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General case

 Juhi Dhanawat
  Pratik Kumar
   Ashar Lodi
Ridhima Sakhuja
   Richa Singh
history
•   Name: Shreya
•   Age : 2.5yrs , sex: female
•   From: Shimoga
•   Informant: mother, Reliability : good
•   Came to RAPCC 15days back with
•   Chief complaints:
•   enlarged head , loss of vision , seizure since
    2yrs4months
History of presenting illness
• Child was apparently normal 2yrs4months back when she
  developed fever for which the mother gave paracetamol
  tablet. Fever decreased but spiked up again on the 8th day
  with one episode of seizure. There was no frothing of saliva
  or biting of tongue. The hands and legs became stiff which
  normalized within 2-3 minutes. Baby was admitted in the
  ICU at a local hospital ,treated and was discharged on
  medications for seizure, a tablet and syrup.
• Every time the mother gives the child head bath, she
  develops seizure.
• History of loss of vision since 2yrs 4 months.
• The child does not recognize the mother and no social
  smile present.
• No other episodes of fever. No history of vomiting.
• No history of difficulty in feeding .
• When the child did not improve, mother took her to
  manipal hospital 2months back where scans were done
  and mother was told an operation would be required.
  She was referred to RAPCC.
• No history of difficulty in chewing.
• No history of lateral gaze palsy.
• No history of hearing deficit, drooling of saliva, nasal
  regurgitation .
• History of difficulty in wearing napkins.
Birth history
• Antenatal history: primigravida
Ist trmester:Concieved spontaneously.
No history of fever with rash, burning
   micturition.
No history of exposure to radiation or intake of
   drugs.
Ultrasound scan not done.
2nd trimester: quickening felt at 6th month.
No history of fever with rash.
No history of GDM, PIH.
One USG scan done-nnormal
3rd trimester:
No history of GDM, PIH.
USG scan done-normal.
• Full term delivery.
• Elective caesarian section due to decrease in
  fetal movements.
• Baby cried immediately after birth.
• Birth weight:2.75 kg
• Meconium and urine passed within 24hrs.
• Breast feeding initiated after 4hrs of delivery.
• No postoperative complications.
Developmental history

• Gross motor- head control not achieved
•                baby cannot sit with support
• Fine motor- grasp reflex present.
• Language and communication: bysyllables
  (amma) only word spoken.
• Social: no social smile
•          does not recognise the mother
• Inference: global deveopmental delay
Immunization history
• Immunized for age
• Last vaccine taken: 1.5 yrs- DPT booster and
  OPV.
• Dietary history:
• Exclusive breast feeding till the age of
  5months.
• Weaning : 5th month, cerelac given.
• Presently eats from the family pot.
calories        protiens
8am                     5buiscuits+ I cup   100+ 130= 230   0+7=7
                        milk(200ml)
10am                    ½ dosa              60              1.5
1pm                     1 cup rice+I cup    175+50=225      4+ 2=6
                        curry
3pm                     1 fruit             50              0.5
5pm                     5buicuits +1cup     100+130=230     0+7=7
                        milk
7pm                     I cup rice+ 1 cup   175+50=225      4+2=6
                        curry
9pm                     1cup milk           130             7
total                                       1150            35
required                                    1150            19.5



Inference: no deficit
Family history
• Child born out of consanguinous marriage
Socio economic history
•   5 members in the family
•   3rooms, no over crowding
•    income rs 4000/month
•   Belongs to lower middle class family according
    to Modified Kuppuswamy scale.
Examination
General Condition
• A well nourished, conscious and confortable child
• Decreased alertness to surrounding

• VITALS
     - Afebrile
     - Pulse rate – 96/min
     - Respiratory Rate – 16/min
     - Blood Pressure- 110/ 80 mm hg
Anthropometry
• Weight
  – Actual-       12.5 kg
  – Expected-     13 kg
  – Inference –    96 % Normal (IAP)

• Height
  – Actual -      83 cm
  – Expected -    93 cm
  – Inference -   89 % Grade II stunting (Waterlow)
• BMI – 18 Kg/m2
• Mid Arm Circumference
  – Actual      18 cm
  – Expected    >13.5 cm

