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LETTER TO EDITOR
Year : 2017  |  Volume : 11  |  Issue : 3  |  Page : 354-355

Scoliosis correction in an adolescent patient with Dandy-Walker syndrome: A case report


Department of Anesthesia and Intensive Care Unit, Khoula Hospital, Mina al Fahal, Muscat Sultanate of Oman

Correspondence Address:
Murugesh Sukumar
Department of Anesthesia and Intensive Care Unit, Khoula Hospital, Mina al Fahal, P.o. Box: 90 PC 190
Muscat Sultanate of Oman
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1658-354X.206784

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Date of Web Publication29-Jun-2017
 


How to cite this article:
Sukumar M, Nair S. Scoliosis correction in an adolescent patient with Dandy-Walker syndrome: A case report. Saudi J Anaesth 2017;11:354-5

How to cite this URL:
Sukumar M, Nair S. Scoliosis correction in an adolescent patient with Dandy-Walker syndrome: A case report. Saudi J Anaesth [serial online] 2017 [cited 2017 Sep 20];11:354-5. Available from: http://www.saudija.org/text.asp?2017/11/3/354/206784



Sir,

Dandy-Walker malformation is a rare congenital malformation and involves the cerebellum and fourth ventricle with an incidence between 1 in 25,000 and 1 in 30,000 live births. It is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. Numerous associated problems may be present.[1],[2]

Increased frequency of an association with congenital heart disease, cleft palate, and neural tube defects appears to exist.

We report our clinical experience of a female child diagnosed with Dandy-Walker syndrome posted for scoliosis correction surgery. The patient was a 14-year-old child weighing 40 kg and presenting for scoliosis correction. On preanesthetic examination, she had normal head circumference. Her mouth opening was adequate (MP 1) and neck movements were normal. She gives history of convulsions on treatment with anticonvulsants. Her mental development was normal with delayed milestones. The rest of her systemic examination was normal. Detailed preoperative evaluation was done. Intraoperatively, her vitals were monitored by an electrocardiogram, pulse oximetry, noninvasive blood pressure, and ETCO2. Intravenous access secured inside the operation theater with a 22 G cannula. Optimal position was given before induction of anesthesia.

She was induced with intravenous fentanyl 2 mcg/kg followed by injection propofol with a dose of 3 mg/kg, and injection cisatracurium 0.15 mg/kg was given to facilitate endotracheal intubation. She was intubated with a 6.5 number cuffed flexometallic tube using the standard laryngoscopic technique. Adequate protection to prevent soft tissue injuries of mouth was taken in view of intraoperative neuromonitoring.

One large bore IV cannula was inserted and left radial artery was cannulated after induction. Anesthesia was maintained with minimal sevoflurane maintaining a minimum alveolar concentration of <0.5 in view of neuromonitoring. Injection remifentanil and infusion propofol were used to maintain the depth of anesthesia. No additional dose of relaxant or infusion was used to facilitate efficient neuromonitoring which is an integral part of scoliosis surgery.

During the course of the surgery, she remained hemodynamically stable with her pulse rate being maintained between 60 and 70/min and blood pressure mean maintained at 60–65 mm of hg as part of induced hypotension. She was being maintained on oxygen with air and sevoflurane.

Chances of developing convulsions though rare is a major problem during neuromonitoring.[3]

The procedure took 3½ h and went uneventful. At the end of surgery, the surgeon inserted an epidural catheter under direct vision for postoperative pain management. Before extubation, a bolus of fentanyl/bupivacaine mixture was administered. Extubation was done in 15° head-up position. The patient showed smooth regular respiratory attempts before extubation which supported the decision to extubate. She maintained saturation on room air between 99% and 100%, so we proceeded with reversal with injection neostigmine 0.04 mg/kg and injection glycopyrrolate 0.01 mg/kg. It was uneventful. Postoperatively, the patient was shifted to Intensive Care Unit for further monitoring and pain management including the continuous epidural infusion of fentanyl/bupivacaine (0.1% bupivacaine and fentanyl 2 mic/ml). As an anesthetist, we might be called in for facilitating patients for computed tomography or magnetic resonance imaging investigations. When posted for surgery check for features of raised ICP, it may be exacerbated by any depression of ventilation, so full general anesthesia with controlled ventilation is preferred. Delayed resumption of spontaneous breathing may be encountered because of inherent abnormalities in respiratory control and might warrant a brief period of ventilator support.[4]

In conclusion, literature search did not reveal such a case of Dandy-Walker syndrome undergoing a major surgery like scoliosis correction. We hereby report the first case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Benda CE. The Dandy-Walker syndrome or the so-called atresia of the foramen Magendie. J Neuropathol Exp Neurol 1954;13:14-29.  Back to cited text no. 1
[PUBMED]    
2.
Brown JR. The Dandy-Walker syndrome. In: Vinken PJ, Bruyn GW, Klawans HL, editors. Handbook of Clinical Neurology. Vol. 30. Amsterdam: Elsevier Science Publishers; 1977. p. 62346.  Back to cited text no. 2
    
3.
Davis SF, Altstadt T, Flores R, Kaye A, Oremus G. Report of seizure following intraoperative monitoring of transcranial motor evoked potentials. Ochsner J 2013;13:558-60.  Back to cited text no. 3
[PUBMED]    
4.
Ewart MC, Oh TE. The Dandy-Walker syndrome. Relevance to anaesthesia and intensive care. Anaesthesia 1990;45:646-8.  Back to cited text no. 4
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