Sure Shahbaz Khan Sir

Cerebral palsy

Cerebral palsy
Def.

Cerebral palsy is defined as a non -progressive neuromotor disorder of cerebral orgin
ethiopathogenesis
1. Maldevelopment and disorderly anatomic organisation of brain
2. Perinatal hypoxia
3. Birth trauma
4. Acid base imbalance
5. Indirect hyperbilirubenamia
6. Metabolic disturbance
7. Intra uterine or acquired infection

Pathological lesion such as cerebral atrophy porencephaly leucomalacia. Degeneration of basal ganglia  cerebelar lesion

A) SPASTIC CEREBRAL PALSY

Depending upon distribution of spasticity
it may be
1. Spastic quadriplegia
 it is more commen in term baby . Ht include opisthotonos posture ,pseudobulbar palsy ,feeding difficulties ,restricted voluntary movements. And multiple deficits .

2SPASTIC DIPLEGIA

 commoner in preterm babies  and associated with periventricular leukomalacia . the lower limbs are more severily affected with extension and adduction posturing. Brisk tendon jerks and tendency to contracture .

3.SPASTIC HEMIPLEGIA

 Recognition after 4 to 6 months age due to poor observation
Abnormal persisting fisting., abnormal posture or gait disturbance,vascular insult , porencephaly or cerebral anomalies may be associated

B) HYPOTONIC CEREBRAL PALSY

 Despite pyramidal involvement there patient are atonic or hypotonic .
tendon reflexes are normal or brisk and Babinski personse is positive .
They are often severely mentally retarded .
In cerebral involvement hypotonic is not associated with exaggerated reflexes .

C) EXTRAPYRAMIDAL CP

The clinical features include dyskinesia such as athetosis , choriform movements , dystonia tremors and rigidity
Arms legs trunks may be involved
mental retardation and hearing defect may be present .
Cerebral damage following bilirubin encephalopathy is a classical example and deafness is commonly associated.
In cerebral involvement  there is hypotonia and hyporeflexia.
Ataxia and intentional tremors appear by the age of 2yrs.

ASSOCIATED FEATURES

1. EYES
Strabismus, Paralysis of gaze,
 cataract,colobama, refractive errors.
2. Ear :) loss of hearing
3. speech aphasia ,dysarthria and  dyslalia
4. Sensory defects asereogenosis and spatial disorientation are seen.
5. generalised or focal seizures
6. Mental retardation

DIAGNOSIS

1. Suspected in following cases
low birth weight ,perinatal insult has increased tone ,feeding difficulties and does not keep pace with anticipited normal range of neurological and behavioural development .
2. evaluation of patient include perinatal history ,detailed neurological and developmental examination , electroencephalogram,psychometric  and sensory evaluation .
3. assessement of learning and language difficulties .
4. aminoaciduria should be exclude by chromatography of urine and plasma

PREVENTION

can be prevented by prevention of infection ,fetal of perinatal insult ,hmmm maternal care and freedom from postnatal damage reduce prevalance .
MANAGEMENT
1. SYMPTOMATIC treatment for seizuries.
2. Tranquilizers are administered for behaviour disturbances .
3. Muscle relaxants for improving muscle function
4. Baclofen helps to reduce spasticity ,
Diazepam ,
Dantrolene sodium  for relaxation of skeleton muscle .local phenol block .
Plastic orthoses

OCCUPATIONAL THERAPY

simple movements of self help with progressive development of more intricate activities .

EDUCATIONAL

THE Defect of vision ,speech, and learning are managed by adequate
ORTHOPEDIC SUPPORT by light wt splint
SOCIAL SUPPORT TO FAMILY
REHABILITATION AND VOCATIONIBL GUIDANCE

Carcinoma of Maxillary

Carcinoma of Maxillary 

AETIOLOGY

1.Worker in hardwood  furniture industry nickel refining, leather work and manufacture of mustard gas have shown higher incidence of sinunasal cancer
2.Commn in bantus of south africa where locally made snuff is used which is rich in nickel and chromium .
3. Worker of furniture industry

Disease is common in 40-60 age group with preponderance in male .