• Head Circumference
  – Actual –    55 cm
  – Expected    48 cm
  – Inference   Macrocephaly
Head to toe examination
• Pallor present (palpebral conjunctiva)
• No icterus
      Clubbing
      Cyanosis
      Lymphadenopathy
      Edema
• Head Circumference of 55 cm (macrocephaly)
• Prominent forhead
• No dilated scalp veins
• Anterior Fontanelle – Open, non pulsatile, in
  level, 4x4 cm
• Normal hair distribution and growth
• Eyes- Setting sun sign present
       - Unresponsive to light
Gc  hydrocephalus
•   Normal facial feature
•   Limbs- Increased tone
•   Chest – normal
•   Spine – normal
•   Abdomen – normal
•   Genitals – normal
•   No skin abnormalities
Developmental Assesment
• Expected for age (2.5 yr)

Gross Motor
       - Runs well, Climb stairs
Fine Motor
       - Turns Pages, Dress her/himself
Social
       - Dry by day, listen to stories
Language
       - 3 word simple sentences, refer to self as “I”
Developmental
          assessment(obsereved)
• Gross Motor
  – No head control
  – Cant sit with support
  – DQ- 14%
• Fine motor
  – Grasps finger
  – Can hold objects in one hand
  DQ- 17 %
• Social
  – No social smile
  – Day time bed wetting present
  – DQ- 14%
• Language
  – Monosyllables
  – DQ- 30 %

  – Global Developmental Delay
Systemic Examination
Central Nervous system examination

- Conscious, disinterested in surrounding
- Spastic response to sudden loud sound
- Skull and Spine- no deformity
Cranial nerve examination
I.              Not done
II.             Pupils reactive
                Menace reflex- Absent
III, IV, VI.    Could not be assessed
V.              Normal B/L
VII.            No facial palsy
VIII.            Could not be assessed
IX, X, XI, XII.     Not assessed
Motor System Examination
               Right              Left
               Upper/Lower        Upper/Lower
Nutrition      Normal/ Normal     Normal/Normal

Tone           Increased/Increase Increased/Increase
               d                  d
Power          Grade 3/ Grade 3 ? Grade 3/ Grade 3 ?
Sensory system
• Could not be assessed

• Reflexes
     Superficial     Right     Left
     • Corneal       Normal    Normal
     • Abdominal     Normal    Normal
     • Plantar       Upgoing   Upgoing
Deep refelexes
                        Right       Left
•   Triceps             Grade 2            Grade 2
•   Biceps              Grade 2            Grade 2
•   Knee                Grade 3            Grade 3
•   Ankle               Grade 3            Grade 3

Visceral- No bladder control
Gait- could not be assessed, scissoring of lower
limbs present.
• Respiratory System
- Trachea central, B/L symmetrical chest
  movements
- Normal vesicular breath sound heard
- No added sounds
• Cardiovascular system examination
  – PR- 90/min
  – Apex beat- 5th ICS medial to mid clavicular line
  – S1 S2 heard
  – No murmur
• Per abdomen

Abdomen- soft, non tender
       no organomegaly
Summary
•   Decreased alertness to surrounding
•   Grade II Stunting
•   Macrocephaly with open ant fontanelle
•   Setting sun sign, loss of vision, Pallor
•   Hypertonia
•   Severe Global developmental delay
•   Grade 3 lower limb reflexes
DIFFERENTIAL DIAGNOSIS OF
       LARGE HEAD
• MEGALENCEPHALY :
  - No signs of increased intracranial pressure.
  - Ventricles are not large, nor under increased
   pressure
  Causes :
 1. Hurler’s syndrome
 2. Metachromatic leukodystrophy
 3. Tay Sach’s disease
• Chronic Subdural hematoma :
 - Causes large head,mostly located in the parietal
   region without prominent scalp veins or sunset
   sign.
• Others :
 1. Hydranencephaly
 2. Rickets
 3. Achondroplasia
 4. Hemolytic anemias
 5. Familial macrocephalies
INVESTIGATIONS
Haematological investigations
• Haemoglobin

