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Lessons Learned From a Burn-Injured Pediatric Patient With Dandy–Walker Syndrome After Multiple Anesthetics: A Case Report

Khan, Asif MD; Kurtz, Brian MD; Ambardekar, Aditee MD, MSEd

doi: 10.1213/XAA.0000000000001014
Case Reports
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Dandy–Walker syndrome is a rare congenital brain malformation that requires unique anesthetic considerations. We present a rare case of an 18-month-old boy with Dandy–Walker syndrome presenting with 17% total body surface area burns undergoing multiple general anesthetics for both operative and bedside procedures. Numerous lessons were learned during multiple anesthetics, which influenced and guided our subsequent anesthetic management. The preoperative assessment, risk for respiratory apnea, opioid management, risk for laryngospasm, and postoperative care of patients with Dandy–Walker syndrome were all lessons learned.

From the Department of Anesthesiology and Pain Management, University of Texas Southwestern Medical Center, Dallas, Texas.

Accepted for publication February 26, 2019.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Aditee Ambardekar, MD, MSEd, Department of Anesthesiology and Pain Management, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390. Address e-mail to aditee.ambardekar@utsouthwestern.edu.

Dandy–Walker syndrome is characterized as cystic dilation of the fourth ventricle, hypoplasia of the cerebellar vermis, and hydrocephalus. The incidence of Dandy–Walker malformation is 1:25,000–30,000 of live births.1 In addition to the neurologic sequelae of Dandy–Walker syndrome, one-third of patients with Dandy–Walker syndrome will have other congenital anomalies.2 Patients with Dandy–Walker syndrome may also have craniofacial abnormalities, which may present significant challenges in safe airway management.3 These patients will require multiple anesthetics over their lifetime, because 80% will develop significant hydrocephalus requiring surgical intervention and anesthetic management.1 Furthermore, many will require interval computed tomography/magnetic resonance imaging, which can require anesthesia. With only a handful of cases in the literature reviewing the anesthetic management of Dandy–Walker syndrome, we present a case report of a toddler with Dandy–Walker syndrome who underwent multiple anesthetics. A written consent, which complied with the Health Insurance Portability and Accountability Act (HIPAA), was obtained from the patient’s guardian to use and disclose existing protected health information.

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CASE REPORT

An 18-month-old boy (13.6 kg) with a medical history of prematurity (born at 28 weeks gestational age) and Dandy–Walker syndrome was brought to our emergency department with 17% burns that were mixed superficial and deep partial thickness in a circumferential spread around his anterior and posterior trunk. He was admitted to the burn intensive care unit for fluid resuscitation and stabilization before going to the operating room for debridement and split thickness allografting.

Preoperatively, neurosurgery was consulted and a head computed tomography was obtained to assess the status of his Dandy–Walker malformation. Imaging revealed hydrocephalus with a larger retrocerebellar cyst and more diminutive brainstem than previously imaged over 6 months ago. Neurosurgery recommended surgical intervention to prevent further progression of his hydrocephalus, but it should not delay surgical management of his acute burns. No physical signs of increased intracranial pressure (ICP) or associated congenital anomalies were noted. As such, his burn injury and care took precedence and the team proceeded with debridement and split thickness allografting.

General anesthesia with an endotracheal tube (ETT) was planned due to the size of the burn, anticipated length of surgery, risk for significant blood loss, and high risk for postoperative apnea. No craniofacial or airway anomalies were apparent by physical examination; however, a video laryngoscope and fiber-optic scope were readily available at induction of anesthesia due to the associated risk of difficult airway in Dandy–Walker syndrome. An IV induction with propofol, fentanyl, and vecuronium was performed. A grade 1 Cormack–Lehane view was obtained using direct laryngoscopy with a Miller 1 blade. The patient was intubated with a 4.0-mm uncuffed ETT on the first attempt without complication. Maintenance of general anesthesia was accomplished with a sevoflurane (<1 minimum alveolar concentration [MAC]) regimen mixed with a total IV anesthetic (propofol/remifentanil), aimed at minimizing ICP.

During the patient’s first general anesthetic, he remained hemodynamically stable without the need for any transfusion products. Because neuromuscular blockade was used only to facilitate intubation, not redosed during the anesthetic, and the patient was demonstrating readiness for neuromuscular blockade reversal, we empirically reversed with glycopyrrolate and neostigmine without train-of-four monitoring. He received a total of 45 µg of fentanyl and 1 mg of morphine intraoperatively. Notably, immediately after extubation, the patient experienced an episode of apnea which required mask ventilation until naloxone (0.2 mg; 0.015 mg/kg) was administered. The last opioid administered before extubation was 0.5 mg of IV morphine 45 minutes prior. The patient responded well to naloxone and returned to the burn intensive care unit for close monitoring of his respiratory status and postoperative wound care. For the remainder of the patient’s hospitalization, he remained in the burn intensive care unit for close monitoring. During his hospitalization, he continued to experience episodes of morphine-induced hypopnea and apnea requiring naloxone reversal but never required a naloxone infusion.

