Pediatric Overview powerpoint

7/28/2019 Pediatric Overview powerpoint

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Neuro 1


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Cranial CT and B. MRI showing

bilateral posterior subdurals andprominent frontal extra-axial fl uid

collections.


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Neuro 2


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Neuro 3


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Cranial CT showing small volume

interhemispheric blood and

reduced attenuation of hemispheres

with reversal sign (cerebellum

higher attenuation than hemispheres).


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Case study


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A 9 year old Asian girl presented with a 2 week history of fever.

There had initially been some diarrhoea, without vomiting, whichhad resolved after 3 days. There was no signifi cant previous history

and no history of recent foreign travel.

On examination she was systemically unwell with a fever of

40 C. There was a faint pink non-pruritic macular rash involving

the face and trunk and reddish macules on the fi ngers and toes.

Generalized lymphadenopathy was evident but no organomegalyInvestigations:

FBC Hb 8.9 g/dL, WCC 3.7 109/L, with lymphopaenia and thrombocytopaenia,

platelets 96 x 109/L

ESR 126 mm/h, CRP 87 mg/dL.Blood cultures: grew Salmonella enteriditis at 24 h

Urinalysis: proteinuria + and haematuria ++ on dipstick testing.

What is the differential diagnosis?


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The most likely diagnosis is SLE. There is a well established association

between SLE and non-typhoidal salmonellosis. Although

this occurs most often in patients with active disease on intensifi

ed immunosuppression, Salmonella bacteraemia can occur at

first presentation. It is hypothesized that patients with SLE have

a specific immune defect predisposing to invasive disease.

What would you expect on her autoimmune

screen?She had strongly positive ANA and positive anti-dsDNA antibodies.

She was ant-Ro and anti-La positive. There was marked

hypocomplementaemia. The uncharacteristically raised CRP

was ascribed to her bacteraemia.She went on to develop severe lupus nephritis and required treatment

with hydroxychloroquine and mycophenolate mofetil.

Subsequently her general disease became active, especially in the

skin. This was treated with rituximab.


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SLE: A. Butterfly malar facial rash. B.

Truncal rash.


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Hip ultrasound scan: A. Normal. B.

Joint effusion.

Hip ultrasound is sensitive for detectionof an intracapsular effusion with capsular distension

>2 mm (Fig 16.12). An effusion is nearly always present in TS,

but ultrasound scan cannot reliably distinguish between TS

and septic arthritis. Absence of an effusion should prompt acontinued search for the source of the pain.


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Rheumatology 1


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Polyarticular JIA: hands.Polyarticular JIA

This may be rheumatoid factor positive or negative. Arthritis

affects 5 or more joints in the fi rst 6 months. Large and small

joints can be involved, often symmetrically.Polyarticular JIA, rheumatoid factor negative

Affects girls more than boys with a biphasic age distribution:

early peak 24 years, later peak 612 years. Up to 50% are ANA

positive and at risk of uveitis: there is overlap with oligoarthritis

in young females. Cervical spine and temperomandibular joint

involvement is common.

Investigations

FBC: mild anaemia, mild neutrophil leucocytosis, moderate

thrombocytosis

IgM rheumatoid factor: negative

ANA: positive in 2040%.

The course is variable. 50% go into remission within 10 years.

Recurrent episodes cause progressive deformity.

Polyarticular JIA, rheumatoid factor positive.

More common in girls with a peak in late childhood, >8 years or

adolescence. An aggressive, symmetrical arthritis affects particularly

the small joints of the wrists, hands (Fig 16.10), ankles, andfeet. Knees and hips involved early with elbows and other joints

later. The pattern is similar to adult rheumatoid arthritis. Rarely,

rheumatoid nodules occur on pressure points and vasculitis may

cause nail fold lesions.

Investigations

FBC: moderate anaemia, ESR: elevated.

ANA: might be positive.

HLA DR4 associated.

