Case Report Treacher Collins Syndrome : An Alternative...

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Treacher Collins Syndrome : An Alternative Airway Management

Ruenreong Leelanukrom M.D., MPA,*

Petch Wacharasint M.D.*

∫∑§—¥¬àÕ : ∑“ß‡≈◊Õ°¢Õß Airway Management „π Treacher Collins Syndrome √◊Ëπ‡√‘ß ≈’≈“πÿ°√¡ æ.∫., √ª¡.,* ‡æ™√ «—™√‘π∏ÿå æ.∫.*

*¿“§«‘™“«‘—≠≠’«‘∑¬“ §≥–·æ∑¬»“µ√å ®ÿÃ“≈ß°√≥å¡À“«‘∑¬“≈—¬ ª∑ÿ¡«—π °√ÿß‡∑æœ 10330

·≈– mandible ‡≈Á° §≥–ºŸâ√“¬ß“π‰¥â√“¬ß“π∂÷ß°“√„à∑àÕÀ“¬

„®„πºŸâªÉ«¬ Treacher Collins syndrome ∑’Ë¡“√—∫°“√ºà“µ—¥

‡√‘¡‚Àπ°·°â¡ ‚¥¬¥—¥·ª≈ß‡∑§π‘§ “two anesthesiologist”

√à«¡°—∫°“√„™â soft-tip stylet „π°“√„à∑àÕÀ“¬„®

Thai J Anesthesiology 2006 ; 32(3) : 199-203.

*Department of Anesthesiology, Faculty of Medicine, Chulalongkorn University, Rama IV Road, Bangkok 10330, Thailand

Treacher Collins syndrome ‡ªìπ§«“¡º‘¥ª°µ‘·µà

°”‡π‘¥∑’Ëæ∫πâÕ¬ ª√–°Õ∫¥â«¬ À“ßµ“‡©’¬ß≈ß, coloboma ¢Õß

Àπ—ßµ“, µ“‡≈Á°, choanal atresia, °√–¥Ÿ° zygoma, maxilla,

mandible ‡®√‘≠πâÕ¬°«à“ª°µ‘ ÀŸÀπ«° À—«„®º‘¥ª°µ‘ ·≈–

Õ“®µ—«‡≈Á°°«à“ª°µ‘ °“√∑” direct laryngoscopy ·≈–„à∑àÕ

À“¬„®„πºŸâªÉ«¬°≈ÿà¡π’È®–≈”∫“°°«à“ª°µ‘ ‡æ√“–°√–¥Ÿ° maxilla

Introduction Treacher Collins syndrome is a rare mandi-

bulofacial disorder and often requires plastic surgery to

correct mandibulofacial abnormalities. This syndrome is

a great challenge to the anesthesiologist because the

distortions of mandibulofacial structures make airway

management and tracheal intubation problematic and

difficult.

Case report A twenty-year-old female, weighing 45 kg, with

bilateral malar hypoplasia was scheduled for malar aug-

mentation with rapid prototype. She was born at term,

with a birth weight of 3.8 kg after the onset of spon-

taneous labour and a normal vaginal delivery. The dys-

morphic features of this infant suggested a diagnosis of

Treacher Collins syndrome and thereafter she was fol-

lowed up by the department of plastic surgery regularly.

At fifteen years of age, she was admitted to the hospital

for reconstruction of left middle ear. Because of predicted

difficult airway, after laryngoscopy revealed difficulty in

visualizing the larynx (Cormack and Lehane grade IV)

with the application of anterior external laryngeal mani-

pulation, the airway management was shifted to a size-3

laryngeal mask airway (LMATM) under propofol anes-

thesia including initial induction dose of 3 mg/kg and

total dose of 4.6 mg/kg intravenously, but owing to the

Case Report

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200

201Thai Journal of AnesthesiologyVol. 32, No. 3, July-September 2006

inadequate depth of anesthesia, laryngospasm had oc-

curred. However, it was successfully treated with intra-

venous succinylcholine 25 mg (1.6 mg/kg). Spontaneous

ventilation became more active and was clinically satis-

factory ; both regular rhythmic of ETCO2 and oxygen

saturation curve and movement of the anesthesia bag was

observed. At the end of the surgery, the laryngeal mask

was removed by the patient’ s herself in the operating

room without complication. On the day of discharge, she

was active, cooperate and no complication was detected.

At sixteen years of age, she was admitted to the

hospital again twice for undergoing multiple sliding

genioplasty and zygomatic osteotomy with iliac bone

graft. Because of the difficulty to visualize the larynx

history, both anesthetic techniques were spontaneous

ventilation and fiberoptic bronchoscope assisted intuba-

tion after inhalation induction of sevoflurane with nitrous

oxide in oxygen. There was no complication of these two

previous anesthesia.

