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CASE REPORT
Year : 2008  |  Volume : 52  |  Issue : 5  |  Page : 577 Table of Contents     

Osteogenesis Imperfecta:No Place for Imperfect Anaesthesiologist


1 Assistant Professor Anaesthesiology, U.F.H.T. Medical College (Haldwani-Nainital), India
2 Assistant Professor Surgery, U.F.H.T. Medical College (Haldwani-Nainital), India
3 Professor, Anaesthesiology, LNH and MAMC New Delhi, India
4 Associate Professor, Anaesthesiology, LNH and MAMC New Delhi, India
5 P.G Student, LNH and MAMC New Delhi, India
6 Specialist, LNH and MAMC New Delhi, India

Date of Acceptance12-Jul-2008
Date of Web Publication19-Mar-2010

Correspondence Address:
Geeta Bhandari
Asstt. Prof.,Department of Anaesthesiolgy And Intensive Care, Kamal Kunj, Rampur Road, North ManPur, Haldwani-Nainital (U.K.) Pin-263139
India
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Source of Support: None, Conflict of Interest: None


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Osteogenesis imperfecta, an inherited disease of connective tissue, is associated with anatomic and physiologic abnormalities which make any form of anaesthesia a challenging task for the anaesthesiologist. We report a case of Osteogenesis imperfecta type -IV with severe anatomic deformities, who underwent replacement nailing procedure for periprosthetic fracture of shaft femur under general anaesthesia. We used a proseal LMA in the case, patient suffered a posterior dislocation of right shoulder on repositioning at the end of the surgery.

Keywords: Osteogenesis imperfecta; Proseal LMA; General anaesthesia;


How to cite this article:
Bhandari G, Shahi K S, Bhadoria P, Bhalotra AR, Sandhya O D, Arya M. Osteogenesis Imperfecta:No Place for Imperfect Anaesthesiologist. Indian J Anaesth 2008;52:577

How to cite this URL:
Bhandari G, Shahi K S, Bhadoria P, Bhalotra AR, Sandhya O D, Arya M. Osteogenesis Imperfecta:No Place for Imperfect Anaesthesiologist. Indian J Anaesth [serial online] 2008 [cited 2020 Jan 23];52:577. Available from: http://www.ijaweb.org/text.asp?2008/52/5/577/60679


   Introduction Top


Osteogenesis imperfecta is an inherited disease of connective tissue that affects bone, sclera and the inner ear, caused by mutations of the Collagen type 1­COLIA1 or COLIA2 genes [1] .

Osteogenesis imperfecta classically divided into two syndromes: the congenita form, which has a high infant mortality rate and the tarda form, which is asso­ciated with a normal life expectancy.

According to Sillence classification disease has been classified into four distinct types [2] . Type -II and Type -III are autosomally recessive; Type -II is perinatally fatal and subjects affected with Type -III usually die in childhood from severe kyphoscoliosis. Type -I and Type-IV , both autosomally dominant, are characterized by short stature, bone fragility leading to frequent fractures and dentinogenesis resulting in easily broken teeth. The fractures in type -I, the most com­mon of the four disorders are generally non-deforming, while fractures in type-IV tend to cause deformities of long bones and thoracic cage. Type-I is characterized also by the presence of distinct blue sclera. [3]

The defect in skeletal growth is a result of lack of normal ossification of endochondrial bone resulting in increased fragility of bones. These patients usually have history of recurrent fracture of bones; present with hypermobile limbs and other associated skeletal de­formities like kyphoscoliosis, short neck, pigeon chest with difficult airway and risk of odonto-axial dislocation, cervical vertebra, mandible and teeth fractures during laryngoscopy and intubation [4] .

The disease may cause cardiac valvular lesions, cor-pulmonale, neurologic abnormalities, hyperhydrosis, cleft palate, metabolic abnormalities, malignant and non malignant hyperthermia and obstructive uropathy fol­lowing renal and ureteric stones and platelet dysfunction [5],[6],[7],[8],[9],[10] .

