs. [38][39][40] A theme throughout these case reports is the optimization of patients' health before surgery to reduce the risk of intraoperative and postoperative complications. These patients are at risk for developing dilated cardiomyopathy, arrhythmias, and chronic renal failure, so a preoperative workup, including echocardiogram, electrocardiogram, and baseline laboratory testing may be prudent.…”

Section: Discussionmentioning

confidence: 99%

“…Preoperative administration of intravenous fluids containing 10% dextrose with electrolytes at 1.5 to 2 times maintenance and continuing throughout and after surgery can reduce the risk of acute exacerbation. [38][39][40] In addition, the postoperative use of opioids should be monitored carefully because respiratory depression can reduce the ability to compensate for acidosis and increase the risk of postoperative respiratory infection. 39 Incentive spirometry is useful in preventing these complications.…”

Section: Discussionmentioning

confidence: 99%

Orthopaedic Problems in 35 Patients With Organic Acid Disorders

Prasad¹,

Gottlich²,

Nhan³

et al. 2021

Journal of Pediatric Orthopaedics

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Introduction: Organic acid disorders (OADs) are a subset of inborn errors of metabolism that result in a toxic accumulation of organic acids in the body, which can lead to metabolic derangements and encephalopathy. Patients with these disorders are managed by a team of biochemical geneticists and metabolic nutritionists. However, subspecialists such as neurologists and orthopaedic surgeons are often needed to help manage the sequelae of the metabolic derangements. The breadth of orthopaedic sequelae of these disease states is poorly understood. Herein, we describe orthopaedic problems associated with 5 types of OAD most commonly seen at our institution: maple syrup urine disease, methylmalonic aciduria, propionic aciduria, pyruvate dehydrogenase deficiency, and glutaric aciduria type 1. Methods: We retrospectively reviewed medical records of 35 patients with an OAD who were seen at our academic tertiary care center from May 1999 to May 2020. Patients were grouped into cohorts according to OAD type and analyzed for orthopaedic presentations of hip, knee, or foot disorders, presence and severity of scoliosis, history of fracture, movement disorders, and osteopenia/osteoporosis. Results: Of the 35 patients, 13 had maple syrup urine disease, 12 had methylmalonic aciduria, 4 had propionic aciduria, 4 had pyruvate dehydrogenase deficiency, and 2 had glutaric aciduria type 1. Associated orthopaedic problems included spasticity causing neuromuscular scoliosis and/or hip subluxation or dislocation (10 patients), fractures (7 patients), and osteopenia/osteoporosis (7 patients). Overall, 22 of 35 patients had some orthopaedic condition. Conclusions: Most in this cohort of patients with OAD also had an orthopaedic abnormality. It is important for physicians treating these patients to understand their propensity for musculoskeletal problems. When treating patients with OAD, it is important to initiate and maintain communication with specialists in several disciplines and to develop collaborative treatments for this unique population.

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“…Due to the risk of acidosis, we elected to use sevoflurane as our mainstay anesthetic over a propofol infusion. The lactate in Lactated Ringer's is poorly metabolized by the mitochondria in patients with PA and can also contribute to increased acid load [8]. Opioids can cause oversedation in patients who are developmentally delayed and should be used sparingly to avoid prolonged sedation from exaggerated depressive effects to the central nervous system.…”

Section: Figurementioning

confidence: 99%

Anesthetic Management of Children With Propionic Acidemia Undergoing Esophagogastroduodenoscopy

Alex¹,

Kiss²,

Saynhalath³

et al. 2021

Cureus

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Propionic acidemia is a rare genetic disorder of metabolism that predisposes patients to metabolic acidosis, lethargy, neurologic dysfunction, developmental delays, and cardiomyopathy. Perioperative anesthetic management is guided toward mitigating the effects of preoperative fasting times, maintaining normovolemia, and preventing cardiovascular complications secondary to underlying cardiomyopathy. Commonly used anesthetic agents may have undesirable side effects in these patients. Propofol, the lactate in Lactated Ringer's, and neuromuscular blocking agents that undergo ester hydrolysis are poorly metabolized and can lead to metabolic acidosis. Opioids, such as fentanyl and morphine, should be used judiciously in patients with coexisting developmental delays to avoid oversedation and delayed time to resuming oral intake postanesthesia. In addition, inhaled anesthetics have direct myocardial depressive effects and can compromise cardiac function in the setting of pre-existing cardiomyopathy. The perioperative period represents a critical time in this population and appropriate planning is crucial to prevent perioperative morbidity. We present a case of an eight-year-old child undergoing esophagogastroduodenoscopy under general anesthesia and describe the anesthetic concerns we addressed to provide a safe perioperative course.

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Anesthesia and organic aciduria: is the use of lactated Ringer's solution absolutely contraindicated?

Cited by 7 publications

References 26 publications

Liver Transplantation for Propionic Acidemia and Methylmalonic Acidemia: Perioperative Management and Clinical Outcomes

Liver Transplantation for Propionic Acidemia and Methylmalonic Acidemia: Perioperative Management and Clinical Outcomes

Orthopaedic Problems in 35 Patients With Organic Acid Disorders

Anesthetic Management of Children With Propionic Acidemia Undergoing Esophagogastroduodenoscopy

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