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Nishkarsh Gupta

1Department the Onco.Anaesthesia and also Palliative Medicine, Dr. BRAIRCH, AIIMS, brand-new Delhi, India


Address because that correspondence: Dr. Anju Gupta, A-437, Sarita Vihar, brand-new Delhi - 110 076, India. E-mail: moc.liamffider
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You are watching: Fibrodysplasia ossificans progressiva stone man syndrome


Stone male syndrome or fibrodysplasia ossificans progressiva (FOP) is an extremely rare (1 in 2 million) hereditary disorder qualified by ectopic ossification the the skeletal and connective tissues leading to progressive fusion of axial and also appendicular skeleton. Surgery and anaesthesia-induced trauma have the right to lead to condition flare-up if due precautions are not taken and also disable the patience further. However, rarity that the an illness may cause its common misdiagnosis and also anaesthesiologist might be captured unaware. Over there is relative paucity that literature about anaesthetic administration of children with FOP. Videolaryngoscopes (VLs) carry out a non-line-of-sight view and also require less anterior force to visualise the glottis, may carry out an alternate to fibreoptic intubation for airway administration in together cases. Use of VL has only to be reported when in one adult through FOP because that nasotracheal intubation. We explain the successful anaesthetic management of one 11-year-old son with FOP and also anticipated challenging airway.


Keywords: Fibreoptic intubation, fibrodysplasia ossificans progressiva, heterotopic ossification, videolaryngoscope

INTRODUCTION

Fibrodysplasia ossificans progressiva (FOP), additionally known as rock man syndrome, is a severely disabling and catastrophic-inherited disorder of connective tissue characterised by congenital malformation that the an excellent toes, thumbs and also vertebrae connected with gradual ossification of striated muscles.<1,2> In such patients, progressive fusion of axial and also appendicular skeleton, temporomandibular joint (TMJ) ankylosis, connected restrictive lung disease and sensitivity to also trivial oral trauma make airway administration challenging.<1,2,3,4>


CASE REPORT

An 11-year-old, 30 kg male child, suspected to it is in a situation of cysticercosis, presented through multiple hard swellings end the nape that neck, paraspinal region, arms, thighs and also legs. He was posted because that excision biopsy of the ago swellings. He had received antitubercular treatment and also albendazole through no remission. Pre-operative airway review revealed a Mallampati class III, minimal neck motion (45°) and mouth opening (MO) the 2.5 cm. Cardio-respiratory examination and also all regimen investigations including chest X-ray and also electrocardiogram (ECG) were discovered to it is in normal. In the operation room, ECG, non-invasive blood pressure, finish tidal carbon dioxide and also pulse oximeter were attached. Intravenous (IV) accessibility was secured and injection fentanyl 60 µg was given. The just fibreoptic bronchoscope (FOB) in the room was no working. Hence, Truview PCD™ video laryngoscope (Truview video clip laryngoscope , Netanya, Israel)-guided intubation to be planned. Difficult airway dare was preserved ready. Anaesthesia to be induced with sevoflurane 5%–8% (1.5 minimum alveolar concentration) in oxygen and supplemented v propofol 30 mg. Throughout laryngoscopy through TVL, a Cormack and Lehane (CL) class 3 the glottis was obtained which improved to CL 2b through optimum external laryngeal manipulation. A pipe introducer could be placed into the glottis, and a size 6.0 flexo metallic endotracheal tube was threaded end it. Thereafter, muscle be safe was completed with injection atracurium 10 mg, and the patient was positioned susceptible for surgery. Adequate padding was applied to eyes, bony prominences, deformities of upper limbs (shoulder and also elbow) and also swellings of reduced limbs. During excision biopsy, the operated doctor reported bony tissue in the nodules and also got suspiciously of FOP. The surgical procedure was abandoned, the patient was made supine and his trachea was extubated after ~ reversal of neuromuscular blockade. The patient to be shifted come post-operative room for close observation. Injection diclofenac salt 50 mg was given as sluggish IV infusion because that post-operative pain. Succeeding histopathology of the biopsy tissue revealed tires osseous tissue, audiometry proved conductive hearing loss and also skeletal inspection revealed ectopic bone formation over the back, neck, upper limbs and also lower limbs, therefore confirming the diagnosis the FOP .2>. The patient was discharged home 2 work later.