• Total count and differential count

• ESR

• Platelets
In our patient
• Haemoglobin- 12.2g%

• Total count-11,800/cc

• Differential count
  N-32% L-59% M-6% E-3%

• Platelets-2.3 lakh/cumm
Biochemical investigations
• Electrolytes

• Serum urea and creatinine

• Liver function tests
In our patient
• Electrolytes
 Na⁺ 143meq/L (136-149meq/L)
 K⁺ 4.4meq/L (3.5-5.3meq/L)
 Cl⁻ 107.3meq/L (98-111meq/L)
 HCO₃⁻ 23.3meq/L (23-27meq/L)
• Serum urea-26mg/dl (5-18mg/dl)
INCREASED
• Serum creatinine-0.4mg/dl (0.3-0.7mg/dl)
• Liver function tests
  total bilirubin-0.1mg/dl (0.2-1.2mg/dl)
  direct bilirubin-0.0mg/dl (upto 0.3mg/dl)
Radiological investigations


• CT scan of brain
• Ultrasound
• MRI
In our patient
• CT scan brain
   grossly distended lateral,3rd ,4th ventricles
   communicating hydrocephalus
Other investigations
•   CSF analysis
•   Urine screening tests
•   Visual assessment
•   Hearing assessment
•   EEG
•   EMG
•   Metabolic work-up
In our patient
• Hearing assessment
 BERA
 OAE
 Intermittance
Impression-bilateral adequate hearing for
  speech and language development
TREATMENT
TREATMENTMENT OF
          HYDROCEPHALUS
 MEDICAL TREATMENT
-It provides temporary relief & includes the use
 of drugs which act either by decreasing CSF
 secretion by choroid plexus (Acetazolamide &
 loop diuretics ) , or by increasing CSF
 resorption (isosorbide).
• Since, the increase in head size is associated with
   progressive symptoms, therefore it is necessary to
   intervene surgically.
1. VENTRICULO-PERITONEAL SHUNT
  -CSF directly drained into circulation or peritoneal
     cavity.
  -Advantage- shunt need not be lengthened as the child
     grows.
2. VENTRICULO ATRIAL SHUNT ( VASCULAR SHUNT )
3. VENTRICULOSTOMY
  -by endoscopic approach.