On postoperative day 5, the patient required a second general anesthetic for wound care and surgical dressing change. A supraglottic airway was obtained with a size 2 laryngeal mask airway without complication. Maintenance anesthesia was >1 MAC of sevoflurane with pressure-supported spontaneous ventilation. At the end of the case, after removal of the laryngeal mask airway, under deep anesthesia (MAC >1), the patient experienced laryngospasm during transport to the intensive care unit. This episode was refractory to positive-pressure bag-mask ventilation and propofol, necessitating rapid reintubation with propofol. Days later, during mask induction for another general anesthetic, the patient experienced laryngospasm with rapid desaturation in oxygenation. This episode was managed with positive-pressure mask ventilation and deepening of the inhalational anesthetic.

The patient’s 2-month hospital course was further complicated by pseudomonal sepsis, disseminated intravascular coagulopathy, and gastrointestinal bleeding. His entire hospitalization consisted of a total of 10 general anesthetics, 8 for operative procedures and 2 for bedside procedures. After 57 days, the patient was discharged home in good condition with outpatient neurosurgery and burn care follow-up.

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DISCUSSION

Dandy–Walker syndrome was first characterized by Dandy and Blackfan4 in 1914, and then expanded on by Taggart and Walker5 in 1942. The term “Dandy–Walker Syndrome” was introduced by Benda in 1954 to group this triad of posterior fossa abnormalities.6 Our unique opportunity to provide 10 general anesthetics to a toddler with Dandy–Walker syndrome taught us critical lessons in the anesthetic management of these complex patients and the ability to contribute to the anesthesia literature on this rare topic.

Patients with Dandy–Walker syndrome require careful and complete preoperative assessment based on the extent of their posterior fossa abnormalities and any associated congenital abnormalities. With one-third of patients having congenital abnormalities, and 27% of those being cardiac abnormalities, it is critical that a full preoperative assessment takes this into consideration.2 Assessing for a history of difficulty feeding, recurrent chest infections, cardiac murmurs, abnormal pulses, or a history of cyanosis can help in determining whether a pediatric cardiology workup is necessary.

The most common presenting sign for patients with Dandy–Walker syndrome is hydrocephalus, with over 80% presenting before the first year of life.7 Preoperative clinical assessment for signs of increased ICP can be important in determining whether neurosurgery needs to be consulted and whether there is a need for a ventriculoperitoneal shunt. Irritability, seizures, and vomiting can all be signs of increased ICP warranting head imaging and neurosurgical consultation. Patients with known hydrocephalus might require repeat imaging, if it has been >6 months, to check for progression of their hydrocephaly and the need for any surgical intervention. In our case, neurosurgery requested interval head imaging, which demonstrated progression of his hydrocephaly.

Dandy–Walker syndrome is associated with congenital malformations that could yield a difficult airway. The presence of a cleft palate, macroglossia, or micrognathia can make laryngoscopy and tracheal intubation difficult.3 This can be further complicated if the patient demonstrates a dolichocephalic shaped head with a large occiput due to hydrocephaly, which causes neck hyperflexion when supine.8 A robust difficult airway plan should be developed. Supraglottic airway devices have been used as rescue airway devices with success.9

The risk for respiratory failure or apnea is significant for patients with Dandy–Walker syndrome due to pontine lesions that can interfere with the medullary control of respiration and lead to respiratory spasm, apneustic breathing, or apnea.3,10 Therefore, the return to spontaneous ventilation can be delayed and might require postoperative ventilation. Given the risk for respiratory failure, use of neuromuscular blockade should be minimized, and followed by train-of-four monitoring. A preoperative history of breathing abnormalities from caregivers can be an indicator of patients at risk for respiratory failure or apnea.8 A consideration for postoperative observation in an intensive care unit should be made given the stated risk for respiratory failure or apnea. After our patient had an episode of apnea postextubation, we were alerted to his risk, and he remained under intensive care unit observation during his entire hospital course.

