|Year : 2008 | Volume
| Issue : 5 | Page : 577
Osteogenesis Imperfecta:No Place for Imperfect Anaesthesiologist
Geeta Bhandari1, KS Shahi2, Poonam Bhadoria3, Anju R Bhalotra4, OD Sandhya5, Mona Arya6
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 Acceptance||12-Jul-2008|
|Date of Web Publication||19-Mar-2010|
Asstt. Prof.,Department of Anaesthesiolgy And Intensive Care, Kamal Kunj, Rampur Road, North ManPur, Haldwani-Nainital (U.K.) Pin-263139
Source of Support: None, Conflict of Interest: None
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|| |
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  .
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  . 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. 
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  .
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 ,,,,, .
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|| |
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|| |
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)  .
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  . 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  . 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  .
Due to abnormal skeletal growth, short stature and hypermobile joints, difficult airway must always be anticipated in such patients  . 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 , .An automated arterial pressure cuff may be hazardous,as overinflation can result in a fracture, therefore, we used a manual sphygmomanometer  .
The bleeding may occur despite normal results of coagulation studies and bleeding times, making predictions about intraoperative bleeding difficult  . Coagulopathy with sudden development of widespread petechiae has also been reported  . 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  .
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.
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