Anaesthesia in an adult with Rubenstein–Taybi syndrome using the ProSeal laryngeal mask airway

S. J. Twigg* and T. M. Cook

Department of Anaesthesia, Royal United Hospital, Combe Park, Bath BA1 2NG, UK*Corresponding author

{dagger}LMA® is the property of Intavent Limited.

Accepted for publication: May 12, 2002


    Abstract
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
We report the anaesthetic management of an adult with Rubenstein–Taybi syndrome. This rare congenital syndrome is characterized by severe learning difficulties, cardiac abnormalities, gastrooesophageal reflux, and cranio-facial abnormalities with the likelihood of difficult intubation. A ProSeal laryngeal mask airway was used to ventilate the patient for eye surgery.

Br J Anaesth 2002; 89: 786–7

Keywords: complications, difficult intubation; complications, Rubenstein–Taybi syndrome; equipment, masks anaesthesia; gastrointestinal tract, gastroesophageal reflux


    Introduction
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
Rubenstein–Taybi syndrome (RTS) is a rare autosomal dominant genetic disorder first described in 1963.1 The underlying abnormality is disruption of a gene on chromosome 16. Severe learning difficulty with progressive blindness and deafness is universal. Cranio-facial abnormalities suggest difficult tracheal intubation (Fig. 1).2 3 Gastro-oesophageal reflux has been described and may lead to recurrent respiratory infections. Congenital heart disease is present in one-third of patients.4 Administration of suxamethonium has been associated with multiple cardiac arrhythmias.5 We describe the anesthetic management of an adult patient with RTS listed for bilateral phaecoemulsification and intra-ocular lens implants.



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Fig 1 A similar patient with RTS, illustrating characteristic cranio-facial abnormalities. Reproduced with permission from the BMJ Publishing Group; Berry AC. Rubenstein–Taybi syndrome. J Med Genet 1987; 24: 562–6.

 

    Case report
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
A 28-yr-old woman with RTS was seen before elective ophthalmic surgery. She suffered from severe learning difficulties, was deaf and had worsening vision because of cataracts. She was able to communicate by gestures. Her mobility was limited because of contraction deformities of the lower limbs, but she was able to feed and dress herself with help. Regurgitation of food was not reported, nor were recurrent respiratory infections. This suggested that she did not suffer from significant gastro-oesophageal reflux, although it was impossible to obtain a reliable history. Apart from ophthalmic and orthodontic problems she appeared to be in good health. She took no regular medication and had not had a previous anaesthetic.

Examination showed a woman of short stature (120 cm, 45 kg). She had a pronounced kyphoscoliosis. Her head was held flexed and extension of the neck was reduced, apparently by increased muscle tone. She had a small mandible, pronounced incisors, a high arched palate, and a high anterior larynx. The Mallampati airway grading as modified by Samsoon and Young was 3.6 7 We predicted difficult intubation. Examination of the cardiovascular system was normal. A normal full blood count, electrolyte profile, and ECG were obtained. Chest x-ray showed the kyphoscoliosis with small lung fields and a normal cardiac outline.

We planned to ventilate the patient via a ProSeal laryngeal mask airway (PLMA{dagger}), (Laryngeal Mask Company, Henley on Thames, UK). We made sure that two experienced anaesthetists, and equipment necessary to manage a difficult airway, including fibreoptic bronchoscope, were immediately available.

The patient received a H2 antagonist (nizatidine 150 mg), and a sedative (temazepam 20 mg), as premedication. Local anaesthetic cream (ametop) was applied to both hands. After non-invasive monitoring and a peripheral venous cannula had been placed, anaesthesia was induced with propofol 100 mg and fentanyl 50 µg. Adequate ventilation with a bag and mask was confirmed before a non-depolarizing neuromuscular blocking agent (atracurium 25 mg) was administered. We carried out laryngoscopy to ascertain the potential difficulty of future intubation, and the cords were partially seen (Cormack and Lehane Grade II view8). A size 4 PLMA was inserted and intermittent positive pressure ventilation was established. A size 14 orogastric tube was passed via the drainage tube of the PLMA and was aspirated. No fluid was obtained.

Anaesthesia was maintained with isoflurane 1% in 50% oxygen and nitrogen. Ventilation via the PLMA was readily achieved with inflation pressures less than 20 cm of water and with no evidence of gas leakage. The orogastric tube was left in place to vent the stomach throughout the case. The patient had uneventful bilateral phaecoemulsification with intra-ocular lens implants. Neuromuscular block was antagonized at the end of surgery and the PLMA was removed with the orogastric tube after spontaneous breathing had returned. The patient made an uncomplicated recovery from anesthesia and was discharged from hospital later the same day.