Prognosis is poor, with severe joint destruction.


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Rheumatology 2


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Oligoarticular JIA most commonly affects girls


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Systemic-onset SLE

S t i t JIA (SOJIA)


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Systemic-onset JIA (SOJIA)

SOJIA affects males and females equally and occurs

throughout

childhood, most commonly at age 15 years. Thecharacteristic

presenting features include:

Fever: quotidian with 12 spikes >38.5 C per day

Rash: evanescent, salmon pink, non-pruritic rash on trunk andextremities (Fig 16.8)

Arthralgia: symmetrical, affecting one or several joints. Frank

joint swelling may not occur for months after onset.

Additional features include lymphadenopathy,dermographism,

hepatosplenomegaly, and anaemia. Serositis may occur and

manifest

as pericarditis or abdominal pain.


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Rickets: A. Rickety rosary and

Harrison groove.

B.Metaphyseal expansion at wrist


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Rickets is often asymptomatic and a high index of clinical suspicion

should be maintained in infants at risk. Rarely, an acute

infection may precipitate hypocalcaemia and tetany as a presenting

manifestation. The clinical stigmata depend on the stage of

evolution and severity.

Skeletal

Skull: frontal bossing and softening of the skull bones, especially

along suture lines, craniotabes. Delayed closure of anterior

fontanelle and wide sutures.Thorax: expansion at the costo-chondral junctions causes a

rickety rosary. Harrisons grooves, pectus carinatum, and

kyphoscoliosis develop.

Limbs: fl ared metaphyses apparent at distal radius at wrist,femur at the knee and tibia causing double malleoli at the

ankle. Weight-bearing causes bow legs or knock knees. Short

stature develops.

Muscular: generalized hypotonia with delayed walking

Nervous system: irritability, tetany, apathy.


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Rickets: radiological signs. A.Thorax: expansion of

costochondraljunctions.

B. Wrist: flaring and cupping of

metaphyses.

A l i di h f h k


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A plain radiograph of the knee

showing metaphyseal ends

and epiphyses of the femur and tibia isthe best single view in

children


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Achondroplasia. Sagittal T1 MRI: large

cranial vault, smallskull base, cervico-medullary kink,

large suprasellar cistern.


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Imaging: a skeletal survey at birth confirms the diagnosis. The

features include:

large calvarium with narrow foramen and small skull base

vertebral bodies short with wide intervertebral discs,

square-shaped long bones, irregular growth plates

hands: broad short metacarpals and phalanges and trident

configuration.


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h d l l l f d d


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Perthes disease: unilateral left sided.

Plain radiography of the pelvis with AP

and frog lateral views isthe modality of choice.However,

radiographs may benormal in early symptomatic disease.

Bone scan is more sensitive

in the early case. MRI is equally

sensitive and allows more

precise localization of involvement.


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DDH: delayed ossifi cation of femoral head

and increased

slope of acetabulum on right side at age 2

years with subluxation of

the joint.Hip ultrasound scan is indicated if an abnormal hip issuspected

on newborn examination or in at risk infants. After 46

monthsan AP pelvic radiograph is the optimal investigation. This may

reveal delay in ossification of the femoral head, a broken

Shentons line and increased acetabular slope.


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Congenital talipes equinovarus.In this deformity of the foot and ankle (talipes, Latin talus = ankle

+pes= foot), also called club foot, the foot is held in a rigid

equinovarus position. The foot is inverted and supinated

and the forefoot is adducted. The incidence is 1/1000 live

births and it is more common in boys.

CTEV is distinguished frompositional talipes equinovarus which

can be passively corrected, i.e. the foot can be fully dorsiflexed

to touch the front of the lower leg. This resolves spontaneouslywithin 612 weeks.


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Management

Referral is made to an orthopaedic surgeon. Early treatment

improves prognosis. Conservative treatment encompasses the

Ponseti technique:Manipulation and serial plaster casts.