We hereby share an experience of anaesthetic management of a known case of osteogenesis imperfecta tarda type IV, who presented with postprosthetic fracture shaft femur& under went a replacement nail­ing procedure.


   Case report Top


In the pre anaesthetic checkup clinic, an 18-year­old male presented with fracture shaft left femur with intra medullary nail in situ, planned for nail replacement procedure. He was a known case of osteogenesis imperfecta tarda type IV with characteristic features of short stature, brittle bones, hypermobile joints, ky­phoscoliosis with history of recurrent fractures of long bones for which he was operated twice under general anaesthesia, uneventfully. He also had history of lumbar disc prolapse without any neurological involvement, three years back. There was no history of osteogen­esis imperfecta in the family.

On general examination he was short statured, (105 cm), 40 kg , afebrile with normal coloured sclera. His pulse and blood pressure were within normal limit. Respiratory system revealed barrel shaped chest with bilateral equal air entry. On airway assessment he had acceptable flexion and extension at neck with adequate mouth opening and normal dentition. Airway assess­ment was of Mallampatti class II. Kyphoscoliosis was seen on examination of spine. Other systems were within normal limits.

Routine haematological investigations including coagulation profile were normal. Thyroid function test, liver function test, renal function test and creatine ki­nase were normal. Electrocardiogram and Echocar­diogram were normal. Chest radiogram revealed thin gracile ribs with normal cardiac shadow. X- ray spine revealed dorsolumber kyphoscoliosis to left with prolapsed inter vertebral disc of L4-5 vertebrae.Pulmonary function tests (PFT) were sug­gestive of mildly restrictive lung disease.

Patient was accepted for surgery with ASA grade II. In the operating room, the patient was carefully placed in the supine position, routine monitors were then applied (ECG, pulse oximeter, skin temperature probe). Blood pressure was measured manually . In­travenous line secured in left hand with 18 G canula. Total two liters of Ringer lactate and half litre of DNS was given after prewarming to body temperature.

Patient received premedication with pethidine 40 mg and preoxygenated with 100 % O 2 for three min­utes. Anaesthesia was induced with propofol 120 mg and ability to mask ventilation was assessed before administering vecuronium 4 mg. Then IPPV was done for three minutes and proseal laryngeal mask airway # 4 was placed in cervical neutral position and ventila­tion was followed with Bains' circuit on intermittent positive pressure ventilation mode. EtCO 2 monitoring was in place throughout the perioperative period.

Anaesthesia was maintained using intermittent vecuronium with N 2 O& O 2 in 2:1. For analgesia inter­mittent doses of pethidine (10 mg after every 30 min­utes) were used. Right lateral position was required for the planned surgery. Meticulous care was taken when the patient was being shifted to right lateral position in the form of appropriate padding on pressure areas with preformed cushions& cotton pads.

Intraoperative period of 2 hours remained un­eventful. At the end of the surgery, patient was reposi­tioned to supine and reversal of anaesthesia was car­ried out using 100% O 2 with neostigmine 2.0 mg and glycopyrrolate 0.4 mg. Proseal LMA was removed after resumption of regular spontaneous respiration, but at a deeper plane of anaesthesia to prevent return of excessive muscle tone.

However, our patient started complaining of se­vere agonizing pain in the right shoulder joint immedi­ately on return of consciousness and orientation in the operation theatre. On examination patient was diag­nosed to have suffered a posterior dislocation of the right shoulder joint. Subsequent management of the dis­location was done under propofol infusion with pethi­dine 25 mg intravenous and closed reduction was checked with direct imaging. Postoperative period was uneventful and patient was subsequently discharged on the seventh postoperative day.


   Discussion Top


Although our patient received general anaesthe­sia, the best anaesthetic technique in patients with Os­teogenesis imperfecta is conduction block (regional anaesthesia). Firstly, it avoids the necessity for tra­cheal intubation (laryngoscopy and tracheal intubation associated with a risk of odontoaxial dislocation, frac­ture mandible, cervical vertebrae and injury to teeth in patients with osteogenesis imperfecta). Secondly, con­duction block makes the development of hyperthermia less likely as compared to general anaesthesia (as malignant hyperthermia is the result of either an abnor­mal central nervous system temperature regulating mechanism or abnormal cellular energy metabolism). Lastly, it facilitates the detection of thyroid storm (increased serum thyroxin concentrations associated with increased oxygen consumption occur in at least 50% of patients with disease) [4] .

