ROHHAD + Anesthesia Publications

FAQ

What specific anesthetic techniques successfully mitigate severe respiratory and autonomic risks?

Anesthetic management in patients with ROHHAD requires careful planning to minimize both respiratory and autonomic risks. Because central hypoventilation and obstructive sleep apnea are common, anesthetic agents should be selected with caution—short-acting intravenous drugs or volatile agents with low blood-gas solubility are preferred, while ketamine and dexmedetomidine have been used safely. Opioids should be avoided when possible, with non-opioid and regional analgesic techniques prioritized to reduce the risk of postoperative hypoventilation. Non-invasive ventilation (NIV) is often necessary following extubation, and patients should be trained in its use, with close postoperative monitoring that includes end-tidal CO₂ to detect apnea and carbon dioxide retention. Autonomic dysfunction adds further risk, with potential for blood pressure lability, arrhythmias, and thermal dysregulation; therefore, invasive blood pressure monitoring is recommended during major surgeries. Agents such as propofol or thiopental can worsen hypotension, while sevoflurane with ultra-short-acting opioids has been used successfully to maintain stability. Regional anesthesia may be considered but must be used cautiously given the risk of sympathetic blockade in these patients, and core temperature should be continuously monitored to avoid extremes.

Which pre-operative assessments are most critical for optimizing high-risk pediatric ROHHAD patients?

What are the general anesthetic guidelines for patients with ROHHAD?

Anesthetic management in ROHHAD requires meticulous planning to address risks of respiratory failure, endocrine dysfunction, and autonomic instability. Pre-operative evaluation should prioritize respiratory assessment, including pulmonary testing and screening for hypoventilation and obstructive sleep apnea, with training in non-invasive ventilation (NIV) when indicated. Endocrine optimization involves managing central adrenal insufficiency with peri-operative steroids, treating hypothyroidism, monitoring glucose tolerance, and carefully balancing fluids and electrolytes in cases of diabetes insipidus. Gastroparesis should also be assessed due to aspiration risk. Systemic evaluation includes screening for neuroendocrine tumors, assessing cardiac function, monitoring for thermal dysregulation, and reviewing long-term medications such as seizure or psychotropic drugs.

Intra-operatively, anesthesia should be individualized but guided by strategies that minimize respiratory and autonomic compromise. General and regional anesthesia are both viable, with cautious use of premedication. Short-acting intravenous agents with limited respiratory depression are preferred; ketamine and dexmedetomidine are considered safe, while propofol and thiopental may cause hypotension. Sevoflurane is commonly used, and volatile agents with low blood-gas solubility are recommended to facilitate recovery. Muscle relaxants are generally safe, but opioids should be minimized in favor of opioid-sparing and regional analgesia, given their risk of worsening hypoventilation. Ventilation should be carefully adjusted to maintain normocarbia. Continuous invasive blood pressure monitoring and core temperature regulation are critical due to dysautonomia and thermal instability.

Postoperative care should occur in the PICU or equivalent setting, with at least 24 hours of close monitoring. Patients are at high risk for prolonged apnea and CO₂ retention, so end-tidal CO₂ monitoring is essential. NIV should be continued after extubation, particularly in those who used it pre-operatively. Pain must be managed carefully, with a preference for non-opioid regimens to avoid respiratory depression.

Published ARticles

“Peri-operative management of a child with ROHHAD-NET syndrome undergoing neural crest tumour excision"by puri et al. (2022)

This article is a case report detailing the challenging peri-operative management of a nine-year-old girl with ROHHAD-NET syndrome (Rapid-Onset Obesity, Hypothalamic Dysfunction, Hypoventilation, Autonomic Dysregulation, and Neuroendocrine Tumor) undergoing excision of a large paravertebral ganglioneuroma. The report emphasizes that pre-operative optimization, well-planned intra-operative management, and intensive postoperative care are essential for achieving a favorable outcome in children with this complex syndrome due to the involvement of multiple organ systems.

Patient Profile: patient presented with classic features of ROHHAD-NET syndrome, which began around age seven. She presented with a large left paravertebral ganglioneuroma (T8–T12) causing lower limb weakness.

Pre-operative Optimization: Optimization focused on endocrine and respiratory status. Levothyroxine and propranolol were continued until the morning of surgery. The dose of desmopressin was omitted on the day of surgery due to the anticipation of major fluid shifts and prolonged fasting. Monitoring serum electrolytes and fluid balance was deemed essential, especially considering the disordered water balance from diabetes insipidus.

Intra-operative Course:

• Anesthesia: General anesthesia was induced with propofol and atracurium. Maintenance utilized isoflurane in an air–oxygen mixture, with ventilation adjusted to maintain normocarbia.

• Airway: A cuffed tracheal tube smaller than standard formulas predicted was required, possibly due to prior prolonged intubation and tracheostomy.

• Monitoring: Invasive blood pressure monitoring via a radial arterial line was used, which is mandated in major surgeries due to the likelihood of hypotension and hypertension stemming from autonomic dysfunction.