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Gc hydrocephalus

  • 1. General case Juhi Dhanawat Pratik Kumar Ashar Lodi Ridhima Sakhuja Richa Singh
  • 2. history • Name: Shreya • Age : 2.5yrs , sex: female • From: Shimoga • Informant: mother, Reliability : good • Came to RAPCC 15days back with • Chief complaints: • enlarged head , loss of vision , seizure since 2yrs4months
  • 3. History of presenting illness • Child was apparently normal 2yrs4months back when she developed fever for which the mother gave paracetamol tablet. Fever decreased but spiked up again on the 8th day with one episode of seizure. There was no frothing of saliva or biting of tongue. The hands and legs became stiff which normalized within 2-3 minutes. Baby was admitted in the ICU at a local hospital ,treated and was discharged on medications for seizure, a tablet and syrup. • Every time the mother gives the child head bath, she develops seizure. • History of loss of vision since 2yrs 4 months. • The child does not recognize the mother and no social smile present.
  • 4. • No other episodes of fever. No history of vomiting. • No history of difficulty in feeding . • When the child did not improve, mother took her to manipal hospital 2months back where scans were done and mother was told an operation would be required. She was referred to RAPCC. • No history of difficulty in chewing. • No history of lateral gaze palsy. • No history of hearing deficit, drooling of saliva, nasal regurgitation . • History of difficulty in wearing napkins.
  • 5. Birth history • Antenatal history: primigravida Ist trmester:Concieved spontaneously. No history of fever with rash, burning micturition. No history of exposure to radiation or intake of drugs. Ultrasound scan not done.
  • 6. 2nd trimester: quickening felt at 6th month. No history of fever with rash. No history of GDM, PIH. One USG scan done-nnormal 3rd trimester: No history of GDM, PIH. USG scan done-normal.
  • 7. • Full term delivery. • Elective caesarian section due to decrease in fetal movements. • Baby cried immediately after birth. • Birth weight:2.75 kg • Meconium and urine passed within 24hrs. • Breast feeding initiated after 4hrs of delivery. • No postoperative complications.
  • 8. Developmental history • Gross motor- head control not achieved • baby cannot sit with support • Fine motor- grasp reflex present. • Language and communication: bysyllables (amma) only word spoken. • Social: no social smile • does not recognise the mother • Inference: global deveopmental delay
  • 9. Immunization history • Immunized for age • Last vaccine taken: 1.5 yrs- DPT booster and OPV. • Dietary history: • Exclusive breast feeding till the age of 5months. • Weaning : 5th month, cerelac given. • Presently eats from the family pot.
  • 10. calories protiens 8am 5buiscuits+ I cup 100+ 130= 230 0+7=7 milk(200ml) 10am ½ dosa 60 1.5 1pm 1 cup rice+I cup 175+50=225 4+ 2=6 curry 3pm 1 fruit 50 0.5 5pm 5buicuits +1cup 100+130=230 0+7=7 milk 7pm I cup rice+ 1 cup 175+50=225 4+2=6 curry 9pm 1cup milk 130 7 total 1150 35 required 1150 19.5 Inference: no deficit
  • 11. Family history • Child born out of consanguinous marriage
  • 12. Socio economic history • 5 members in the family • 3rooms, no over crowding • income rs 4000/month • Belongs to lower middle class family according to Modified Kuppuswamy scale.
  • 14. General Condition • A well nourished, conscious and confortable child • Decreased alertness to surrounding • VITALS - Afebrile - Pulse rate – 96/min - Respiratory Rate – 16/min - Blood Pressure- 110/ 80 mm hg
  • 15. Anthropometry • Weight – Actual- 12.5 kg – Expected- 13 kg – Inference – 96 % Normal (IAP) • Height – Actual - 83 cm – Expected - 93 cm – Inference - 89 % Grade II stunting (Waterlow)
  • 16. • BMI – 18 Kg/m2 • Mid Arm Circumference – Actual 18 cm – Expected >13.5 cm • Head Circumference – Actual – 55 cm – Expected 48 cm – Inference Macrocephaly
  • 17. Head to toe examination • Pallor present (palpebral conjunctiva) • No icterus Clubbing Cyanosis Lymphadenopathy Edema
  • 18. • Head Circumference of 55 cm (macrocephaly) • Prominent forhead • No dilated scalp veins • Anterior Fontanelle – Open, non pulsatile, in level, 4x4 cm • Normal hair distribution and growth • Eyes- Setting sun sign present - Unresponsive to light
  • 20. Normal facial feature • Limbs- Increased tone • Chest – normal • Spine – normal • Abdomen – normal • Genitals – normal • No skin abnormalities