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Classical indicators of fibrodysplasia ossificans progressiva in ours patient: great toe malformation (a) and extensive heterotopic ossification top top the back (b)


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Figure 2

Head and also neck X-ray with straight ossification the the paraspinal muscles


DISCUSSION

FOP (myositis ossificans progressiva) is autosomal dominant connective tissue disorder characterised by extra skeletal endochondral ossification. It might be associated with mutation in bone morphogenic protein4 (BMP4) antagonist gene which leader to increased production of BMP.<2> the is qualified by progressive heterotopic bone formation in the connective tissue and skeletal muscle. The ossification normally starts in the neck, spine and also shoulder girdle and progressively immobilises every the joints of the axial and also appendicular skeleton throughout the an initial decade the life.<1,2> Conductive hear loss as result of ossification of bones of interior ear is additionally common.<2> boy trauma, biopsy, surgical intervention, etc., may flare increase the disease.<3> Hence, one have to avoid deep intramuscular (IM) injections, injections right into jaws for dental procedures, invasive biopsy, excision measures for heterotopic masses and also manipulations of stiff joints come prevent condition progression.<2,3,4> this patients have actually a typical life span of 40 years and also usually die because of complications of thoracic insufficiency syndrome or pneumonia.<5>

Anaesthetic administration of these patients is complicated due to anticipated an obstacle in airway management (cervical spine fusion and TMJ ankylosis), border lung an illness and their too much sensitivity to trauma.<6,7,8> The typical interventions during anaesthesia such together traumatic IV and also arterial cannulation, im injections, overstretching that joints during positioning and regional blocks have the right to precipitate iatrogenic heterotopic ossification and any such treatment should it is in avoided.<3,4,6>

In at an early stage stages, patients may not have any kind of symptom, and since it is a rarely entity, the diagnosis might be missed.<3> This patient had bony difficult swellings with minimal movements and also was misdiagnosed as calcified muscular cysticercosis initially. Throughout surgery when hard bone-like tissue was uncovered in the nodule throughout dissection, FOP was suspected. Subsequently, a diagnosis of FOP was shown on the communication of two timeless features, specific congenital good toe malformations and heterotopic ossification in certain anatomic fads 2> and mature bone organization on histopathology the the biopsy tissue.

Difficult airway and cannot ventilate cannot intubate instance needing emergency tracheostomy have actually been reported in FOP.<9,10,11,12> straight laryngoscopy and manoeuvres-like jaw thrust may cause ossification and ankylosis due to overstretching that TMJ.<1,2> Hence, overstretching that the oropharyngeal structures must be prevented by staying clear of jaw thrust and ensuring that the MO during laryngoscopy and also intubation stays well listed below the base heat values acquired prior to the anaesthetic.<9,12> awake fibreoptic intubation is taken into consideration the gold standard even in patient with adequate MO and jaw activity to avoid any kind of stimulus to TMJ.<7,8,9,10> to prepare the airway for awake intubation, nebulisation, spray as you walk or straight instillation of local anaesthetic is preferred over airway blocks. Elective tracheostomy should additionally be avoided as ossification that the incision website may result in airway obstruction ~ above decannulation. Our patient had actually a potential difficult airway (restricted MO and also neck movements), to be uncooperative and also the just FOB in ours setup was no working. Anterior larynx with decreased MO invariably requires extreme force throughout conventional laryngoscopy, which might be devastating in a patient through FOP.<13,14,15>

Successful use of Glidescope™ has been stated in one case for nasotracheal intubation of an adult FOP patience (having a common MO) in a case collection of 30 patients, however use the VL has never been explained for a child.<9>


CONCLUSION

Anaesthetic administration for FOP is very challenging, and clinical interventions that might put patient at a threat of brand-new localisation the heterotopic ossification causing substantial influence on their top quality of life need to be diligently avoided. VL assisted with bougie may be considered as an alternative to FOB because that airway management of these patients.


Financial support and sponsorship

Nil.

See more: Father Letter To Son In Law, A Letter To My Eventual Son In Law


Conflicts that interest

There room no conflicts of interest.


REFERENCES


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