Recognizing the risk for respiratory failure and apnea in our patient with Dandy–Walker syndrome caused us to modify and adapt our pain regimen. The use of morphine during our patient’s first general anesthetic led to an episode of postextubation apnea that required naloxone for full opioid reversal. Learning from this, we shifted our future intraoperative analgesia regimen away from morphine toward shorter-acting opioids, such as fentanyl. Furthermore, we incorporated a multimodal approach to analgesia that utilized acetaminophen, gabapentin, and ketamine to help minimize the opioid requirement and limit respiratory depression or apnea.11 While our patient was not a candidate given the extent and location of his burn wounds, regional anesthesia that includes caudal blocks or peripheral nerve blocks should be considered to minimize the opioid burden.

Laryngospasm is a primitive airway reflex characterized by involuntary spasm of the laryngeal muscles designed to protect against aspiration.12 In patients with brainstem lesions, neural plastic changes to laryngeal control networks can lead to controlling neurons being hypersensitive and patients being at high risk for laryngospasm.13 Due to the brainstem lesions associated with Dandy–Walker syndrome, a significant concern for laryngospasm should be associated with Dandy–Walker syndrome patients. During our 10 anesthetics, we had 2 episodes of laryngospasm. After an episode of laryngospasm with a supraglottic airway, we decided that all future general anesthetics should be done with an ETT given the patient’s risk.

In conclusion, Dandy–Walker syndrome is a rare congenital malformation with a wide range of clinical manifestations that have significant anesthetic implications. These patients require thorough preoperative assessment for associated congenital anomalies, increased ICP, or presentation with a difficult airway. Their risk for respiratory failure and apnea requires careful opioid consideration, multimodal analgesia, and possible postoperative intensive care unit observation. Brainstem lesions associated with Dandy–Walker syndrome can make these patients more susceptible to laryngospasm.

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DISCLOSURES

Name: Asif Khan, MD.

Contribution: This author helped conceive the report, analyze the data, and draft the manuscript.

Name: Brian Kurtz, MD.

Contribution: This author helped conceive the report, analyze the data, and draft the manuscript.

Name: Aditee Ambardekar, MD, MSEd.

Contribution: This author helped conceive the report, analyze the data, and draft the manuscript.

This manuscript was handled by: Kent H. Rehfeldt, MD.

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REFERENCES

1. Hirsch JF, Pierre-Kahn A, Renier D, Sainte-Rose C, Hoppe-Hirsch E. The Dandy-Walker malformation. A review of 40 cases. J Neurosurg. 1984;61:515–522.
2. Stambolliu E, Ioakeim-Ioannidou M, Kontokostas K, Dakoutrou M, Kousoulis AA. The most common comorbidities in Dandy-Walker syndrome patients: a systematic review of case reports. J Child Neurol. 2017;32:886–902.
3. Ewart MC, Oh TE. The Dandy-Walker syndrome. Relevance to anaesthesia and intensive care. Anaesthesia. 1990;45:646–648.
4. Dandy WE, Blackfan KD. Internal hydrocephalus - An experimental, clinical and pathological study. Am J Dis Child. 1914;8:406.
5. Taggart JK, Walker AE. Congenital atresia of the foramens of luschka and magendie. Arch Neurol Psychiatry. 1942;48:583.
6. Benda CE. The Dandy-Walker syndrome or the so-called atresia of the foramen magendie. J Neuropathol Exp Neurol. 1954;13:14–29.
7. Spennato P, Mirone G, Nastro A. Hydrocephalus in Dandy-Walker malformation. Childs Nerv Syst. 2011;27:1665–1681.
8. Moodley AW, Nel S, Oosthuizen E, Lundgren C. Anaesthetic management for ventriculoperitoneal shunt insertion in an infant with Dandy–Walker syndrome. South Afr J Anaesth Analg. 2017;23:17–20.
9. Selim M, Mowafi H, Al-Ghamdi A, Adu-Gyamfi Y. Intubation via LMA in pediatric patients with difficult airways. Can J Anaesth. 1999;46:891–893.
10. Krieger AJ, Detwiler J, Trooskin S. Respiration in an infant with the Dandy-Walker syndrome. Neurology. 1974;24:1064–1067.
11. Chiaretti A, Pierri F, Valentini P, Russo I, Gargiullo L, Riccardi R. Current practice and recent advances in pediatric pain management. Eur Rev Med Pharmacol Sci. 2013;17suppl 1112–126.
12. Gavel G, Walker RW. Laryngospasm in anaesthesia. Continuing Educ Anaesth Crit Care Pain. 2014;14:47–51.
13. Gdynia HJ, Kassubek J, Sperfeld AD. Laryngospasm in neurological diseases. Neurocrit Care. 2006;4:163–167.
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