  • 21. Developmental Assesment • Expected for age (2.5 yr) Gross Motor - Runs well, Climb stairs Fine Motor - Turns Pages, Dress her/himself Social - Dry by day, listen to stories Language - 3 word simple sentences, refer to self as “I”
  • 22. Developmental assessment(obsereved) • Gross Motor – No head control – Cant sit with support – DQ- 14% • Fine motor – Grasps finger – Can hold objects in one hand DQ- 17 %
  • 23. • Social – No social smile – Day time bed wetting present – DQ- 14% • Language – Monosyllables – DQ- 30 % – Global Developmental Delay
  • 24. Systemic Examination Central Nervous system examination - Conscious, disinterested in surrounding - Spastic response to sudden loud sound - Skull and Spine- no deformity
  • 25. Cranial nerve examination I. Not done II. Pupils reactive Menace reflex- Absent III, IV, VI. Could not be assessed V. Normal B/L VII. No facial palsy VIII. Could not be assessed IX, X, XI, XII. Not assessed
  • 26. Motor System Examination Right Left Upper/Lower Upper/Lower Nutrition Normal/ Normal Normal/Normal Tone Increased/Increase Increased/Increase d d Power Grade 3/ Grade 3 ? Grade 3/ Grade 3 ?
  • 27. Sensory system • Could not be assessed • Reflexes Superficial Right Left • Corneal Normal Normal • Abdominal Normal Normal • Plantar Upgoing Upgoing
  • 28. Deep refelexes Right Left • Triceps Grade 2 Grade 2 • Biceps Grade 2 Grade 2 • Knee Grade 3 Grade 3 • Ankle Grade 3 Grade 3 Visceral- No bladder control Gait- could not be assessed, scissoring of lower limbs present.
  • 29. • Respiratory System - Trachea central, B/L symmetrical chest movements - Normal vesicular breath sound heard - No added sounds
  • 30. • Cardiovascular system examination – PR- 90/min – Apex beat- 5th ICS medial to mid clavicular line – S1 S2 heard – No murmur
  • 31. • Per abdomen Abdomen- soft, non tender no organomegaly
  • 32. Summary • Decreased alertness to surrounding • Grade II Stunting • Macrocephaly with open ant fontanelle • Setting sun sign, loss of vision, Pallor • Hypertonia • Severe Global developmental delay • Grade 3 lower limb reflexes
  • 34. • MEGALENCEPHALY : - No signs of increased intracranial pressure. - Ventricles are not large, nor under increased pressure Causes : 1. Hurler’s syndrome 2. Metachromatic leukodystrophy 3. Tay Sach’s disease
  • 35. • Chronic Subdural hematoma : - Causes large head,mostly located in the parietal region without prominent scalp veins or sunset sign. • Others : 1. Hydranencephaly 2. Rickets 3. Achondroplasia 4. Hemolytic anemias 5. Familial macrocephalies
  • 37. Haematological investigations • Haemoglobin • Total count and differential count • ESR • Platelets
  • 38. In our patient • Haemoglobin- 12.2g% • Total count-11,800/cc • Differential count N-32% L-59% M-6% E-3% • Platelets-2.3 lakh/cumm
  • 39. Biochemical investigations • Electrolytes • Serum urea and creatinine • Liver function tests
  • 40. In our patient • Electrolytes Na⁺ 143meq/L (136-149meq/L) K⁺ 4.4meq/L (3.5-5.3meq/L) Cl⁻ 107.3meq/L (98-111meq/L) HCO₃⁻ 23.3meq/L (23-27meq/L) • Serum urea-26mg/dl (5-18mg/dl) INCREASED • Serum creatinine-0.4mg/dl (0.3-0.7mg/dl) • Liver function tests total bilirubin-0.1mg/dl (0.2-1.2mg/dl) direct bilirubin-0.0mg/dl (upto 0.3mg/dl)
  • 41. Radiological investigations • CT scan of brain • Ultrasound • MRI
  • 42. In our patient • CT scan brain grossly distended lateral,3rd ,4th ventricles communicating hydrocephalus
  • 43. Other investigations • CSF analysis • Urine screening tests • Visual assessment • Hearing assessment • EEG • EMG • Metabolic work-up
  • 44. In our patient • Hearing assessment  BERA  OAE  Intermittance Impression-bilateral adequate hearing for speech and language development
  • 46. TREATMENTMENT OF HYDROCEPHALUS MEDICAL TREATMENT -It provides temporary relief & includes the use of drugs which act either by decreasing CSF secretion by choroid plexus (Acetazolamide & loop diuretics ) , or by increasing CSF resorption (isosorbide).
  • 47. • Since, the increase in head size is associated with progressive symptoms, therefore it is necessary to intervene surgically. 1. VENTRICULO-PERITONEAL SHUNT -CSF directly drained into circulation or peritoneal cavity. -Advantage- shunt need not be lengthened as the child grows. 2. VENTRICULO ATRIAL SHUNT ( VASCULAR SHUNT ) 3. VENTRICULOSTOMY -by endoscopic approach.