Percutaneous tenotomy of Achilles tendon under local

anaesthetic at ~6 weeks.

Boots and bars treatment for fi rst few years as necessary

with further plastering if deformity recurs.

Corrective surgery may be required at 612 months of age if

there is a failure to obtain or maintain reduction of the talonavicular

joint and hindfoot/forefoot alignment. Soft tissue release

and realignment of joints is carried out.The prognosis for idiopathic club foot is good with a functional,

pain free, supple plantigrade foot as the usual outcome. Surgery

is more often required in the context of a neuromuscular disorder

or syndrome and recurrent deformity is not uncommon.


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Case study 2


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A 7 year old girl is admitted with chickenpox. The rash developed

4 days previously and new lesions are continuing to appear.

Concern has arisen on account of two painful, spreading lesions

on her anterior chest wall.On examination she is systemically unwell with high-grade fever

and signs of early circulatory compromise: tachycardia and

prolonged peripheral CRT.

Dx. And Mx.?


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What diagnosis should be considered?

Necrotizing fasciitis is a recognized complication of chickenpox

and presents with tender erythematous mottled lesions which

spread rapidly. Several factors contribute to pathogenesis. Thevesicles create a full-thickness dermal lesion providing a route

for bacteria to spread from the skin surface into the subcutaneous

tissues. Causative organisms include GAS and Staph.

aureus.

What is the management?

This is a life-threatening emergency requiring early aggressive

surgical debridement. Immediate resuscitation and IV broad spectrum

antibiotics are indicated. Preoperative MRI can be

helpful in differentiating necrotizing fasciitis from cellulitis anddelineating the affected tissues to aid surgical planning. However,

any delay in definitive surgical treatment is inadvisable.

Wound swabs grew a heavy growth of Staph. aureus and GAS. She

survived after extensive surgical debridement and intensive care.


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Polymorphous rash and red lips.


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HSP: typical purpuric rash and

distribution.


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Skin: the classical rash is a palpable purpuric rash often

symmetrical over the extensor, dependant surfaces of the

lower limbs and buttocks. This may be preceded by urticarial

or erythematous maculopapular lesions and may range from

petechiae to large ecchymoses


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Cutaneous leishmaniasis.


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Cutaneous leishmaniasis: parasites proliferate locally leading

to an erythematous papule where the bite took place, usually

on an exposed area such as the face. This evolves to become

a nodule and then a shallow ulcer (Fig 15.20). Satellite lesions

and regional lymphadenopathy may occur. A scraping or

punch biopsy sample is stained (Giemsa) for Leishman

Donovan (LD) bodies.


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Meningococcal septicaemia: late stage

rash.


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The characteristic rash is often

non-specifi c initially, with a red,

maculopapular appearance before

evolving into the well knownpetechial or purpuric non-

blanching rash


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Acute bacterial cervical adenitis: an

abscess has formed,requiring surgical drainage.


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Impetigo


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Tubercular meningitis: A. Bilateral

dilated ventricles and meningeal

enhancement. B. Hydrocephalus

and cerebellar tuberculoma.


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Hil l h d th d ili TB


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Hilar lymphadenopathy and miliary TB:

CXR.


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E t l TB i l


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Extrapulmonary TB: cervical

lymphadenopathy.


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MRI showing resulting left basal

ganglia infarct with oedema


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HIV infection: parotid enlargement.

Often associated withgeneralized lymphadenopathy and

LIP.


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Atypical or reactive lymphocytes.

Large with condensed

chromatin pattern in nucleus and

pale blue, basophilic cytoplasm.Seen in EBV infection and also

CMV, toxoplasmosis, viral hepatitis,

and streptococcal infection.


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Chickenpox rash: lesions at different


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Chickenpox rash: lesions at different

stages.

Varicella zoster: shingles of right


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Varicella zoster: shingles of right

thoracic dermatome.


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Measles


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SCID: PCP


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Pg 306


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