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LETTER TO EDITOR
Year : 2018  |  Volume : 19  |  Issue : 2  |  Page : 89-90
 

Laurence–Moon–Biedl–Bardet Syndrome: Its significance to anesthesiologist


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 Publication15-Nov-2018

Correspondence Address:
Dr. Priyanka Sethi
Department of Anaesthesiology and Critical Care, All India Institute of Medical Sciences, Jodhpur - 3420 005, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/TheIAForum.TheIAForum_7_18

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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 2019 Oct 22];19:89-90. Available from: http://www.theiaforum.org/text.asp?2018/19/2/89/245555




Sir,

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.[1],[2] 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.[3],[4],[5] 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.[3] 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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Low J, Brown TK. Bardet-Biedl syndrome: Review of anaesthetic problems. Paediatr Anaesth 1992;2:245-8.  Back to cited text no. 1
    
2.
Cronjé L. Bardet-Biedl syndrome: Expect the unexpected, suspect the unsuspected. South Afr J Anaesth Analg 2017;23:129-38.  Back to cited text no. 2
    
3.
Mahajan R, Kumar Batra Y, Kumar S, Kumar Grover V. Anesthetic management of a patient with Bardet-Biedl syndrome and dilated cardiomyopathy. Minerva Anestesiol 2007;73:191-4.  Back to cited text no. 3
    
4.
Ozgun G, Kahveci K, Ornek D, Ayudin G, Doger C, Elmasli MN. Anesthetic management of a patient with laurence moon Biedl Bardet syndrome. J Anesth Clin Res 2011;2:152.  Back to cited text no. 4
    
5.
Bhat MT, Santhosh MC, Hegde HV, Rao RP. Anesthetic management of a child with Bardet-Biedl syndrome undergoing post-auricular dermoid excision. J Anaesthesiol Clin Pharmacol 2014;30:117-8.  Back to cited text no. 5
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