|LETTER TO EDITOR
|Year : 2018 | Volume
| Issue : 2 | Page : 89-90
Laurence–Moon–Biedl–Bardet Syndrome: Its significance to anesthesiologist
Priyanka Sethi1, Pradeep Bhatia1, Narender Kaloria2, Ankur Sharma1
1 Department of Anaesthesiology and Critical Care, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
2 Department of Neonatology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
|Date of Web Publication||15-Nov-2018|
Dr. Priyanka Sethi
Department of Anaesthesiology and Critical Care, All India Institute of Medical Sciences, Jodhpur - 3420 005, Rajasthan
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sethi P, Bhatia P, Kaloria N, Sharma A. Laurence–Moon–Biedl–Bardet Syndrome: Its significance to anesthesiologist. Indian Anaesth Forum 2018;19:89-90
|How to cite this URL:|
Sethi P, Bhatia P, Kaloria N, Sharma A. Laurence–Moon–Biedl–Bardet Syndrome: Its significance to anesthesiologist. Indian Anaesth Forum [serial online] 2018 [cited 2020 Jul 9];19:89-90. Available from: http://www.theiaforum.org/text.asp?2018/19/2/89/245555
Laurence–Moon–Biedl–Bardet Syndrome (LMBBS) is a rare autosomal recessive genetic disorder with multisystem involvement characterized by obesity, polydactyly, retinitis pigmentosa, mental retardation, genital hypoplasia, cardiac anomalies, and renal dysfunction., There is a paucity of literature on the anesthetic management of patients with this rare syndrome. We report anesthetic management of a 3-year-old child with LMBBS scheduled for bilateral orchidopexy [Figure 1]. During preanesthetic evaluation, the child was found to be obese (body weight = 25 kg and body mass index >30 kg/m2) with polydactyly of both upper and lower limbs. He also had cognitive delay and underdeveloped external genitalia. There was a history of decreased vision with myopic spectacles of power 10 with astigmatism on both eyes and also had retinitis pigmentosa changes on fundoscopic examination. His peripheral veins were not visible, and on airway examination, he had short fatty neck along with small mouth opening and large tongue (Modified Mallampati Grade 3). All other systems were normal. Premedication was given with oral midazolam 10 mg under direct supervision. In operation theater, all routine monitors were connected, and difficult airway cart was kept ready. The child was induced with oxygen and sevoflurane while maintaining spontaneous ventilation and intravenous line was secured with 22-G cannula on the dorsum of the left hand. Intravenous fentanyl 50 μg and atracurium 12 mg were given after checking for adequacy of mask ventilation, and his airway was secured with laryngeal mask airway (LMA) no 2. Anesthesia was maintained with sevoflurane and atracurium intermittent top up while ventilating him on pressure control mode. The surgery lasted for about an hour, and neuromuscular blockade was reversed after completion of surgery. LMA was removed after the patient became fully conscious. Postoperatively, the patient was observed for about 2 h and then shifted to ward. Both intra- and post-operative periods were uneventful. The patient was discharged 2 days after the surgery.
|Figure 1: Child with Laurence-Moon-Biedl-Bardet Syndrome showing polydactyly|
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The anesthetic consideration associated with the syndrome are due to multisystem involvement, obesity, short neck with difficult venous access, cognitive impairment and increase the risk of arrhythmia due to abnormal autonomic response. We thoroughly evaluated the child for other comorbidities. Normal electrocardiogram and echocardiography ruled out any cardiac involvement. Renal function tests and blood sugar were also normal. Obesity is the cardinal feature of LMBBS present in about 85%–90% of these patients that was also present in our case. We had predicted difficult intubation in our patient since our patient had short thick neck and a modified Mallampati score of 3 but we could manage it with LMA. Since these patients may have cognitive impairment and obesity, they should be premedicated under close observation. Both general and regional anesthesia can be used safely in these patients.,, Renal impairment commonly encountered in these patients, therefore judicious use of intravenous fluid with frequent monitoring of serum electrolytes were desired, and it is also better to avoid nephrotoxic drugs. There are no literature to suggest preferred anesthetic and analgesic agents. These patients need anesthesia for various diagnostic and therapeutic procedures. Although we did not encounter any complication in our case, this article highlights the awareness required among anesthesiologists for possible complications and management of LMBBS.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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