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. 2017 Mar;61(3):266-268.
doi: 10.4103/0019-5049.202168.

Anaesthetic management of a child with stone man syndrome: Look before you leap!

Geeta Kamal  1 , Anju Gupta  1 , Sapna Batla  1 , Nishkarsh Gupta  2
Affiliations

Anaesthetic management of a child with stone man syndrome: Look before you leap!

Geeta Kamal et al. Indian J Anaesth. 2017 Mar.

Abstract

Stone Man syndrome or fibrodysplasia ossificans progressiva (FOP) is an extremely rare (1 in 2 million) genetic disorder characterised by ectopic ossification of the skeletal and connective tissues leading to progressive fusion of axial and appendicular skeleton. Surgery and anaesthesia-induced trauma can lead to disease flare-up if due precautions are not taken and disable the patient further. However, rarity of the disease may lead to its common misdiagnosis and anaesthesiologist may be caught unaware. There is relative paucity of literature regarding anaesthetic management of children with FOP. Videolaryngoscopes (VLs) provide a non-line-of-sight view and require less anterior force to visualise the glottis, may provide an alternative to fibreoptic intubation for airway management in such cases. Use of VL has only been reported once in an adult with FOP for nasotracheal intubation. We describe the successful anaesthetic management of an 11-year-old child with FOP and anticipated difficult airway.

Keywords: Fibreoptic intubation; fibrodysplasia ossificans progressiva; heterotopic ossification; videolaryngoscope.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Classical signs of fibrodysplasia ossificans progressiva in our patient: great toe malformation (a) and extensive heterotopic ossification on the back (b)
Figure 2
Figure 2
Head and neck X-ray with linear ossification of the paraspinal muscles
See this image and copyright information in PMC

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