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Anaesthesia and Chronic Pain
 

Anaesthetic Management of Correction of Bilateral Temporo Mandibilar Joint Ankylosis in a Patient with Klippel Feil Syndrome, Mandibular Retrognathia and Kyphoscoliosis

Indrani Hemant Kumar*, S Umbarkar*
 


Abstract
Patients with Klippel Feil syndrome with Tempero Mandibular Joint Ankylosis, Mandibular Retrognathia and Thoracic Kyphoscoliosis pose airway as well as other numerous problems to the anaesthesiologist. The anatomical abnormality and potentially unstable neck provide a potentially difficult tracheal intubation which was undertaken using an awake fibreoptic technique. The anaesthetic management of a patient with Klippel Feil syndrome and Temporo Mandibular Joint Ankylosis is discussed in this report.

Introduction
Klippel Feil syndrome is an inherited
condition characterized by the classic triad of shortness of neck, reduced neck movements resulting from the fusion of several cervical vertebrae or multiple fused hemivertebrae and a low posterior hairline.1 The syndrome is classified according to the site and extension of spinal fusion as follows: Type I – extensive cervical and upper thoracic spinal fusion; Type II – one or two interspace fusions often associated with hemivertebrae and occipito-atlantoid fusion; and Type III – type I or II fusion with co-existing fusion in the lower thoracic or lumbar spine.
A long list of associated features includes torticollis, facial asymmetry, scoliosis and kyphosis; skull malformations, micrognathia, cleft lip and palate, paraesthesia, spastic quadriplegia, synkinesis, ataxia, anaesthesia, posterior fossae dermoid cysts, difficulty in breathing and swallowing, mental retardation, deafness, strabismus, nystagmus, Sprengel’s deformity, torticollis, Cardiovascular, Genitourinary abnormalities and limb deficiencies and various muscle abnormalities.2,4-10
Females are prevalently affected (65%). The aetiology is unknown. It could be an autosomal dominant or recessive disorder with various degree of penetrance.

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Case Report
A 20 year old, 40 kg female presented for correction of severe bilateral Temporo Mandibular Joint Ankylosis. She gave history of trauma to the chin and scalp at the age of 3 years. There was avulsion of maxillary anterior teeth and bone fragments following the trauma. Following this episode, the mouth opening gradually decreased to approximately 5 mm in the premolar region. No treatment for the same condition was taken before.
As she grew up, her parents noticed obvious deformities of the back and mandibular retrognathia. She often complained of food lodgement in the pre-maxillary region with frequent nasal regurgitation, but presently the patient was asymptomatic.
The patient gave medical history of tuberculous cervical lymphadenitis at the age of 6 years on the left side. She had undergone surgical treatment for the same and was on AKT regimen for nine months. She had history of blood transfusion at the time of surgery.
Patient also gave history of polio 14 years ago with mild weakness of the lower limbs.
She gave history of snoring during sleep and nocturia for which she was getting up 5-6 times at night. The sleep studies were reportedly normal. Patient gave history of recurrent rhinitis for which she was under treatment.
Family history was suggestive of tuberculous infection to the father 4 years ago.
On examination, She had a short stature, short neck, cervical spine kyphosis, thoracic lordosis with mild scoliosis. The face was asymmetrical with flattening of right body of mandible. Antigonial notch was accentuated on the left side of mandible. The mouth opening was restricted to 5 mm in the premolar region and the mandible was retrognathic. TMJ movements were absent on L side and reduced on the right side. Deviation of the mouth towards left was present while opening. Scar marks were present on the left side of neck and left forehead.
Neck movements, especially extension was restricted (grade III/IV).15 She underwent a thorough evaluation and was found to have, a low posterior hairline and fusion of C3 – C4 vertebrae on X-ray cervical spine. There were other multiple vertebral anomalies and Kyphoscoliosis of Thoracic vertebrae (5 segments). The CT mandible showed Bony ankylosis of left TMJ with sclerosis of the neck of the condylar process. Shortening of the ramus of mandible and coronoid processes were prominent and highly placed. Nasal septal deviation with defect in the cartilaginous portion of the septum was evident.
A diagnosis of Klippel Feil syndrome was made. Systemic examination revealed no abnormality. Preoperative blood investigations especially BUN, S. Creatinine and S. Electrolytes were normal. Abdominal USG showed no abnormality. We, therefore, did not perform an IVP. X-ray chest, ECG and 2D-ECHO were normal. Cephalometric X-ray analysis showed a posterior airway space of 3 mm, effective mandibular length of 82 mm and ANB angle of 15 degrees (normal being 2 degrees).16 Preoperative PFT suggested variable extrathoracic upper airway obstruction. ABG (on air) showed pH - 7.39, pCO2 - 41.9, pO2 - 98 and saturation - 97%. Limited sleep study (minipolysomnography) showed apnoea hypopnoea index (AHI) of 5 and lowest O2 saturation ( LSAT ) of 92%. IDL could not be performed due to restricted mouth opening. Preoperative nasal endoscopy revealed a more patent right nostril.

