gorham 's syndrome ( gs ) is characterized by proliferation of vascular channels that results in destruction and resorption of osseous matrix . since its initial description by jackson as a case of massive osteolysis of the humerus in a 12-year - old boy in 1838 and then by gorham and stout in 1955,[24 ] the precise aetiology of the disease is largely unknown .
the osteolytic process can affect any bone , although shoulder and pelvis are commonly affected .
, we report the uneventful perioperative care of a 21-year - old patient with gs , for decompression and fusion of upper thoracic spine .
a 21-year - old , 78 kg male patient presented with pain in the upper back . since the last 1 month
, he was having weakness of both lower limbs leading to inability to walk and sit for a long duration .
past history revealed disappearance of right clavicle between 3 and 12 years of age and , subsequently , he was diagnosed as gs by biopsy of right clavicle . physical examination and vital signs were within normal limits .
magnetic resonance imaging of the spine revealed increasing kyphosis at the upper thoracic level centering at d1 - 2 , and cord compression mainly at the d1 - 2 level .
therefore , he was planned for decompression and fusion of c3  d5 spine . just before shifting to the operating room ( or ) , his pulse rate ( pr ) was 78 beats / min , blood pressure ( bp ) was
he was premedicated with butorphanol 1 mg , promethazine 12.5 mg and glycopyrrolate 0.2 mg intramuscular 45 min prior to surgery .
the patient was shifted to the or and standard monitors were attached and midazolam 1.5 mg was given intravenously ( iv ) , followed by 100% inspired oxygen administration for 5 min .
after loss of consciousness , manual in - line stabilisation ( mils ) of the neck was provided by a second anaesthesiologist , and after confirming adequate mask ventilation , iv rocuronium 50 mg was given following which trachea was intubated with 8.5 mm i d cuffed flexometallic tube .
anaesthesia was maintained with 40% oxygen in nitrous oxide and end - tidal sevoflurane 11.5% using circle system .
intraoperative monitoring included ecg , non - invasive bp , capnometry , pulse oximetry , temperature , urine output , train of four using tof watch and gas analyser .
subsequently , the patient was positioned prone for the surgery over the bolsters , taking great care to avoid eye and bony compression .
posterior decompression and fusion of c3 to d5 and interbody cage insertion at d1 - 2 was done .
the patient received 2 l of lactated ringer solution and blood loss was replaced with tetrastarch ( 130/0.42 ) 500 ml .
the patient was reversed with neostigmine 3 mg and glycopyrrolate 0.5 mg iv , and the trachea was extubated .
then , the patient was shifted to the post - anaesthesia care unit ( pacu ) with oxygen 5 l / min using face mask . in the pacu ,
pain management was done with iv paracetamol 1 g and then 6 hourly , over 15 min , iv ketorolac 30 mg and titrated dose of iv morphine using 2-mg aliquots to maintain visual analogue score ( 0 means no pain , 10-worst pain ) of 3 using syringe pump .
the patient was observed overnight in the pacu and shifted to his room the next day without any complications .
gs or disappearing bone disease , or massive osteolysis , is a mysterious bone disease of unknown aetiology , which affects patients from 1.5 to 72 years , although it occurs most commonly in the 2 and 3 decades .
there is no evidence of any endocrine , metabolic , neoplastic , infectious or neurologic disturbances .
the pathologic process is the replacement of normal bone by an aggressively expanding but non - neoplastic vascular tissue , similar to haemangioma or lymphangioma .
clinical manifestation varies from pain and swelling of the affected extremity , to limitation of motion and weakness of the involved limb .
diagnosis is based on clinical and radiological findings , which is confirmed by biopsy of the bone .
surgical treatment options include resection of the lesion and instrumentation using bone grafts and prostheses .
anaesthesia for major spine surgery , such as spinal stabilization , present a number of challenges .
prolonged anaesthesia , significant blood loss , hypothermia , positioning , concerns of air embolism , spinal cord monitoring and respiratory complications make anaesthetic management very challenging .
a proper plan should be made and discussed with surgeons pre - operatively regarding anticipated duration of surgery , blood conservation strategies ( use of antifibrinolytics , hypotensive anaesthesia , cell saver , acute normovolaemic haemodilution ) , neurologic monitoring to prevent spinal cord damage , wake - up test , post - operative ventilation and pain management .
although we had motor - evoked potential monitoring in our hospital , we did not use them , as our patient had lower limb power of 1/5 pre - operatively .
apart from the above - mentioned concerns for major spine surgery , additional problems in our patient were cervical spine protection during induction and positioning .
mils of the cervical spine during airway manoeuvres should always be used in patients without radiological abnormalities . however , in patients with cervical cord compression , awake fibreoptic intubation should be considered .
anaesthesia induction ( intravenous or inhalational ) might need to be modified depending on the presence or not of lesions involving maxillary or mandibular bone in which inhalational induction , especially in children , and mask ventilation might not be possible .
great care should be taken while positioning prone for surgery , especially pressure over eyes , bony points and abdomen should be avoided .
respiratory system involvement in the form of chylothorax in gs patients is not an infrequent complication and , hence , needs special mention .
patients might present for surgery with this complication or they might develop it post - operatively .
diseases of ribs , scapula or thoracic vertebrae may lead to the development of chylothorax from direct extension of lymphangiectasia into the pleural cavity or via invasion of thoracic duct .
these patients should undergo complete respiratory assessment ( arterial blood gas analysis , spirometry ) .
if there is restrictive lung disease , ventilation should be undertaken by using low tidal volume and high respiratory rate . in these patients
post - operative ventilatory problem leading to re - intubation and prolonged ventilation has been described in the literature .
therefore , extubation has to be planned carefully , with due preparation for prolonged intensive care unit management .
fortunately , our patient did not have any respiratory involvement and complication as well . in a very recent case report
, a nulliparous lady with gs was managed successfully under spinal anaesthesia for urgent caesarean section .
the same patient had a fused cervical spine due to surgery of cervical spine thus posing a serious challenge for airway management .
hence , neuraxial anaesthesia can be safely employed in infra - umbilical surgeries provided there is an absence of lumbar spinal osteolytic lesions .
however , chylothorax - induced hypoproteinaemia dictates cautious use of high protein - bound drugs .
succinylcholine may produce unpredictable fasciculation and the possibility of fracture in osteoporotic bones is a concern ; hence , better to be avoided .
fortunately , our patient had an uneventful recovery , without any further neurological sequelae . apart from regular concerns for major spine surgery
, special attention should be given to airway management and positioning , and anticipation of post - operative pulmonary complication will go a long a way in safe anaesthesia for this rare disorder .
 gorham 's syndrome ( gs ) is a rare disorder characterized by proliferation of vascular channels resulting in destruction and resorption of osseous matrix leading to bone loss . 
 bone loss leads to joint instability and problems during airway management and positioning for surgery . 
 respiratory involvement further complicates anaesthesia management . 
 we report the anaesthetic care of a 21-year - old male patient of known gs for spine decompression and fusion in prone position . 
 airway management , induction technique , pathophysiology of the disease , drug selection and other concerns of anaesthesia for major spine surgery has been discussed reviewing the sparse literature available . 
INTRODUCTION
CASE REPORT
DISCUSSION