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					PEDIA-NEURO
CONFERENCE
 May 12, 2009
General Data
•   Z. S., 6 month old male
•   Filipino
•   Cagayan Valley
•   Methodist
•   Date of Birth: Nov. 6, 2008
•   Date of Admission: May 4, 2009
•   Informant: Mother
•   Reliability: good
Chief Complaint
• Progressive enlargement of the head
History of Present Illness
• Maternal History
  – Born to a 19 year old
  – G1P0
  – First prenatal check-up at 7 months AOG
      • UTZ (2x): (+) hydrocephalus
  – (+) Urinary tract infection on the 3rd trimester, given Amoxicillin
    500mg/tab x TID x 7 days
  – (-) TORCH screening done
  – (-) Roentgen or chemical exposure, drug intake
  – Non-smoker, non-alcoholic beverage drinker, no illicit drug use
  – OGTT, Hepa B screening not done
  – Folic acid and FeSo4
History of Present Illness
• Birth History
  – Term (36-37 AOG)
  – Via C/S
  – Tertiary hospital, OB-Gyn
  – Birth weight: 2.5 kg
History of Present Illness
• Neonatal History
  – Good cry, spontaneous motor activity
  – Normal head circumference
  – (-) Seizures, infections
  – NICU for 6 days ~ monitoring
    • 4 days post-partum
       – CT Scan: Findings in the left temporo-parietal and left
         occipital areas may be suggestive of arachnoid or
         porencephalic cyst
History of Present Illness

                             • Mother noticed enlargement of
  4 MONTHS PTA                 the head with accompanying
                               soft, tender mass on the root of
                               the nose
                             • No consult done



                             • Progression of the head size and
                               mass on the nose
                             •Consult was done (local doctor)
   2 weeks PTA               •Referred to another institution
                             • Consult at Surgery OPD
                             • MRI done


                 ADMISSION
      MRI Results (4/27/09)
• Marked hydrocephalus
• Consider Porencephalic cyst
  communicating with the left lateral
  ventricle
• Frontoethmoidal encephalocoele
• Possible arachnoid cyst superoposterior to
  the cerebellum
Review of Systems
• General: active movements, good
             appetite
• Cutaneous: (-) jaundice, (-) active dermatosis
• Cardiovascular: no cyanosis, fainting spells
• Respiratory: no cough
• Gastrointestinal: no vomiting, diarrhea, constipation, milk intolerance
• Genitourinary: light to dark yellow urine, no discharge, no edema
• Endocrine: no breast asymmetry, discharge, palpitations, polyuria
• Nervous/Behavioral: no tremors, sleep problems, convulsions,
                         paralysis, behavioral changes, eating problems
• Musculoskeletal: no muscle or joint swelling
• Hematopoietic: no pallor, bleeding manifestations, easy bruisability
Past Medical History
• Feeding
  – Multivitamins
  – Breastfeeds every 2-3 hours, 3-5 minutes
    each
Past Medical History
• Growth and Development:
  – reaches with either hand
  – transfers to other hand
  – laughs and plays with examiner
  – (–) roll over
  – (–) cannot be pulled to sitting position
  – (–) lift head on prone
  – (–) good head control
Past Medical History
• Immunizations
  – BCG, Hepatitis B 1, Hepatitis B 2, OPV1,
    OPV2, DPT 1, DPT 2
  – At a local health center
• Past Illnesses: none
Family History
•   (-) hydrocephalus
•   (-) congenital defects
•   (+) HPN – grandfather, mother side
•   (-) DM
•   (-) Heart disease
•   (-) CA
•   (-) Asthma, allergies
•   (-) Seizures
•   (-) Neurologic or psychiatric illnesses
•   (-) TB
PHYSICAL EXAMINATION
• Alert, awake, not in CP distress, well-nourished,
  well-hydrated
• PR=108 bpm RR=36 cpm T=36.5°C
  Wt=9 kg (p75-90) Lt=69 cm (p75)
  HC=59.5 cm (>p95)
• Warm moist skin
• Bulging anterior fontanel 8cm x 17cm, wide
  sutures, distended scalp veins, (+)
  transillumination of the left side of the head, (+)
  Macewen sign, (-) bruits
PHYSICAL EXAMINATION
• Pink palpebral conjunctivae, anicteric sclerae,
  pupils 2-3mm ER, (+) setting sun sign
• No tragal tenderness, no aural discharge, non-
  hyperemic EAC, intact TM
• (+) 3cm x 2cm soft, tender, hyperpigmented
  mass on the nasal bridge, midline septum, no
  nasal discharge
• Moist buccal mucosa, non-hyperemic posterior
  pharyngeal wall, tonsils not enlarged
PHYSICAL EXAMINATION
• Supple neck, no palpable cervical lymph nodes,
  no anterior neck mass, trachea midline
• Symmetrical chest expansion, no retractions,
  resonant, clear and equal breath sounds
• Adynamic precordium, AB at 4 th LICS, no
  murmurs
• Globular abdomen, NABS, soft non-tender,
  tympanitic, liver palpable 1 cm below the
  subcostal region
• Bilaterally descended testes, no phimosis, no
  discharge
• Pulses full and equal, no cyanosis, no edema
NEUROLOGIC EXAMINATION
• Alert, awake
• Cranial Nerves
  – (+) ROR
  – Visual pursuit from side to side
  – Gross hearing intact
  – (+) Facial symmetry when smiling and crying
  – Uvula midline
  – Tongue midline on protrusion
NEUROLOGIC EXAMINATION
• Motor examination
  – Posture: flexed
  – Symmetrical movement of extremities
• Developmental reflexes:
  – (+) Moro reflex
  – (+) Palmar and plantar grasp reflex
  – (–) Rooting and sucking reflex
                 Salient Features
•   6 month old male
•   prenatal diagnosis of hydrocephalus
•   progressive enlargement of the head
•   soft, tender mass on the bridge of the nose
•   head circumference = 59.5 cm (>p95)
•   bulging anterior fontanel 8cm x 17cm
•   distended scalp veins
•   (+) transillumination of the left side of the head
•   (+) Macewen sign
•   (+) Setting sun sign
        Approach to Diagnosis
• Look for a symptom, sign or laboratory finding pointing
  to an organ or part of an organ system
       Presenting Manifestations
•   Progressive head enlargement
•   (+) Transillumination of the left side of the head
•   (+) Macewen sign
•   (+) Setting sun sign
•   Soft, tender mass on the bridge of the nose
  Causes of Macrocephaly from
     Birth through 6 Months
Hydrocephalus         Subdural collection           Familial Macrocephaly
Accumulation of CSF   Often either a hematoma       Autosomal dominantly
                      from traumatic or difficult   transmitted
                      deliveries or an effusion
                      following meningitis
       Clinical Assessment
• Hydrocephalus
• Encephalocoele
Discussion
CSF Circulation
     Lateral Ventricle
              Foramen of Monroe
      Third Ventricle
              Cerebral aqueduct
     Fourth Ventricle
              Foramen of Luschka and Magendie
   Subarachnoid Space