CLINICAL FEATURES

early features of maxillary sinus malignancy are
1. nasal stiffiness
2.Blood stained nasal discharge
3.Facial paraesthesias or pain and epiphora

DIRECTIN OF SPREAD AND EXTENT OF GROWTH

1.Medial spread to nasal cavity gives rise to nasal obstruction, discharge and epistaxis.
2.Anterior spread  cause swelling of the cheek and later invasion of the facial skin.
3.Inferior spread causes expansion of alveolus with dental pain ,loosening of teeth ,poor fitting of dentures. Ulceration of gingiva  and swelling in the hard palate .
4.Superior spread : invades the orbit causing proptosis ,diplopia,occular pain and epiphora.
5.Posterior spread ir into pterygomaxhllary fossa ,pterygoid plates and the musckes causing trismus. Growth may also spread to the nasopharynx,sphenoid sinus and base of skull
6.Intracranial spread
to ethmoid ,cribriform plate or foramen or foramen lacerum
7.Lymphatic spread  occur in the late stage of disease .
Submandibular and upper jugular nodes are enlarged .Maxillary and ethmoid sinuses drain primarily into retropharyngeal nodes, but these nodes are inaccessible to palpation.
8.Systemic metastases
 may be seen in the lungs and occasionally in bone

CLASSIFICATION

A] Ohngren's clas.
An imaginary line is drawn extending between medial canthus of eye & angle of mandible.
Growths situated above this plane have a poorer prognosis.

B] Lederman's clas.
It uses two lines horizontal lines of Sebileau one passing through the floors of orbits & d other through floors of antra. There r 3 parts.
1. Suprastructure. Ethmoid, sphenoid & frontal sinuses & olfactory area
2. Mesostructure. Maxillary sinus & respiratory part of nose.
3. Infrastructure. Containing alveolar process.
Vertical lines extending down d medial walls of orbit to separate ethmoid sinuses & nasal fossa fm d maxillary sinuses.

C] American Joint Committee on Cancer(AJCC) clas.
Used only 4 squamous cell carcinoma. Histopathologically it is graded as
1. Well differentiated
2. Moderately diff.
3. Poorly diff.

D] TNM Clas. & staging system.

Maxillary sinus
T1
tumor limited to maxillary sinus mucosa with no erosion or destruction of bone.
T2
tumor cousing bone erosion or destruction including extension into d hard palate & or middle nasal meatus, except extension to post. wall of maxillary sinus and pterygoid plates
T3
tumor invades any one of following. Bone of post. wall of maxillary sinus, subcutaneous tissues, floor or medial wall of orbit, pterygoid fossa & ethmoid sinuses
T4a
tumor invades ant. orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinuses
T4b
tumor invades any of d following: orbital apex, dura, brain, middle cranial fossa, brain, cranial nerves other than maxillary division of trigeminal, nasopharynx or clivus.

Regional Lymph nodes( N )
Nx
minimum criteria of assessment cannot be met with.
No
No regional LN metastasis
N1
Metastasis in a single ipsilateral LN, 3cm or less in greatest dimension
N2a
Metastasis in a single ipsilateral LN more than 3cm but not more than 6cm in greatest dimenson
N2b
Metastasis in multiple ipsilateral LN none more than 6cm in greatest dimension
N2c
metastasis in bilateral or contralateral LN none more than 6cm in greatest dimension.
N3
Metastasis in a LN more than 6cm in greatest dimension

Distant Metastasis ( M )
Mx Distant metastasis cannot be assessed.
M0 No distant metastasis
M1 distant metastasis

STAGE GROUPING OF CANCER OF MAXILLARY SINUS
StageI. T1 N0 M0
StageII. T2 NO MO
StageIII. T3 N0 M0
          T1 orT2 orT3 with N1 M0
StageIV A. T4 N0 M0
             T4 N1 M0
StageIV B. Any T N2 M0
              Any T N3 M0
StageIV C. Any T Any N M1

