Successful management of a case of Tessier's cleft number 0 and 14 with associated encephalocoele.

INTRODUCTION

Facial cleft is the second most common congenital malformation of the entire body.[1] Various classifications have been described with Tessier's being most popular.[2]

Tessier's number 0 and 14 constitute the midline cleft.[2] Its association with encephalocoele and hypertelorism is very common. It is important for the anaesthesiologists to understand the complexity of surgery and that the main goal of early postnatal reconstruction is establishing proper airway.

CASE REPORT

An 8-month-old female infant weighing 5 kg diagnosed with Tessier's cleft number 0 and 14 with frontal encephalocoele was posted for reconstruction surgery. The child belonged to ASA Class 1. She had hypertelorism, bifid nose and bifid upper lip with palate along with malrotated bilateral maxilloalveolar bony parts [Figure 1]. Systemic examination and routine blood investigations were within normal limits. Computerised tomography scan of the infant revealed midline defect in maxilla, absent nasal bone and nasal septum with frontonasal encephalocoele [Figure 1]. The child was prepared as per standard guidelines. Electrocardiography (ECG), peripheral oxygen saturation (SPO2), non-invasive blood pressure (BP), end-tidal CO2 (ETCO2) and temperature were monitored. On receiving in the operation theatre, she had a pulse rate of 154/min, BP of 80/40 mmHg and SpO2 97% on room air. ECG and echocardiogram were within normal limits. Anaesthesia was induced with halothane in incremental concentrations (at a fresh gas flow of 5 L/min) with oxygen till the child lost her consciousness. Adequate seal of face mask was attained. Intravenous (IV) access was secured using 24 gauge cannula. Fentanyl 10 μg and propofol 10 mg were injected IV. Check laryngoscopy revealed good view of cord (Cormack-Lehane Grade 1). Injection vecuronium 0.5 mg was administered IV, and the trachea was intubated using uncuffed South Pole RAE tube of size 4. The tube was secured after confirming its correct placement and ensuring bilateral equal air entry. Ryle's tube was inserted, and packing of the throat was performed to prevent tube dislodgement. The patient was catheterised to monitor urine output. Anaesthesia was maintained with oxygen, nitrous oxide and isoflurane in titrated doses. Other drugs administered included intramuscular morphine (1.5 mg), IV fentanyl (12.5 μg), paracetamol suppository (170 mg), IV dexamethasone (2.5 mg) and IV sulbactam sodium/cefoperazone sodium (140 mg). Precaution was taken to prevent hypothermia. Blood, fresh frozen plasma (FFP) and colloid were transfused in calculated doses keeping a strict eye on blood loss. Intraoperative blood sugar was 134 mg/dl and arterial blood gas analysis (ABG) was within normal limits. Successful facial cleft, cleft lip and cranial fossa reconstruction with closure of hard palate were performed [Figure 1b]. The total duration of surgery was 7 h with a blood loss of 400 ml. The intraoperative course was uneventful. Trachea was extubated on the second post-operative day. Post-operative blood investigations and ABG were within normal limits. Her intensive care unit (ICU) stay was uneventful and was transferred to the general ward on 3rd post-operative day and discharged from hospital on 15th post-operative day.

Figure 1

Maxillary cleft with hypertelorism, bifid nose and bifid upper lip with palate (a), immediate post-operative state with midline closure and end tidal tube in situ (b), pre-operative computerised tomography-widely placed nasal cavities (small arrows), bifid hard palate and maxilla (long arrows) (c), coronal reformatted images showing encephalocele (black arrow) (d) and bony defects better seen in volume rendered computerised tomography image (e)

DISCUSSION

Through 50–60th gestational days, the palatal shelves and eventually hard, soft palate are formed. Tessier's cleft 0 and 14 (midline cleft) develops due to failure of structures derived from first/second branchial arches involving frontal bone along with duplication of Crista galli, midline of nose and upper lip, maxilla.

Various genetic and environmental factors described for its aetiopathogenesis are correlated with changes in transmembrane protein1, gad1, glutamate decarboxylase, maternal advanced age, stress, malnutrition, smoking, alcohol and drug abuse.[34] The potential anaesthetic difficulties associated with this case are anticipated difficulty in mask ventilation owing to associated facial deformity, difficulty in intubation due to associated encephalocoele, rapid circulatory volume loss leading to haemodynamic disturbances are a few to mention. We anticipated these problems pre-operatively and took adequate measures beforehand, for example, difficult airway cart including tracheostomy set and fibreoptic bronchoscope was kept ready. Inability to ventilate constitutes the most common cause of airway-related morbidity in paediatric population. One debate related to this case is use of muscle relaxant before intubation owing to anticipated difficult airway, but Gunawardana et al. in his 10 years prospective study on 800 patients with cleft lip and palate commented that use of neuromuscular blockers after confirmation of ability to ventilate the lung was associated with intubation success.[5] We also used muscle relaxant after doing check laryngoscopy in a deeper plane. Intraoperative care needed to be taken for reducing pressure on the brain during cranial remodelling. Although this surgery is extradural, reduction in brain bulk can be done by using drugs such as mannitol, 30° head up position and use of mild hyperventilation for reduced partial pressure of oxygen. Bradycardia can also occur due to pressure on the brain and pull on the eyeballs. Haemodynamically significant bradycardia only needs to be treated with IV atropine if there is no response on release of pressure on eyeball. Venous air embolism is another complication to be anticipated. Many venous channels of skull remain open during surgery in head up position. Prevention is possible by covering the sites with bone wax and saline soaked cotton pellets, maintaining adequate hydration. We kept a close observation for evidence of air embolism by monitoring ETCO2. Extensive blood and third space loss from scalp and cranium were anticipated; blood and FFP was kept ready for the same. Fluid status was maintained by replacement with crystalloids, colloids and packed RBCs by meticulous calculation (6-8 ml/kg) and maintaining adequate urine output. Blood loss was also reduced by judicious infiltration of vasopressors such as adrenaline (1:400,000) and meticulous surgical technique. Liaison with ICU in pre-operative period is also mandatory in view of the risk of major haemorrhage, ensuing complication of volume replacement and potential requirement of post-operative ventilatory support as these patients are prone to respiratory depression following opioids and sedatives. The causes for deterioration are airway obstruction, hypoxia, hypercarbia, cerebral oedema and intracranial bleeding, hypoglycaemia and electrolyte imbalance. Surgery was uneventful as were post-operative ICU stay, extubation and recovery in the present case.

CONCLUSION

Management of Tessier's cleft number 0 and 14 with encephalocoele can be successful with anticipation of difficulties, adequate preparation and team approach.

Declaration of patient consent

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Conflicts of interest

There are no conflicts of interest.