    Discussion
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 Abstract
 Introduction
 Case report
 Discussion
 References
 
Whilst RTS is rare, patients may present when they require surgery for the correction of orthopedic, orthodontic, ophthalmic or cardiac lesions, or for incidental surgical conditions. We found no reports of the anaesthetic management of adults with RTS in English language journals. The associated problems pose significant difficulty for anaesthetic management. Difficult intubation and recurrent respiratory problems might initially suggest regional anaesthesia would be preferable for suitable cases, but communication difficulties are likely to hamper compliance with such techniques. For the same reason awake fibre-optic intubation would be unsuitable. The reported high incidence of gastro-oesophageal reflux suggests that a rapid sequence induction with suxamethonium would be prudent, but the report of adverse reaction to this drug would be against this.5 Clearly, assessment must be made on an individual basis.

We considered difficult intubation as the biggest risk for this individual. However, the procedure planned was ophthalmic surgery for which the laryngeal mask airway is preferable from a surgical, anaesthetic, and patient perspective.9 The patient we describe had no significant history of gastro-oesophageal reflux but we considered some protection from aspiration of stomach contents advisable. The PLMA offers a compromise between the two options. It is specifically designed for intermittent positive pressure ventilation unlike the standard or reinforced LMA. The design aims to separate the respiratory and gastrointestinal tracts. The drain tube is designed to vent stomach contents and may, therefore, reduce the risk of regurgitation of stomach contents.10 An orogastric tube may also be reliably passed via this lumen into the stomach.11 In addition, the PLMA achieves a seal against the larynx that is approximately 50% higher than the classic LMA,11 12 reducing the likelihood of failed or inadequate ventilation. Whilst this patient could have been safely anaesthetized in several ways, by using the PLMA we were able to reliably ventilate the patient, confirm that the stomach was empty, and still provide an LMA-based anaesthetic, which is desirable for such ophthalmic cases.


    References
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 Abstract
 Introduction
 Case report
 Discussion
 References
 
1 Rubenstein JH, Taybi H. Broad thumbs and facial abnormalities. Am J Dis Child 1963; 105: 588–608[ISI]

2 Stirt JA. Anaesthetic problems in Rubenstein–Taybi syndrome. Anaesth Analg 1981; 60: 534–6[ISI][Medline]

3 Critchley LA, Gin T, Stuart JC. Anaesthesia in an infant with Rubenstein–Taybi syndrome. Anaesthesia 1995; 50: 37–8[ISI][Medline]

4 Stevens CA, Bhakta MG. Cardiac abnormalities in the Rubenstein-Taybi syndrome. Am J Med Genet 1995; 59: 346–8[ISI][Medline]

5 Stirt JA. Succinylcholine in Rubenstein–Taybi syndrome. Anesthesiology 1982; 57; 429

6 Mallampati SR, Gatt SP, Gugino LD, et al. A clinical sign to predict difficult tracheal intubation. Can Anaesth Soc J 1985; 32: 429–34[ISI][Medline]

7 Samsoon GLT, Young JRB. Difficult tracheal intubation: a retrospective study. Anaesthesia 1987; 42: 487–90[ISI][Medline]

8 Cormack RS, Lehane J. Difficult intubation in obstetrics. Anaesthesia 1984; 39: 1105–11[ISI][Medline]

9 Akhtar TM, McMurray P, Kerr WJ, Kenny GN. A comparison of laryngeal mask airway with tracheal tube for intra-ocular ophthalmic surgery. Anaesthesia 1992; 47: 668–71[ISI][Medline]

10 Keller C, Brimacombe J, Kleinsasser A, Loeckinger A. Does the ProSeal laryngeal mask airway prevent aspiration of regurgitated fluid. Anaesth Analg 2000; 91: 1017–20[Abstract/Free Full Text]

11 Cook TM, Nolan JP, Vergese C, et al. Randomized crossover comparison of the ProSeal with the classic laryngeal mask airway in unparalysed anaesthetized patients. Br J Anaesth 2002; 88: 527–33[Abstract/Free Full Text]

12 Keller C, Brimacombe J. Mucosal pressure and oropharyngeal leak pressure with the ProSeal vs laryngeal mask airway in anaesthetized paralysed patients. Br J Anaesth 2000; 85: 262–6[Abstract/Free Full Text]