On examination, the patient showed several

dysmorphic features affecting the face. The combination

of microstomia, micrognathia, malar and mandibular

hypoplasia, and auricular defect was presented (Figure 1).

The results of preoperative laboratory investigations were

unremarkable. On the operative day, to minimize the risk

of airway obstruction, no premedication drug was given

before induction. A flexible fiberoptic bronchoscope

(FOB ; Olympus LF-2) was readily available. Standard

monitors including non-invasive blood pressure, electro-

cardiogram, pulse oximetry were applied. Venous access

was not difficult and a 18-G intravenous catheter was

sited in the dorsum of left hand. After preoxygenation

with 100% oxygen, anesthesia was induced with sevo-

flurane and spontaneous ventilation was continuously

maintained with sevoflurane titration up to 8%. As the

level of anesthesia deepened, mask ventilation became

difficult because of micrognathia, however, the 100%

peripheral oxygen saturation (SpO2) was well maintained

from her spontaneous ventilation. After the first laryn-

goscopy revealed difficulty in visualizing the larynx

(Cormack and Lehane grade IV) with the application of

anterior external laryngeal manipulation, the second

attempted direct laryngoscopy was done by more ex-

perienced anesthesiologist and the “two anesthesiologist

technique” was performed by one manipulated the

laryngoscope and larynx and the other inserted the tube

(Figure 2). At this time, the epiglottis was visualized

and revealed the larynx grade I. However, due to mi-

Figure 1 The typical appearance of treacher Collins syndrome : antimongoloid biquity of the palpebreal fissures, microstomia, microtia, micrognathia, choanal atresia, hypoplasia of the zygoma, maxillary, and mandibular bones

Figure 2 The modified “two anesthesiologist technique” was illustrated by one anesthesiologist manipulated the laryngscope and larynx and the other one inerted thesoft tip stylet

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200


crostomia appearance and anterior location of the larynx,

the tracheal tube was difficult to pass into the oral

cavity. This problem was solved successfully by using

the soft-tip stylet (Rusch no.3) inserted into the trachea

under direct visualization (Figure 3). Then, the Mallin-

ckrodt tracheal tube (PVC, 7.0 mm ID, 9.6 mm OD)

Discussion Treacher Collins syndrome was characterized by

Treacher Collins.1 This syndrome, which is also referred

to as mandibulofacial dysostosis, consists of down-

slanting palpebral fissures, colobomata of the lower

eyelid, scantly lower eyelashes, malar hypoplasia, and

micro-or retrognathia. There is a palatal cleft in 35% of

patients, and additional 30 to 40% have congenital

palatopharyngeal incompetence.2,3 Ear anomalies are

common and vary from minor malformation to severe

microtia which the operation is needed as described in

this case. Hearing loss is common, and can occur secon-

dary to atresia of the auditory canals or ossicular

malformation of the middle ear. The most life-threatening

concomitant morbidities in patients with Treacher Collins

syndrome are respiratory problems and respiratory

failure. Initially, there conditions were thought to be

secondary to glossoptosis. More recently, it has been

shown that the anatomy of the airway plays a major

role in determining the risk of airway compromise.

Patients with Treacher Collins syndrome might have

basicranial kyphosis, narrowing of the pharynx, and

severe microretrognathia with deficient ramal height.4,5

Patient with this syndrome may present anesthetic

problems involving difficulties in intubation and mask

fit. In addition, the space in the oral cavity that needed

to be displaced during laryngoscopy was diminished.6

Various techniques of airway management in Treacher

Collins syndrome were reported.7-10 But every approach

has its drawbacks. Bahk et al. reported a four-year-old

patient with Treacher Collins syndrome. The difficult

airway was managed by fibreoptic assisted intubation

through LMA.8 They did not attempt direct laryngoscopy

before inserting the LMA, so we did not know whether

intubation via direct laryngoscope was possible. Inser-

tion of LMA with owing to the light level of anes-

thesia, laryngospasm, coughing, or breath holding are

the potential complications. There have been several

reports of failure to correctly place the LMA and ven-

Figure 3 A soft-tip stylet (Rusch no.3)

was railroaded and advanced into the trachea. Finally,

the soft-tip stylet was removed. Spontaneous breathing

was maintained during airway procedure. Fiberoptic

laryngoscopy was not performed. Anesthesia was main-

tained with nitrous oxide 66% in oxygen and isoflurane

titration to clinical endpoints of heart rate and blood

pressure. Intravenous morphine was administered for

intraoperative and postoperative analgesia. The muscle

relaxant for maintenance was atracurium 25 mg intra-

venously. The anesthesia and surgery were uneventful

and lasted 360 min. There were no signs or symptoms

of upper airway obstruction observed. The tracheal tube

was removed in the operating room when the patient

demonstrated spontaneous eye opening, purposeful mo-

vement and adequate spontaneous ventilation. The patient

was transferred to the recovery unit and discharged

home 2 days later with good health.