      Arachnoid Villi


     Venous Sinuses


   Internal Jugular Vein
            Hydrocephalus
• Diverse group of conditions that result
  from impaired circulation and absorption of
  CSF, or in the rare circumstance, from
  increased production by a choroid plexus
  papilloma
                     Hydrocephalus




Increased CSF production          Decreased CSF drainage




                           Communicating        Non-communicating
           Sites of Obstruction
  Lateral Ventricle
           Foramen of Monroe
   Third Ventricle                           Non-communicating
           Cerebral aqueduct
  Fourth Ventricle
           Foramen of Luschka and Magendie
Subarachnoid Space


   Arachnoid Villi


  Venous Sinuses


Internal Jugular Vein
           Sites of Obstruction
  Lateral Ventricle
           Foramen of Monroe
   Third Ventricle
           Cerebral aqueduct
  Fourth Ventricle
           Foramen of Luschka and Magendie
Subarachnoid Space


   Arachnoid Villi
                                             Communicating
  Venous Sinuses


Internal Jugular Vein
         Clinical Manifestations
• Irritability, lethargy, poor appetite, vomiting, poor head
  control
• Full and bulging fontanels
• Enlargement and engorgement of scalp veins
• Macewen’s sign
• 6th nerve palsy
• Setting sun sign
• Hyperactive reflexes
• Widened cranial sutures
• Symptoms of increased ICP (in older children)
                         Plans
• DIAGNOSTIC
  – CBC, PT/aPTT, CXR, MRI
• THERAPEUTIC
  – MEDICAL
    • Azetazolamide
    • Furosemide
  – SURGICAL
    • Ventriculoperitoneal shunt
               Prognosis
• Depends on the size of the of the dilated
  ventricles not the size of the head
• Mean intelligence quotient is reduced
• Performance tasks > verbal abilities
• Abnormalities in memory functions
• Visual problems
• Some show aggressive and delinquent
  behavior
     Other Findings on Imaging
              Studies
• Porencephalic cyst
• Arachnoid cyst
             Porencephalic Cyst
• CSF-filled cystic cavitation within the cerebral
  hemisphere in communication with the subarachnoid
  space or the ventricular cavity
• Caused by trauma, infarction, post-surgical resection,
  infection or congenital malformation (developmental
  abnormalities of cell migration)
• Manifestations depend on the location
   – Intellectual, motor, sensory deficits
• Skull prominence on the area of the cyst
               Arachnoid Cyst
• CSF-filled closed cystic malformation within the
  arachnoid that does not communicate with the
  subarachnoid space or ventricular cavity
• Most do not produce symptoms
               Encephalocoele
• Sac plus cerebral cortex, cerebellum, or portions of the
  brainstem
• Possible sites
   – Occipital
   – Fronto-ethmoidal
   – Basal
   – Posterior fossa
Thank You!

				
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posted:8/23/2011
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