In our case anatomic deformity was much severe than physiologic abnormality. Presence of dorsolumbar kyphoscoliosis with prolapsed lumbar vertebrae made the general anaesthesia the choice to be opted. Ky­phoscoliosis can predispose these patients to inadvert­ent dural puncture and coupled with short stature, may make it difficult to predict the level of any block pro­duced by a given dose of local anaesthetic [11] . Though the patient had kyphoscoloisis with barrel shaped chest but the pulmonary function tests were suggestive of only mildly restrictive lung disease. Therefore, we preferred the use of supraglottic device, proseal LMA in cervical neutral position to avoid the fracture or dislocation during ex­tension/hyperextension at neck and put on intermittent positive pressure ventilation, as the case was elective one and required right lateral position. Though Karabiyk et al have recommended TIVA along with intubating LMA to manage the elective case [11] . Sachin et al noted a significant degree of movement between first and sec­ond cervical vertebrae (odontoaxial ) during direct laryn­goscopy and with the use of intubating LMA [12] .

Due to abnormal skeletal growth, short stature and hypermobile joints, difficult airway must always be anticipated in such patients [13] . Therefore, we were ready with difficult airway kit (including ILMA and fibreoptic device).

The use of inhalation agents eg. halothane or isoflurane, would have been considered but the fact that these patients are susceptible to develop malignant hyperthermia made us to avoid it. Therefore, we also avoided the use of atropine and suxamethonium and continuously monitored the skin temperature. suxame­thonium induced fasciculations may cause fractures, as may hyperextension of neck and risk to trigger the malignant hyperthermia, hence it was avoided in this case [8],[14] .An automated arterial pressure cuff may be hazardous,as overinflation can result in a fracture, there­fore, we used a manual sphygmomanometer [15] .

The bleeding may occur despite normal results of coagulation studies and bleeding times, making predic­tions about intraoperative bleeding difficult [16] . Coag­ulopathy with sudden development of widespread pete­chiae has also been reported [17] . Therefore, due pre­cautions regarding any unexpected bleeding were taken in the form of availability of adequate blood, fresh fro­zen plasma and platelet concentrates.

Despite all the necessary precautions being taken to prevent any potential complications, we faced a problem in the form of a right shoulder dislocation (pos­terior) at the end of surgery after an uneventful intraop­erative period . Malde et al has reported fracture of right shaft of femur in his patient, which occurred dur­ing transfer to the recovery room [13] .

In our opinion, extra caution needs to be taken in patients of osteogenesis imperfecta undergoing opera­tion in the lateral position and under general anaesthesia since the combination of muscle relaxants and the possi­bility of overlying weight of the upper body on the de­pendent shoulder in this patient further increased the chances of dislocation as well as fracture in view of their already lax joints and brittle bone. Further, the possibility of such problems remaining undetected in the uncon­scious, anaesthetitized patient should be borne in mind. Standard auxiliary rules may be custom made for a pa­tient in the lateral position, prior to induction of anaesthesia. Repeated intra operative checks of pulses will also help in excluding or detecting occurrence of any such events in the intraoperative period. After recovery from muscle relaxants with the analgesia and sedation any underlying problem associated with position e.g. pres­sure on neurovascular bundle may lead to neuropraxia which may remain undetected for a longer period and thus patient may have consequent damage resulting in lawsuits in consumer court for the damages.

Regional anaesthesia is the technique of choice in such cases, but when general anaesthesia is consid­ered in view of proposed surgical procedure or due to relative contra indication of regional block, as in this case, meticulous attention is required especially with the use of neuromuscular blocking agents, inhalational agents, airway management, positioning of the patient and acute pain management.