• Complications: Multiple episodes of hypotension (lowest recorded 54/30 mmHg) occurred during tumor handling, resolving when retractors were released. This instability was potentially contributed to by the underlying autonomic dysfunction.

• Analgesia: Analgesia included intravenous morphine and paracetamol, and local infiltration with ropivacaine. Epidural analgesia was avoided due to the tumor’s neural origin and the patient’s history of lower limb weakness; ultrasound-guided bilateral subcostal transverse abdominis plane block was suggested as a non-opioid alternative. The report discusses that using non-opioid analgesics and regional analgesia is advised to modify the technique and prevent hypoventilation

Postoperative Challenges and Outcome: Due to the prolonged surgery, past difficult weaning, and intra-operative hypotension, the patient was transferred to the Intensive Care Unit (ICU) for continued ventilation. Weaning was difficult, resulting in two failed extubation attempts. Successful tracheal extubation was achieved on postoperative day 20 following a staged weaning program. Upon discharge on postoperative day 29, the patient required domiciliary nasal BiPAP therapy. The report concludes that non-invasive ventilation following tracheal extubation is a reasonable and safe approach in children who continue to demonstrate hypoventilation. The patient was gradually weaned off BiPAP support over the following five months.

Full article: Peri-operative management of a child with ROHHAD-NET syndrome undergoing neural crest tumour excision-Web of Science Core Collection

"Anesthesia in a pediatric patient with ROHADD syndrome” by isasa et al. (2018)

This is a case report detailing the anesthetic challenges encountered during a minor surgical procedure in a child diagnosed with ROHHAD syndrome. A 10-year-old boy with ROHHAD (BMI 34.9) underwent port-a-cath insertion under general anesthesia. His comorbidities included rapid-onset obesity, hypothalamic dysfunction with severe electrolyte imbalance, central adrenal insufficiency, hypothyroidism, and severe central hypoventilation with oxygen dependence. He also demonstrated autonomic dysregulation (labile vitals, diaphoresis, fever) and aggressive behavioral disorder.

During anesthesia, multiple complications occurred. Apnea developed on induction with sevoflurane after oral midazolam, and again after fentanyl administration, requiring tracheal intubation. Mask ventilation was difficult, and a laryngeal airway was temporarily used. Maintenance with sevoflurane and remifentanil preserved stable hemodynamics, but administration of metamizole triggered a severe anaphylactoid reaction with wheezing, desaturation (<70%), and hypotension, requiring epinephrine and postoperative noradrenaline support. He was transferred to the PICU, extubated after 48 hours, and recovered.

This case highlights the high anesthetic risk in ROHHAD, with respiratory depression from sedatives and opioids posing the greatest danger. The authors emphasize opioid-sparing strategies, noting ketamine and dexmedetomidine as safer alternatives. Agents like propofol and thiopental should be used cautiously due to hypotension risk from autonomic instability. Overall, the case illustrates how respiratory compromise, obesity, and autonomic dysregulation compound perioperative risk and necessitate vigilant monitoring and tailored anesthetic management.
Full article: Anesthesia in a pediatric patient with ROHADD syndrome-Web of Science Core Collection

"Anaesthesia recommendations for patients suffering from ROHHAD syndrome” by Chandrakantan et al. (2018)

This document consists of the guidelines from the OrphanAnesthesia project concerning the anesthetic recommendations for patients suffering from ROHHAD syndrome. The OrphanAnesthesia project, led by the German Society of Anaesthesiology and Intensive Care Medicine, publishes standardized anesthesia recommendations for rare diseases to improve safety, drawing from scientific evidence and expert experience.

Peri-operative planning requires a thorough history and physical, with emphasis on respiratory evaluation (pulmonary testing, sleep studies, and training in non-invasive ventilation), endocrine assessment (thyroid, adrenal, glucose regulation, diabetes insipidus), electrolyte balance, autonomic and cardiac function (including pacemaker status and thermal dysregulation), and gastrointestinal motility. Long-term medications, especially seizure and psychotropic drugs, must also be reviewed.

For anesthetic management, both general and regional anesthesia are acceptable but require vigilance. Benzodiazepines may be used cautiously as premedication, while short-acting IV agents such as ketamine or dexmedetomidine are preferred intraoperatively. Opioids should be minimized to reduce risk of hypoventilation, with non-opioid analgesics or regional techniques considered. Regional anesthesia must be used cautiously due to the risk of sympathetic blockade in patients with autonomic dysfunction. Obesity and OSA increase the likelihood of difficult mask ventilation, though difficult intubation is not consistently reported.

Monitoring is critical: end-tidal CO₂ and blood pressure must be closely observed, and strict thermal regulation maintained. The most common complications occur when ROHHAD is unrecognized, often manifesting as prolonged postoperative apnea. In postoperative care, PICU admission and at least 24 hours of inpatient monitoring are recommended. Patients should continue preoperative NIV postoperatively, and pain should be controlled with judicious, opioid-sparing regimens to avoid respiratory depression.

Full article: Anaesthesia recommendations for patients suffering from ROHHAD syndrome-Web of Science Core Collection