Pooja Kamat^1*, Govinda Innani²

¹Junior Consultant, ²Speciality Medical Officer, Department of Anaesthesia, Khan Bahadur Bhabha Hospital, Kurla, 400070, Mumbai., INDIA.

Email: kamatpooja102@gmail.com

Figure 1                                             Figure 2

Figure 1: Mallampatti score; Figure 1: Shortened thyromental distance

Our plan A was to give general anaesthesia with Macintosh blade 3 with backup plan of reinforced laryngeal mask airway and emergency tracheostomy as plan B and C respectively in case of failed intubation with plan A. Adequate psychological preparation was insured to patient and parent as well. In operating room, ASA standard monitors were attached. Intramuscular injection of glycopyrrolate 4ug/kg given as antisialogogue. Preoxygenation was performed with 100% O2 for 5 minutes. Injection Fentanyl 2ugs/kg followed by injection propofol 2mg/kg given. Injection rocuronium 1mg/kg given. Intubation was done with Macintosh blade 3, bougie guided, with Cormack Lahane grade IIb using Endotracheal tube number 6. Confirmation of Endotracheal intubation done by capnography. Patient was maintained on oxygen, nitrous oxide and sevoflurane with maintaining on low airway pressure to prevent the risk of pneumothorax. Reversion of neuromuscular blockade was made with 2 mg of Inj. neostigmine and 0.6 mg of Inj. atropine. Patient was extubated after surgery uneventfully. Intraoperative and postoperative vitals were good without complications.

DISCUSSION

The diagnosis of classic Marfan’s syndrome is based on presence of major criteria in at least two organ systems and involvement of a third organ system in absence of positive family history.⁵ Majority of Marfan’s syndrome patients are on beta blocker to slow the aortic dilatation by its negative inotropic and chronotropic effects.⁶ It also has a beneficial effect in mitral valve prolapse (MVP) which is present in 80% patients of Marfan’s syndrome.⁷ In our case, we had mild Aortic regurgitation with tricuspid regurgitation. New diagnostic criteria for Marfans syndrome give more emphasis to aortic root aneurysm and ectopia lentis, with or without family history or positive FBN1 gene test leaving the systemic features on the backburner.⁸ Proper diagnosis is crucial for the appropriate evaluation of the patient and to avoid predictable and potentially fatal complications such as rupture of an aortic aneurysm. On physical examination, one should be alert to signs of congestive heart failure. Echocardiography as indicated to assess the size of the aortic root and valvular function. Pulmonary function tests, with or without arterial blood gas should be considered if thoracic skeletal abnormalities are severe. As is anticipated, patients with Marfan's syndrome present with an increased incidence for ocular, cardio- vascular or musculoakeletal surgery. Major threats to life, severe cardiovascular complications, may occur at any time from infancy to the seventh decade. These complications include dilatation, dissection, or rupture of the aorta and severe regurgitation of the aortic or mitral valve.⁹ Scoliosis is common and may occur at multiple sites along the thoracolumbar spine.¹⁰ Even patients without a skeletal basis for pulmonary dysfunction demonstrate lower forced vital capacities than predicted, presumably as a result of earlier airway closure due to a lack of small airway elastic tissue support.¹¹  Proper positioning and limb support must be assured, considering the ligamentous hyperlaxity and increased risk of joint damage. The anaesthesiologist should be prepared for a potentially difficult intubation owing to factors related with high arch palate, retrognathia and ligamentous hyperlaxity that can lead to joint luxation during neck extension (cervical spine, temporomandibular joint).¹² Excessive traction at laryngoscopy should be avoided to prevent temporomandibular joint dislocation. Intraoperative cardiovascular monitoring is the core concern. Laryngoscopy should be as smooth as possible to prevent hypertension and subsequently increased risk of dissection. Regarding lung function, Marfans syndrome patients normally present with restrictive ventilatory defects, not only because of the underlying emphysema, but also due to the musculoskeletal changes that affect thoracic expansion.¹³ Ventilatory pressures must be kept as low as possible to prevent barotrauma and reduce the risk of pneumothorax. Spontaneous pneumothorax are a frequent occurrence. The potential for the development of a pneumothorax must always be borne in mind during anaesthesia, particularly with positive pressure ventilation. Changes on AV conduction or perioperative dysrhythmias are common small arteries supplying the sinus and AV nodes were especially involved, probably explaining the higher frequency of arrhythmias and conduction disturbances.¹⁴ Tracheomalacia has been reported as a potential complication.¹⁵ As for intraoperative fluid therapy, the primary goal is to maintain blood volume in order to decrease the chances of aortic and/or mitral valve prolapse.¹

CONCLUSION

Pre-existing cardiovascular disease and the potential for acute cardiovascular and respiratory complications in patients with Marfans syndrome demand careful preoperative assessment and the use of skilful anaesthetic technique to avoid fatal complications. Blood pressure control is the central component of perioperative management. The risk of perioperative morbidity and mortality, including unexplained death, is high.¹⁷ Multidisciplinary approach is key. Whatever the anaesthetic technique, the primary consideration is the avoidance of sudden increases in myocardial contractility, producing an increase in aortic wall tension. Beat to beat monitoring by an intra-arterial cannula is helpful, but should be performed carefully in these patients who may be at increased risk from morbidity attributable to weakened arterial walls.

DECLARATION OF PATIENT CONSENT

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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