Rajarajeshwari Medical College and Research hospital
DR.CHAITANYA, MD Anesthesia
Departmentof Anesthesiology and Critical Care
Rajarajeshwari Medical College and Research hospital
Anesthesiologists come across pediatric patients with rare diseases and syndromes scheduled for various operative interventions. Treacher Collins syndrome is a rare genetic disorder characterized by craniofacial deformities, the incidence being 1 in 40,000 - 70,000 births1,3 . Treacher Collins syndrome (TCS) poses serious problem in securing and maintaining airway due to facial deformity. Difficulty in intubation increases as the patient’s age increases. It requires meticulous planning and assessment of the airway prior to each anaesthetic technique. Here we describe and discuss successful anaesthetic management of an 8 year old boy posted for cannuloplasty of the right ear.
Treacher Collin’s syndrome,
Treacher-Collins syndrome is a rare disorder of craniofacial development which results in mandibulafacial dysostosis. It is a congenital malformation of the first and second bronchial arches inherited as autosomal dominant trait with a variable expressivity.
Most of the patients with TCS present with the following classic facial features: down-sloping palpebral fissures, colobomata of the lower eyelid, scanty lower eyelashes, malar hypoplasia, and micrognathia or retrognathia.
The patients with TCS have a serious problem in mask ventilation, securing and maintaining airway in the perioperative period. Retrognathia, macroglossia and other associated abnormalities result in limited mouth opening, reduced extension of the head on the neck, hypoplastic mandible and limited forward movement of hyoid.
During the postoperative period, pharyngeal and laryngeal edema may result in respiratory distress and sudden death which have been reported in the literature.
We report a case of general anaesthesia in a child with TCS who was scheduled for cannaloplasty of the right ear.
CASE REPORT An 8-year-old boy weighing about 20kg, diagnosed as Treacher-Collins syndrome was scheduled for cannaloplasty of the right ear. On evaluation the patient was found to have hypoplasia of facial bones, down sloping palpebral fissures, coloboma of the lower eyelids, scanty lower eye lashes and micrognathia and retrognathia. The child had narrowing of auditory canals on both sides, for which he was taken up for cannaloplasty of right ear. Airway assessment revealed adequate mouth opening with Mallampatti class 4 airway. Neck movements and spine were normal. All investigations and systemic examination were normal.
In view of the difficult airway, tracheostomy consent was obtained. A difficult airway cart was kept ready including LMA, tracheostomy set and emergency cricothyroidotomy set. The child was fasted for six hours. As age was a limitation for awake intubation, we planned for smooth induction with a deeper plane of anesthesia ensuring adequate ventilation and preventing trauma to the airway. Child was premedicated with glycopyrrolate 0.2mg IV to reduce the secretions. With standard Monitoring and proxygenation anaesthesia was induced with incremental dose of sevoflurane & IV propofol 1mg.kg-1. Initially mask ventilation seemed to be difficult due to a poor mask fit but improved to some extent after an orophrayngeal airway insertion and gauze packing of the space between the mask and the cheek. Adequate ventilation was achieved after applying jaw thrust. After induction, a gentle laryngoscopy showed a Class IV of glottis visualization as per Cormack Lehane classification.
With incremental doses of sevoflurane, appropriate depth of anaesthesia was achieved. With the child’s head turned slightly to the left and the assistant was asked to give a very good backward upward right-ward pressure (BURP). With this maneuver laryngoscopy revealed a Cormack Lehane of glottis visualization as Class III. Intubation was then accomplished with no.6 cuffed endotracheal tube with the help of a stylet. Correct placement of the tracheal tube was confirmed. Further anaesthesia was maintained with N2O+ O2+ sevoflurane and atracurium with fentanyl 2µkg-1 IV for analgesia. Adequate oxygenation and good haemodynamic stability was maintained through the surgery. Dexamethasone 2mg was also given intravenously. At the end of the surgery, the neuromuscular block was reversed with neostigmine 0.05mg.kg-1 and glycopyrrolate 0.02mg.kg-1. Extubation was carried out when the child got awake and thereafter shifted to recovery ward for further postoperative monitoring. The patient was discharged after 7 days without any complications.
The patients with TCS, posted for various surgeries, pose a problem to the anesthetists with regards to their airway management5. It may be due to relative macroglossia as a consequence of skeletal abnormalities. 3,5. The abnormalities which are associated with TCS may be limited mouth opening, reduced extension of the head on the neck, a hypoplastic mandible and a limited forward movement of the hyoid. Often, multiple mechanisms may be present in an individual case5,6.
Our patient presented with classical features of Treacher-Collins syndrome with significant airway distortion, because of which we had expected difficulty in maintaining airway as well as difficult tracheal intubation.
Awake fibreoptic intubation, can be uncomfortable and stressful for the patient and it requires expertise and patient’s cooperaton8.
Retro grade tracheal intubation provides higher success rate It is traumatic & painful especially in pediatric patients6,8,12.
These difficulties can be easily overcome by using LMA & ILMA (Intubating LMA) . However, the pediatric patients with Treacher-Collins syndrome have the posteriorly protruded tongue which displaces the LMA, makes the glottis move considerably anterior and interfere with the attempts to enter the trachea with a bougie. Further downward displacement of the epiglottis can also impair the intubation technique through LMA. Similar problems are encountered while attempting light wand guided intubation7,9,10.
Recently airway management of children with Treacher Collins syndrome (TCS) has been reviewed by Hosking,j.et al. in 240 anesthetics. MCL grade increased with increasing age (P = 0.007) 11
Considering the pros & cons of the various techniques, and keeping difficult airway trolley & tracheostomy kit as standby, we tried attempting conventional approach with direct laryngoscopy with suitable modifications. We avoided the use of sedatives that depress respiration preoperatively. Intraoperatively we achieved smooth & deeper plane of anaesthesia by inhalational agents. Patient was kept breathing spontaneously and deeper plane of anaesthesia was achieved to provide adequate time to attempt intubation. The forward lift of both the angles of the mandible by an assistant facilitated ventilation to a great extent as retrognathia is main constraint in TCS patients. And finally intubation was facilitated by a straight blade, lateral approach, very good backward upward and rightward pressure (BURP) by an assistant which made Cormack Lehane of glottis visualization as Class III.
The straight blade causes minimal compression on the soft tissues of the large sized tongue thus allowing an equal distribution of tongue tissue on either side of the blade and making a clear central path for proper visualization of the glottis and intubation12, 14. Improved view by extension of the head is possible with use of straight blade. Lateral placement of the blade by-passes the tongue13, 14.
Anticipation of a difficult airway and intubation is very crucial in successful management of these patients. We employed modified conventional technique using lateral approach to laryngoscopy, forward lifting of both the angles of the mandible and BURP maneuver to intubate our patient.
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