DIAGNOSIS

A)Investigations
 Non Invasion
1. x rays water's view,max sinus partly
2. usg
3.CT SCAN with contrast
B)Invasive
1.Antral puncture
2.Biopsy
3.ECG

1.Radiograph of sinuses
opacity of the involved sinus with expansion and destruction  of the bony walls
2.CT scan if available this is the best non invasive method to find the extent of disease . CT scan done both in axial and coronal planes .It also helps in staging of disease.
3.BIOPSY :if growth presents in the more or mouth .Biopsy can be easily taken . In early cases ,with suspicion of malignacy ,sinus should be explored by caldwell luc operatiom .Direct visualisation  of the site of tumour in the sinus also helps in staging of the tumour .0
4.ENDOSCOPY of the nose and maxillary sinus will provide detailed examination ..
TREAMENT.
1.Surgery
2.Radiology
3.Chemotheraphy

1 .SURGERY

Maxillectomy
 two types
PARTIAL  Maxillectomy
1)vertical partial
2)horizontal partial Maxillectomy
TOTAL Maxillectomy
INCISION. 2 to 3 mm below lid margin not too far or not too close.

2. RADIOTHERAPY

a. External beam. X ray, beta rays or gamma rays using elements like co 60 and radium using proper precaution.
b. Interstitial. Beads or needle with radioactive material implanted in tumor by caldwell luc op.

3. CHEMOTHERAPY

a. Local. Through feeding vessels
b. Systemic. Used if there is distant metastasis or radio resistant or palliative care.
EG. Methotrexate, 5 fluro niacin, bleomycin

DIFFERENTIAL DIAGNOSIS

1. Malignant tumors
@lymphoreticular system tumors like lymphoma, extramedullary plasmocytosis
@olfactory neurofibroblastoma
@salivary gland malignant tumors like  adenoidcystic ca, adenocarcinoma etc.
@melanoma
@sarcomas like rhabdomyosarcoma, kaposi

2. Benign tumors
hemangioma, meningioma, chondroma, angiofibroma, amyloblastoma

3. Cherubism

Carbuncle

Carbuncle

CARBUNCLE= this is infective gangrene of subcutaneous tissue caused by staph. aureus.         Commonly occurs in diabetic pts, poor immunity, radiotherapy pts. A)sites- nape of neck is commonest followed by back, shoulder region.  B) PATHOLOGY=   1- initial lesion is in d form of hair follicle infection with perifolliculitis. 2-infection take virulent course n results in necrosis of subcutaneous fat wich give rise to multiple absbesses.  3- these abscesses r intercommunicating n open to exterior by multiple openings. 4- this appearance is called cribriform appearance.  C)CLINICAL FEATURES= 1- typically pt is diabetic. 2- severe pain n swelling in nape of neck. 3- fever with chills n rigors r severe.     4- surface is read, angry looking like red hot coal.  5- surrounding area is indurated. 6-cribriform apperance., 7-end result is development of crateriform ulcer with central slough.  D) TREATMENT= 1- diabetes control with injectible insulin.. 2- parenteral antibiotics given..cloxacillin, flucloxacillin, erythromycin n some cephalosporins.. 3- improve gen health of pt. 4- surgery required whn thr is pus. Cruciate incision is taken. Edges of skin flap r excised. Pus is drained. Loculi r broken down, slough is excised n cavity treated wid antiseptic agents.. E)COMPLICATIONS=1- worsening of diabetic status.    2-extensive necrosis of skin..  3-septicemia, toxaemia. 

Beriberi

Beriberi

Wet beriberi =high cardiac output failure.
Dry beriberi =type of peripheral neuropathy.