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tilation through it after induction of general anesthesia.11

The original “two anesthesiologist” technique

described that one anesthesiologist manipulated the

laryngoscope with one hand and applied pressure on the

larynx with the other hand. In this way, optimum pres-

sure can be applied to bring the larynx into view. The

other anesthesiologist inserted the tracheal tube.12 How-

ever, microstomia in this patient limited the oral space

for tracheal tube insertion. We solved this problem by

inserting a soft-tip stylet (Rusch 3) into the trachea as

a Gum Elastic Bougie.TM The tracheal tube was then

loaded and advanced into the trachea. The soft-tip stylet

was smaller and hence it was easier to insert into the

trachea than tracheal tube with minimal tissue trauma.

This technique was useful for patient who had difficulty

in visualization of the larynx with microstomia.

In conclusion, Treacher Collins syndrome is a

syndrome associated with difficult mask ventilation and

difficult intubation. The anesthetic and airway manage-

ment should be prepared promptly for difficult airway.

However, when fibreoptic bronchoscope, laryngeal mask

airway or any invasive airway procedure are not avai-

lable, the direct laryngoscopy with the modified “two

anesthesiologist technique” with the soft-tip stylet as-

sisted tracheal intubation is a useful alternative airway

management.

References

1. Treacher CE. Case with symmetrical congenital notches in the outer part of each lid and defective development of the malar bones. Trans Opthalmol Soc UK 1900 ; 20 : 190-2.

2. Gorlin RJ, Toriello HV, Cohen MM Jr. Hereditary Hearing Loss and Its Syndromes. New York : Oxford University Press 1995 ; 62-5.

3. Dixon MJ. Treacher Collins syndrome. Hum Mol genet 1996 ; 5 : 1391-6.

4. Shapira J, Gleicher H, Moskovitz M, Peretz B. Respiratory arrest in Treacher Collins syndrome : Implications for dental management : Case report. Pediatr Dent 1996 ; 18 : 242-4.

5. Arvystas M, Shprintzen RJ. Craniofacial morphology in Treacher Collins syndrome. Cleft Palate Craniofac J 1991 ; 28 : 226-31.

6. Berry FA. Anesthesia for the child with a difficult airway. In : Berry FA, ed. Anesthetic management of difficult and routine pediatric patients. 2nd ed. Churchill Livingstone, New York 1990 : 142-67.

7. Miyabe M, Dohi S, Homma E. Tracheal intubation in an infant with Treacher-Collins syndrome-pulling out the tongue by a forceps (letter). Anesthesiology 1985 ; 62 : 213-4.

8. Bahk JH, Han SM, Kim SD. Management of difficult airways with a laryngeal mask airway under propofol anesthesia. Paediatr Anaesth 1999 ; 9 : 163-6.

9. Bahk JH, Kim JK, Kim CS. Use of the laryngeal mask airway to preoxygenate in a pediatric patient with Treacher-Collins syndrome. Paediatr Anaesth 1998 ; 8 : 273-6.

10. Bucx MJL, Grolman W, Kruisinga FH, et al. The prolonged use of the laryngeal mask airway in a neonate with airway obstruction and Treacher Collins syndrome. Case report. Paediatr Anaesth 2003 ; 13 : 530-3.

11. Benumof JL. The laryngeal mask airway and the ASA difficult airway algorithm. Anesthesiology 1996 ; 84 : 686-9.

12. Brown TCK, Fisk GC. Anaesthesia for Children, 2nd edn. Oxford: Blackwell Scientific Publications, 1992 ; 116-7.

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Treacher Collins Syndrome : An Alternative Airway Management

Abstract

Treacher Collins stndrome is a rare congenital disorder characterized by antimongoloid obiquity of the

palpebral fissures, coloboma, microphthalmia, choanal atresia, hypoplasia of the zygoma, maxillary and mandibular

bones, deafness, congenital heart defects, and occasionally dwarfism. The anesthetic challenge is difficult airway

from hypoplasia of maxillary, and mandibular bones which make direct visualization of the larynx and intubation

difficult. We present a case of this syndrome who was successfully intubated with the modified “two anesthesiologist

technique” with soft-tip stylet assisted tracheal intubation

Keywords : Treacher Collins syndrome, anesthesia, two anesthesiologist technique, soft-tip stylet assisted tracheal

intubation

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