 
   References Top

1.Marini JC. Osteogenesis imperfecta-managing brittle bones. N Engl J Med 1998:339:986-7.  Back to cited text no. 1      
2.Sillence D.Osteogenesis imperfecta: an expanding pan­orama of variants, Clinical Orthopaedics and Related Research 1981;159:11-25.  Back to cited text no. 2      
3.Katz J, Benumof J, Kadis L. Anesthesia and Uncommon Diseases: Pathophysiologic and Clinical Correlations, 2 nd Edn. Philadephia: W.B. Saunders 1981;577-578.  Back to cited text no. 3      
4.Colvin MP, Wilkinson K. Patient Position. In: Taylor TH, Major E, eds. Hazards and complications of anaesthesia. Edinburgh: Churchhill Livingstone, Inc 1993:535-60.  Back to cited text no. 4      
5.Wood SJ, Thomas J, Brainbridge MV, et al. Mitral valve disease and open heart surgery in Osteogenesis Imperfecta tarda: Report of a case. Br Heart J 1973;31:03-6.  Back to cited text no. 5      
6.Heppner RL, Babitt HI, Bianchine JW, Warbasse JR. Aortic regurgiatationa and aneurysm of sinus of valsalva associated with osteognesis imperfecta. Am J cardiol1973;31:654-6.  Back to cited text no. 6  [PUBMED]    
7.Cropp GJA, Myers DN. Physioligical evidence of hy­permetabolism in Osteogenesis Imperfecta. Pediatrics 1972;49:375-91.  Back to cited text no. 7      
8.Porsberg P, Astrup G, Bendixen D, Lund AM, Ording H. Osteogenesis Imperfecta and malignant hyperthermia. Is there a relationship. Anaesthesia 1996;51:863-65.  Back to cited text no. 8      
9.Venugopala D,BabuS, Korath MP, Jagadeesan K. Renal stone disease as extra skeletal manifestation of osteogenesia imperfecta. J Assoc physicians India 2000;48:1027-28.  Back to cited text no. 9      
10.Glosten B. Osteogenesis imperfecta. In: Gambling, DR, Douglas MJ, eds. Obstetric anaesthesia and uncom­mon disorders. Philadelphia: WB Saunders 1998:213-8.  Back to cited text no. 10      
11.Karabiyik L, Parpucu M, Kurtipek O. Total intravenous anaesthesia and the use of an intubating laryngeal Mask in a patient with osteogenesis imperfecta. Acta Anaesthesiol Scand 2002;46:618-19.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]  
12.Sachin A, Salman MA, Erden IA, Aypar U. Upper cervi­cal vertebral movement during intubating laryngeal mask, fibreoptic and direct laryngeoscopy :A video fluo­roscopic study. Eur J Anaesthesiol 2004;21:819-23.  Back to cited text no. 12      
13.Malade AD,Jagtap SR, Pantvaidy SH, Kenkare JS. Os­teogenesis Imperfecta:Anaesthetic management of a patient for abdominal hysterectomy ( a case report). In­dian J Anaesth 1993;41:203-06.  Back to cited text no. 13      
14.Kostopanagiotou G, Coussi T, Tsaroucha N,Voros D. Ana­esthesia using a laryngeal mask airway in a patient with Osteogenesis Imperfecta. Anaesthesia 2000; 55;489-518.  Back to cited text no. 14      
15.Libman R. Anesthetic considerations for the patient with osteogenesis imperfecta. Clinical orthopaedics and Re­lated Research 1981;159:123-125.  Back to cited text no. 15      
16.Wong RS. Follis FM, shively BK, Wernly JA. Osteo­genesis imperfecta and cardiovascular diseases. Ann Thorac Surg 1995;60: 1439-43.  Back to cited text no. 16      
17.Edge G, Okafor B, Fennelly ME, Ransford AO. An un­usual manifestation of bleeding diathesis in a patient with osteogenesis imperfecta . Eur J Anaesthesiol 1997;14:215-9.  Back to cited text no. 17  [PUBMED]    




 

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