MRCPCH Hemiplegia 3 year old boy General – head – scar,VP shunt, look for abdo scar, can feel for shunt blockage (hard, non compressible) Microcephaly, Gait – exposure, shoes off ?hemiplegic / wide based Tone, power, reflexes difficult as child and not always compliaint CP – if preterm – likely diplegia If HIE – likely dyskinetic CP Hemiplegic – stroke / tumour / haemorrhage Hypotonia approach Paralytic – weak Non paralytic Anatomical classification Spinal cord Anterior horn Peripheral nerve NMJ Muscle Distal / proximal Muscle wasting, look for tongue SMA – tongue fasciculations Normal intelligence DDx congenital myopathy – usually worse intelligence. E.g. central core, nemaline , myotuiular Muscular dystrophy – inflammatory condition Tone, power – distal vs proximal, reflexes, sensation, coordination Infantile spasm Idiopathic, cryptogenic, symptomatic (e.g. syndromic, genetic) TS Vigiabatran – vision – visual field – lifetime communitive dose Ambiguos genitalia - approach Resp Jul 19 L pleural effusion 8yo F has presented with fever Optimize positioning Look, inspect, resp rate (count!), palpate (trachea / expansion), percussion, ausculate If sit up, can do both back and front together Reduced chest expansion at bases Reduced air entry Also associated with percussion dullness ➔ Effusion 17 m old with acute respiratory distress PE/ Playful but stranger anxiety Mild resp distress Observe Need to count RR! Some pectus excavatum Sc insucking Stridor (biphasic) Rhonchi DDx: Croup Upper airway obstruction – subglottic stenosis, haemangioma, less likely epiglottitis. Background of Chronic lung disease Developmental delay / ex prem UCH 1) Congenital myopathy 7 year old bed bound non comunicable with limited spontaneous movements. He has a expressionless face. There are foot splints by bedside, he is on ventilator via tracheostomy and has oxygen saturation monitoring. On inspection, there is a scar over L thigh most likely due to muscle biopsy. There is also generalized muscle wasting and hypotonia but no fasciculations. The power is grade 1 out of 5 with flicker of movements. He is areflexic. The spine Is grossly normal. There is no tongue fasciculations. The findings are consistent with lower motor neurone lesion and my differential diagnosis is congenital myopathy. Other ddx includes muscular dystrophy (but no calf hypertrophy), spinal muscular dystrophy. 2) Acute Transverse myelitis This is a 6 year old subacutely with unsteady gait. On examination, he has lack of power over left lower limb. Reflexes present, tone normal, power deficient and sensation also deficient. Sensory exam important. Sensory level. Spinal level of T4 but with partical recovery. Sensation impaired over Left lower limb. Ddx ADEM – if encelopathy Demyelination / GBS Chronic demyelinating polyneuropathy Mx: IVIG / steroids 3) Laurence–Moon syndrome Syndrome Obesity Type two dm DM diagnosis Fasting Glucose >7 or HbA1C OGTT not so useful in children Management – meds Ching Wai L This is a 3 yr old boy with visual impariement Gather appropriate info / history and asses this child’s funciontal vision Please also asses his gross motor skills. Read question! Approach: Quick history The assessment Then may summaries / supplement history Don’t forget psychosocial support History: Birth, BW, gestation Neonatal stay HPI Severity of illness, ecmo / CPR, MRI scan/ brain damage Genetics if relevant FHx Rehabilitation hx: PT/OT/ST/Dietican/Nurse/P&O/ Ortho / Feeding team FU neuro/ ENT/eye / ortho / DK Early intervention program for visual impaire to build up language recognition skills Visual impairment SCCC Ebenezer Blind: Social support – support group Disability allowance Social worker Psychology GM : Sit unsupported straight back Not yet crawl Not yet turn over but history says able turn over Vision: Follows light Follow facies