Anaesthetic Management
We planned an awake nasal intubation using a fibreoptic bronchoscope. The patient was explained about the procedure and consent was obtained. Jet ventilation, Cricothyroidotomy and Tracheostomy were kept ready. No sedative premedication was administered.
Xylometazoline drops were instilled into the right nostril. Bilateral superior laryngeal nerve block was performed using 2 ml of 2% adrenalized lignocaine and 2 ml of 4% lignocaine was injected transtracheally. The oropharynx was sprayed with 4% lignocaine spray.
Fibreoptic intubation was done using 7 no. lubricated Portex endotracheal tube through the right nostril. Endotracheal position of the tube was further confirmed using capnometry and bilateral chest auscultation. The patient was then anaesthetized with Propofol and paralysed with Vecuronium bromide and maintained on Nitrous Oxide and Oxygen on controlled ventilation using closed circuit with circle absorber. A Propofol infusion was given at 3 mg/kg to maintain the depth of anaesthesia.
Mandibular advancement and an advancement genioplasty were done using bilateral mandibular osteotomy and placement of intraoral bilateral distraction appliances. The patient maintained stable haemodynamics and blood loss was insignificant.
At the end of surgery, residual neuromuscular block was reversed with neostigmine and atropine. Adequacy of reversal was confirmed using Double Burst Stimulation on the peripheral nerve stimulator. ABG done post operatively was normal. After confirming that the patient was maintaining normal EtCO2 and pulse oximetry values, she was shifted to the postoperative recovery room with the endotracheal tube in place and was administered oxygen using a T piece. She was extubated 24 hours later. Post extubation, the patient was carefully monitored in the recovery room for periods of apnoea and obstructed breathing. Sleep studies were not required post operatively since patient showed marked improvement clinically with no snoring during sleep and the patient had an uneventful postoperative course.

Discussion
Our patient had Type II fusion, and a grossly abnormal appearance which led us to the diagnosis of Klippel Feil syndrome. Associated congenital anomalies should be evaluated preoperatively in all patients.
Mandibular hypoplasia occurs in a few patients with Klippel Feil syndrome and can contribute to upper airway obstruction and result in obstructive sleep apnoea (OSA)11 syndrome. Patients typically give a history of restless sleep and snoring. Patients with a suspected breathing sleep disorder can be evaluated by polysomnography in a sleep laboratory. Surgical treatment is indicated in patients with moderate to severe OSA.12 Our patient had borderline OSA and had come only for cosmetic correction. Cephalometric X-ray analysis19 is useful to examine the possibility of an airway impairment. Posterior airway space (measured from a point on the posterior outline of the base of the tongue to the closest point on the posterior pharyngeal wall) < 10 mm is an indicator of possible airway impairment. Patients with OSA are very sensitive to all central depressant drugs with upper airway obstruction or respiratory arrest occurring even with minimal doses. Sedative and opioid premedication should be used cautiously. Perioperative monitoring for apnoea, desaturation and dysrhythmias is essential. These patients have a potentially difficult airway and awake intubation as well as extubation is the safest approach to airway control.
Patients with Klippel Feil syndrome have an increased susceptibility to cervical osteoarthritis and trauma and are at high risk for spinal cord injury during manouvres such as laryngoscopy, tracheal intubation and operative positioning.3,13 An awake fibreoptic guided intubation is ideal in such patients.

