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• 1 year old male child diagnosed trisomy 21 at birth came with pain and swelling of scrotom 2 days • suspected torsion testis • Came for emergency surgery

Sony downsyndrom

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Page 1: Sony downsyndrom

• 1 year old male child diagnosed trisomy 21 at birth came with pain and swelling of scrotom 2 days

• suspected torsion testis• Came for emergency surgery

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history

• Diagnosed Downsyndrom by karyotyping after birth

• He makes noise at sleeping• h/o mild recurrent respiratory infection• Eccho cardiograph done showed some minor abnormality report not available• Occasional cough during feeding

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examination

• Downsyndrom facial features present• Airway normal• Weight 6.5kg small for age• Scrotum appear normal• Not in pain• Cvs appear normal• Chest clear• Muscle tone appear normal• Blood results appear normal• Is fasting for 6 hours

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DOWN SYNDROME

Down Syndrome, or trisomy 21, is the most common chromosomal

anomaly, occurring in 1/700 to 1/1100 of live births.

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downsyndrom

• mental retardation (100% have IQ's below 65)• seizure disorder (2-9%), • congenital heart disease (50%), • cervical spinal laxity, • neonatal GI lesions, GI reflux, • respiratory infections• immunodeficiency• leukemia

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Congenital heart disease in downsyndrom

• VSD (30-40%), endocardial cushion defects (AV canal) (32%), and other defects associated with pulmonary hypertension.

• The combination of intracardiac shunts and pulmonary infections leads to the early development of cyanosis and Eisenmenger's syndrome.

• Meticulous air bubble precautions and SBE prophylaxis warranted

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Airway problem

• immunodeficiency and recurrent pulmonary infections combined with heart disease and craniofacial hypoplasia increases the risk of difficult oxygenation during the induction of anesthesia. These children may have a high arched palate, small upper face and nose, as well as a large tongue. To some degree, the joint laxity balances these factors so that intubation is usually not difficult

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Cervical spine abnormality

• Care should be taken not to hyperextend the neck,

• 10-30% of all children with Down Syndrome will have cervical spinal stenosis or atlanto-axial subluxation on C-spine films

• only 1-2% will become symptomatic. These symptoms include neck pain, torticollis, quadraplegia, clonus or weakness in the lower extremities.

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• Approximately 25% of children require an ETT that is 1-2 sizes smaller than expected) due to generalized smaller size. Adults may also have relatively smaller tracheas. Postoperative stridor and other respiratory complications are more common than in the general population. Postoperative airway obstruction may also be due to macroglossia and poor pharyngeal tone.

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• gastrointestinal defects are common. Imperforate anus, tracheoesophageal fistula, and duodenal atresia, Gastro-oesophageal reflux are the most common.

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Endocrine system

• 50% have hypothyroidisum

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CNS abnormalities

• CNS abnormalities in patients with Down syndrome may be associated with altered concentrations of neurotransmitters, such as serotonin, dopamine, norepinephrine and acetylcholine. Therefore, very careful in the administration of sedatives and narcotics to these patients.

• There have been reports of exaggerated response to atropine, with tachycardia and cycloplegia.

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anesthesia

• The baby came with iv canula• Sedated with ketamine 5mg and .05mg

glycopyrolate iv on receving the baby• Induced with oxygen nitrous and isoflurane• Igel 1 size inserted and fixed• Spontanious respiration• Caudal .25% bupivacaine 7 ml

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maintenance

• Oxygen nitrous isoflurane .2%• Intra op uneventfull• Suspected as orchitis testis was fixed by

sutures• Post op wake up immediately• crying• Post op analgesia 2mg/kg tremadol?• Post op was uneventfull

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Thank you