Wet beriberi
 1) oriental beriberi caused by eating diets in which most calories r derived 4m polished,highly milled rice.
 2) oedema=caused by metabolites like pyruvate nd lactate which cause extreme peripheral vasodilatation nd leakage of fluid 4m capillaries.
 3) beriberi heart disease charactorised by =
 -peripheral vasodilatation
 -high cardiac output state with warn extremities.
 -biventricular failure.
 -retention of Na nd water.
 -extreme tachycardia.
 -venous congestive state charactorised by raised jvp nd tender hepatomegaly.
 -hyperdynamic circulation.
 -dyspnoea
 -cardiomegaly.

   BIOCHEMICAL TESTS
 1)measurement of bld thiamine,pyruvate nd lactate levels.
 2)whole bld or erythrocyte transketolase activity.

   MANAGEMENT
1)complete bed rest.
2)100 mg thiamine IM 4 fist 7 days, then 10 mg/day orally 4 several months.

    DRY BERIBERI
1)nutritional polyneuropathy is charactorised by symmetric impairement of sensory,motor nd reflex functions dat affect distal segments of limbs more severly dan proximal ones.
 2)histology= non inflamatory degeneration of myelin sheaths.
 3) 3 types of nervous system involved in beriberi.
 - peripheral neuropathy.
 -  cerebral beriberi
 - Korsakoffs syndrome.

    INFANTILE BERIBERI
seen in breast feed infants of thiamine deficent mothers.Usual age of onset is 2-3 months.

BHP

BHP
Aetiopathogenesis_
there r 2 theories to explain BPH
1 Harmonal theory_as the age avances,the level of androges comes down.There is corresponding increase in the oestrogen which stimulates the prostate gland n produce BPH.
2 neoplastic theory_according to this theory there is proliferation of all the elements of prostate_fibrous,musculaq n glandular resulting in fibromyoadenoma.
Clinical features_
1 frequency
to start with,frequecy is present
duing the day time followed by day n night.-5 to 10 times during during the night.It is due ineffective emptyinp of the bladder.It result in residual urine in the bladder precipitating cystitis.
2 urgency_as the prostate enlarges there is visceral introversion of sensitive mucous membrane of prostatic urethra within the bladder.This causes the internal spincter to streth n prevents contraction.This result in urgency.
3 Hesitancy_pt hesitate to pass urine because micturation is not effective due to obstruction.
4 haematuria is rare.
5 BPH can b present with acute retention of urine
6 complications like stones,diverticuli,renal failure.
Diagnosis of BPH-per rectal examination-enlarge lat lobe can b easily felt.Rectal mucosa is free.
Investiagations_
1 blood urea n creatinine_ raised level indicate renal failure.
2 uroflowmetry_person is asked to void urine frm his full bladder into flowmeter.The flow rate is assesed.
Normal peak flow rate is 20 ml/sec.Doubtfull peak obstruction._10-15 ml/sec.
Definite peak obstruction_less than 10 ml/sec.
3  ultrasonogram-to asses the size n wt of prostate,to assess the residual urine n to look for hydrouretonephrosis
4 IVP to study the renal changes in selected cases.
5 cystoscopy as part of treatment.
Treatment_
1 medical treatment_
if the pt has mere frequency of micturation, n if the residual urine is not much,uroflowmetry shows more than 15 ml/sec of urine flow,the pt can b reassuared n advise to avoid heavy alcohol consumption which may lead to prostatic congestion n acute retention of urine.To avoid overdistension of bladder,he has to void the urine as n when he feel the urinary sensation of micturation.
Drugs_fibnasteride acetate
5 mg daily for 6 mönths.
Alpha adrenargic blocker relax the internal spincter for better drainage of the bladder.
2 surgical treatment_
indication_1 acute retention of urine
2 chronic retention of urine with posvoidal urine more than 200 ml
3 if the frequency of micturation is so much that it disturb the normal life style during day time.
4 complication like haematuria.
Surgical method-
1 transvesical suprapubic prostatectomy_this method is now restricted to gland more than 100 gm in wt.
Through an extraperitoneal approch the bladder is opened,prostate is enucleated with finger,bleeding is controlled by inflating foley bulb with about 30-50 ml of air n by ligature.
Bladder is drained by malecots catheter.
2 transurethral resection of prostate _most popular method today.
A rectoscope is passd to urethra n under vision with constant irrigation with water,prosate is resected into multiple piece n removed.Haemostasis is achieved with the help of cautery.
3 retropubic prostectomy.
4 perineal prostatectomy.