Conclusion
In a patient with Klippel Feil syndrome difficult intubation is anticipated due to limited neck mobility and distorted anatomy due to micrognathia. There is also a risk of neurological injury during laryngoscopy and tracheal intubation. Endotracheal intubation is therefore best done using a fibreoptic bronchoscope or a blind, awake technique avoiding laryngoscopy.
Micrognathia can cause upper airway obstruction leading to OSA syndrome. These patients are sensitive to central depressant drugs. They should be carefully monitored post operatively for periods of apnoea or dysrhythmia in a high dependency area.

References
1. Daum REO, Jones DJ. Fibreoptic intubation in Klippel Feil syndrome. Anesthesia 1988; 43 : 18– 22.
2. Nagashima H, Morio Y, Teshima R. No neurological involvement for more than 40 years in Klippel Feil syndrome with severe hypermobility of the upper cervical spine. Archives of Orthopaedic and Trauma Surgery 2001; 121 (1-2) : 99-101.
3. Elster AD. Quadriplegia after minor trauma in the Klippel Feil syndrome. J Bone Joint Surg 1984; 66A : 1473–74.
4. Naguib M, Maxwell RE, Chou SN. Identification and management of high risk patients with Klippel Feil syndrome. J Neuro Surg 1984; 61: 523–30.
5. Aksoy FG, Aksoy OG, Gomori JM. Klippel Feil syndrome in association with posterior fossa sub occipital dermoid cyst. European Radiology 2001.; 11 (1) : 142-44.
6. Helmi C, Pruzansky S. Cranifacial and extracranial malformations in the Klippel Feil syndrome. Cleft Palate J 1980; 17 : 65-88.
7. Roa NL, Moss KS. Treacher Collins syndrome with sleep apnoea – Anesthetic considerations. Anesthesiology 1984; 60 : 7–83.
8. Moore WB, Matthews TJ, Rabinowitz R. Genitourinary anomalies associated with Klippel Feil syndrome. J Bone and Joint Surg 1975; 57A : 355–57.
9. Baum V, O’Flaherty J. Anesthesia for genetic, metabolic and dysmorphic syndromes of childhood. Edn. 1999: pp 164–5.
10. Thomsen M, Krober M, Schneider U, Carstens C. Congenital Limb deficiencies associated with Klippel Feil syndrome: a survey of 57 subjects. Acta Orthopaedica Scandinavica 2000; 71 (5) : 461-64.
11. Boushra N. Anesthetic management of patients with sleep apnea syndrome. Can J Anesth 1996; 43 (6) : 599–616.
12. Millman RP, Carlisle CC, Rosenberg C, Kahn D, McRae R, Kramer N. Simple predictors of Uvulopalatopharyngoplasty outcome in the treatment of Obstructive Sleep Apnea. Chest 2000; 118 (4) : 1025-30.
13. Naguib M, Farag H, Ibrahim AE. Anesthetic considerations in Klippel Feil syndrome. Can Anesth Soc J 1986; 33 : 66–70.
 

VALVULAR HEART DISEASES IN ELDERLY PEOPLE
The continuous decrease in the incidence of rheumatic fever during the second half of the last century led to a decrease in the prevalence of rheumatic heart diseases. The shift from rheumatic towards degenerative causes accounts for the sustained prevalence of valvular heart diseases and changes in patients’ characteristics.
The increase in prevalence with age is inherent to the definition of degenerative diseases. However, the term degenerative is not appropriate in valvular heart diseases. In particular, there are large differences between degenerative aortic stenosis and degenerative regurgitations. Findings consistently show that aortic stenosis shares many common features with atherosclerosis, in terms of histological lesions and common risk factors. However, myxomatous mitral valves causing degenerative mitral regurgitation are characterised by different valvular remodelling.
Most frequent non-cardiac comorbidity was chronic obstructive pulmonary disease in 15% of the patients. In patients with severe symptomatic aortic stenosis aged over 75 years, as many as 44% had at least one non-cardiac comorbidity.

The Lancet, 2006; 368 : 969-70.
 
 
 
 
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