Uses stripes card to follow (large to small) Pick up cubes / other objects Make sure NO sound Deveclopment: 4 spheres: Social / self care GM FM / vision Speech / language SMA Infant – 2 year old SMA type 1 With LMN lesions, bed bound, tongue fasciculations CMT: 8 year old with mild clumsiness on prolong walking Inspect : foot drop hammer toe pes cavas Mild wasting No scars Aflexia Mild hypotonia Power 4/5 (only achieve one grade if can complete the criteria) No scars DDx: CMT / peripheral neuropathy / muscular dystrophy (but no muscle hypertrophy) Downs T21 facies With scar over C spine TT over neck 4 limb hypertonia, weakness grade 2/5 (no complete movement against gravity) 4 limb hyperreflexia Non communicable Sensation difficulty to elicit kWH teachings: 1) GSD type 3 19 yr massive old hepatosplenomegaly Normal intelligence No jaundice or pallor Exam system abdo : peripheral, inspect, palpate, ausculate 2) 17yo abdo Multiple scars Bilateral thoracotomy, midline sternotomy, midline laparotomy, transverse laparotomy, right iliac fossa oblique scar Renal mass RLQ, hirsutism (steroids), also has clubbing, cyanoic heart disease, Tracheo esophageal fistula repair, renal transplant (CKD), failure to thrive, complex congenital heart repair and abdominal surgery for imperated anus. 3) CVS 7 year old with thrill and 4/6 PSM loudest over LLSE No signs of heart failure, thriving, no scars 4) JIA Teenage girl with difficulty in using hands Look, feel more function PGALS No scars, Muscle wasting over Left palm Over short and small bilateral 4/5 MCP Swelling over L wrist Other joints ok Feel – swelling but no tenderness or warmth Move – active – limited range of movement over the L wrist with prayer sign R wrist less affected Skin – no rash Nails – normal Other PGALS – normal Imp: Polyarticular JIA / oliarticular JIA with arthropathy L wrist and bilateral MCP RFve CCP+Ve Worse prognosis. Ddx: Psoriatic / oligo / other forms of JIA SLE Mx: PT, analgesisa, MTX. PGALS Look, feel, move, (active, passive), function 1) 7yo Dextro VSD PSM R sternal edge No s/s of heart failure 2) 18 y o with R hemiplegia Hemiplegic gait, short stance phase, reduced arm swing MUSCLE WASTING! No scars Spastic Hypertonic Reflexes mostly equal 3) HbH disease 16y o with tinge jaundice / pallor, hepatosplenomegaly. Guardlers line towards to ?anterior axillary line Laparscopic SCARS! For previous cholecystectomy 4) 6 year old with PS (no clicks) 3/6 ESM over LUSB Cornelia de Lange syndrome (CdLS) is a syndrome of multiple congenital anomalies characterized by a distinctive facial appearance, prenatal and postnatal growth deficiency, feeding difficulties, psychomotor delay, behavioral problems, and associated malformations that mainly involve the upper extremities Systems: limbs, development, GI, General: Marfanoid features Long thin built, slim, increase arm span Aortic, FHx,, hypermobility, PTX, eyes, arch, scoliosis General: Café au lait spots Freckles, Puberty, Eyes – lich nodule , optic glioma, visual field , Abdomen Renal bruits Neurofibroma / sensation Fhx Bone TKO Neuro TS – angiofibroma, skin – café au lait, ashleaf macules, shagreen patch Other systems: abdomine for angiomyolipoma, heart for rhabdomyosarcoma, Macrocephaly 5 year old with complex cyanotic – PA with aberrant pul artery shunt Signs: multiple midline scar, L thoracotomy. Cyanosis, Continuous murmur Mitral regurgitation with syndromal – unknown syndrome Atrial septic defect in young infant Fixed splitting 2nd HS with ESM 2/6 General exam HbH hepatosplenomegaly Spleen>liver Jaundice, no definite pallor, No transfusion dependent. marf Nephrology MMF Aciclovir DMD with SVT heart failure Hydronephrosis / UTI Ex prem CLD exacerbation PWH Development: cortical dysplasia HbH x2 PVL with diplegia DMD / proximal muscle weakness Williams syndrome with AS Stoma – Crohns