Biliary cirrhosis

Biliary cirrhosis

Biliary cirrhosis is the cirrhosis of the liver secondary to prolonged obstruction of biliary system, anywhere between the interlobular bile ducts and the papilla of Vater. Obstruction results in progressive destruction of bile ducts.

Primary biliary cirrhosis.
Occurs in chronic inflammation and obliteration of intrahepatic bike ducts.
Aetiology.
Mac predominantly in females in the middle age.
Due to immune reactions resulting in liver damage.

Pathology.
Chronic granulomatous inflammation destroying the interlobular ducts, Resulting in fibrosis and later cirrhosis of the livers and its complications.

Clinical features.
1. Cardinal features are pruritis, hyperpigmentation, and jaundice.
2. Liver involvement.
 Progressive jaundice, later becomes intense.
 Patients acquires a ' bottle green colour'.
 Scratch marks, froger clubbing.
 Hepatospleenomegaly.
 Hepatocellular failure, portal hypertension and ascitis.
3. Hypercholesterolaemia.
 Xanthelasmas around the eyes.
 Xanthomas over joints, tendons, hand creases, elbows and knees.
 Pain, tingling and numbness over feet and hands due to peripheral neuropathy resulting from lip infiltration of peripheral nerves.
4. Malabsorption
 steatorrhoea and diarrhoea  from malabsorption of fat.
 Easy bruising and ecchymosis from vitamin K deficiency.
 Hepatic osteodystrophy -
 might blindness due to vitamin A deficiency.

Investigations.
 Hyperbilirubinaemia of conjugated type.
 Mile elevation of transaminases.
 Two to five dole rise of serum alkaline phosphatase.
 Marked rise of serum 5'- nucleotidase activity
 hyperlipidaemia.
 More than 90% of the patients have antimitochondrial antibodies and increased levels of cryoproteins consisting of immune complexes.
 Antinuclear, antismooth muscle antibodies.
 Lives biopsy confirms the diagnosis.

Management.
Ursodeoxycholic acid(10-15mg/kg) improves bilirubin and aminotransferase values.
 Steroids may improve biochemical and histological disease but may lead to osteoporosis.
 Other therapies azathioprine, colchicine, methottrexate.
 Steatorrhoea is treated by limiting eat intake and substituting long chain triglycerides with medium chain triglycerides in the diet.
 Monthly injections of vitamin K.
 Vitamin D 1mg/day.
 Calcium supplementation in the form of calcium gluconate 2-4 g/day.
 Airpinsignocter to reduce osteoporosis.
 Lives transplantation.
 Management of pruritis.
 *cholestyramine 4-16 g/day.
 *Rifampicin, ondansetron and opiate antagonists.

Secondary biliary cirrhosis.
*results from prolonged obstruction to large bike duct by ;
 stones.
 Bile duct strictures.
 Sclerosing cholangitis.

Clinical features
1. Recurrent abdominal pain in stones.
2. Fluctuating jaundice in stones.3. Previous history of abdominal surgery in strictures.
4. Chronic cholestasis with episodes of ascending cholangitis and even lives abscess.
5. Right upper quadrant pain due to cholangitis or biliary colic
6. Cirrhosis, ascites and portal hypertension are late features.
Investigations.
1 hyperbilirubinaemia of the conjugated type.
2. Markedly elevated serum alkaline phosphatase activity.
3. Ultrasound and CT of abdomen.
4 .ERCP.
5. Lives biopsy.

Treatment.
1. Relief of obstruction to bike flow by ERCP or surgery.
2. Antibiotics in sclerosing cholangitis.