Dandy Walker syndrome with giant occipital meningocele with craniovertebral anomalies: Challenges faced during anaesthesia

Sir, The coexistence of rare congenital neurodevelopmental and craniofacial abnormalities make proper positioning for endotracheal intubation difficult and alternative methods of positioning and intubation must be sought for. Although difficult airway cart should be in hand, one must give emphasis on proper positioning of patient. A sound knowledge and proper laryngoscopy by experienced anaesthesiologist results in successful intubation even in cases of anticipated difficult airway and thus, prevents the need of surgical airway. We report a rare case of an 8-month-old child weighing 5 kg with Dandy–Walker syndrome (DWS) associated with a giant occipito-cervical meningocele and craniovertebral anomalies, and posted for shunt surgery. Pre-anaesthetic check-up suggested no history of seizure, vomiting, difficulty in feeding, cyanosis or respiratory distress. There were no cardiac or respiratory signs. Power in all limbs was equal. The child had a large head and a giant occipital cervical meningocele measuring 10 cm × 10 cm, extending from occipital protuberance to mid scapula, causing fixed flexion of the neck [Figure 1]. Magnetic resonance imaging (MRI) of the brain showed enlarged posterior fossa with multiple craniovertebral anomalies with meningocele in occipital and upper cervical region communicating with fourth ventricle, hypoplasia of inferior vermis coupled with hypoplasia of cerebellar hemisphere, suggestive of DWS with occipital meningocele. MRI spine [Figure 2] showed multiple craniovertebral anomalies such as cranial bifida and spina bifida in upper cervical vertebrae coupled with occipito-cervical meningocoele. Figure 1 Extension of occipita cervical swelling along with large head Figure 2 Magnetic resonance imaging spine showing huge occipita cervical meningocele On arrival of the child in the operation theatre, monitoring was established with an electrocardiogram pulse oximeter non-invasive blood pressure (BP) and temperature probe. Baseline parameters included BP of 94/70 mmHg pulse rate of 118/min and oxygen saturation of 100%. Injection glycopyrrolate 0.02 mg intravenous (IV) and injection fentanyl 5 μg was given in the cannula in situ. As there was a large and firm swelling at occipito-cervical region along with flexed neck with no neck movement, supine positioning could not be done in our case. The patient was positioned in the left lateral position with head beyond the edge of the table with an assistant stabilising the head of the baby from below and other supporting the body. After pre-oxygenation with 100% oxygen, injection thiopentone IV was administered in titrated manner, and a total of 20 mg was injected. Check laryngoscopy in lateral position was done with Miller's straight blade one by the senior anaesthesiologist. Laryngoscopy revealed Cormack–Lehane laryngeal view Grade 2. Injection succinylcholine, 10 mg IV was administered and eventually trachea was intubated with no. 3.5 mm uncuffed endotracheal tube. After confirmation of bilateral equal air entry, the endotracheal tube was fixed. Injection atracurium 2.5 mg was given, and anaesthesia was maintained with nitrous oxide-oxygen and sevoflurane (1%). Paracetamol 80 mg suppository was placed for post-operative analgesia. Once the tube was properly fixed, the patient was positioned supine by placing large ring under the head and large cotton roll under shoulder for proper surgical field view. All the pressure points were adequately padded. Intraoperative course was uneventful. After completion of surgery, neuromuscular blockade was reversed with injection neostigmine 0.4 mg and injection glycopyrrolate 0.008 mg. Extubation was performed once the patient became awake. After satisfactory recovery, the patient was shifted to paediatric Intensive Care Unit. DWS although a rare clinical entity with incidence being about 1 in 25000-30000 live births with a slight female predominance.[1,2] It is often associated with other congenital anomalies such as cleft lip, cleft palate, hypertelorism and micrognathia making airway management challenging.[3] Ewart and Oh reported a case of 2 weeks old DWS patient with micrognathia and anterior larynx in whom they found difficulty in awake intubation.[4] Our child was positioned laterally during induction and intubation. An alternative technique such was suggested by Mowafi et al.[5] in which child was placed supine in silicon platform so that height of silicon platform equals the size of the sac. We did not go for this technique as the occipital swelling was extending until mid-scapula, making positioning more difficult. Other major concern in patients of DWS is avoidance of further rise in intracranial pressure (ICP). Endotracheal intubation should be as gentle as possible. Inhalational agent causes a dose-dependent rise in intracranial pressure and so better avoided. However, it has been shown that isoflurane and sevoflurane in <1 MAC concentration do not cause a significant rise in ICP.[6] In conclusion, patients with DWS with other associated anomalies can be successfully managed by a thorough evaluation of airway, emphasis on proper positioning during laryngoscopy, preparation for difficult intubation, avoidance of rise in ICP during anaesthesia and proper post-operative care. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.