ABSTRACT:Coarctation of aorta is a congenital disorder with 5-10% incidence. It occurs in about 1 in 10,000 births .It may remain asymptomatic till adulthood. During pregnancy, early gestational hypertension is the presenting sign. Difference in blood pressure in upper and lower limb is a characteristic finding of coarctation of aorta. Association of bicuspid aortic valve and ventricular septal defect is seen in 50% patients of coarctation. Major cardiovascular complications are infrequent but continue to be a source of concern for patients with coarctation who become pregnant. Coarctation of aorta commonly located at the junction of the arch of aorta and proximal descending aorta at the level of ductal structure, may be diagnosed for the first time during pregnancy. Dilatation and dissection of the aorta can lead to increased maternal mortality; significant stenosis is a contraindication to pregnancy. However, successful pregnancies have been reported in women with uncorrected coarctation if preconception risk stratification is done. Here is a case of LSCS with coarctation of aorta and Takayasu arteritis done successfully under epidural anaesthesia.

BACKGROUND: Osteopetrosis is a collective term used for a pathological condition with defective function of osteoclasts presented with range of sclerosing bone diseases along with skeletal, renal, haematological & neurological manifestation. It may be autosomal recessive (ARO), autosomal dominant (ADO) and X-linked. It presents with hydrocephalus, short stature and anaemia, involvement of ocular nerve or facial nerve. Hypocalcemia, tetany, seizures & secondary hypoparathyroidism is known to occur. CASE REPORT: Two patients of osteopetrosis were posted for orthopaedic surgery. Both patients were operated under combined spinal epidural anaesthesia. 2ml of 0.5%Bupivacaine+30mcg clonidine was given intrathecally & epidural supplementation was given with Bupivacaine 3 cc increments after two segment regression of sensory level. Postoperative analgesia was provided by epidural Bupivacaine 0.125% along with Inj. Tramadol 50 mg on patient's request. CONCLUSION: Even if administration of anesthesia is a challenge in patients of Osteopetrosis, regional anaesthesia can be given safely with proper preoperative preparation & intraoperative care. KEY WORDS: osteopetrosis, femur fracture, combined spinal & epidural anaesthesia INTRODUCTION: Osteopetrosis is a collective term used for a pathological condition with defective function of osteoclasts presented with range of sclerosing bone diseases along with skeletal, renal, haematological & neurological manifestation. It is classified as Infantile malignant or (ARO), Intermediate type or (ARO) , Adult onset or (ADO)&X-linked Osteopetrosis. ARO is a life threatening condition manifests in first few months with life span of 6 to 10 yrs present with seizures with normal Calcium levels, renal tubular acidosis, cerebral calcification, developmental delay, hypotonia, retinal atrophy & sensorineural deafness. ADO is type I and type II. Type I is associated with reduced number & size of osteoclasts & involvement of ocular nerve while type II is associated with proliferation of large & multinucleated osteoclasts, involvement of facial nerve , bony sclerosis, renal tubular acidosis & cerebral calcification. In X-linked Osteopetrosis severe immunodeficiency is observed with ectodermal changes. The difficulties faced by Anaesthetists are difficult intubation due to facial deformities, head & mandibular involvement cervicomedullery stenosis may lead to cord trauma during intubation difficult spinal & epidural anaesthesia due to scoliosis and short stature. Leucoerythroblastic anaemia, pancytopenia, thrombocytopenia leading to excessive bleeding

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INTRODUCTION: IVRA is a simple, reliable, and effective technique with rapid onset of action, rapid and prompt recovery after tourniquet release. It provides good analgesia, adequate muscle relaxation, & bloodless operative field. It is widely applicable to patients of different ages and physical status for operations & cost effective. Lignocaine though preferred local anesthetic agent has limitation of short duration of anesthesia & inability to provide postoperative analgesia various additives were added to it. In this study we compared efficacy of clonidine as an adjuvant to lignocaine with plain Lignocaine. MATERIAL & METHOD: Patients undergoing upper arm surgery were included in this study & are divided in two groups. The proximal circulatory isolation of arm was done by placing a pneumatic tourniquet around arm. In group C IVRA was given by 1ug/kg clonidine & 0.5% preservative free lignocaine in a dose of 200mg diluted up to 40 ml & in group L 0.5% preservative free lignocaine 200 mg diluted up to 40 ml. Tourniquet was deflated at least 30 mines after injection of drug. AIMS & OBJECTIVES: To compare onset and quality of sensory analgesia, Onset and quality of motor blockade, onset & severity of tourniquet pain, Complications like hypotension &bradycardia during the procedure, recovery from sensory and motor blockade duration of Postoperative analgesia in group C with group L. CONCLUSION: We observed that using Clonidine in dose of 1 ug/kg as an adjuvant to Lignocaine in IVRA does not have early onset of sensory blockade, increased tourniquet tolerance, delayed tourniquet pain and extended post-operative analgesia .Neither systemic side effects like nausea, bradycardia, hypotension, convulsion nor local complications like hematoma were observed. INTRODUCTION: Intravenous regional anaesthesia was originally introduced by the German surgeon August K. G. Bier 1 in 1908; thus the name, "Bier block". Dr. Bier described a complete anaesthesia and motor paralysis after intravenous injection of Prilocaine into a previously exsanguinated limb. It is a simple, reliable, and effective technique with rapid onset of action, and prompt recovery after tourniquet release. It provides good analgesia, adequate muscle relaxation, & bloodless operative field, widely applicable to patients of different ages and physical status for operations & cost effective. Poor postoperative analgesia, limited duration of anaesthesia (<90 minutes), the potential for local anesthetic toxicity, nerve damage and compartment syndrome are the disadvantages of intravenous regional anaesthesia.