Case presentationDr.Eman Aly Mohamed

Specialist of pediatrics & neonatologyEL NASR N.I.C.U

Female baby called Ganna neseem ,27 days old. C.S delivery ,not attended delivery ,G2-p2 birth date : 9/12 /2011 Gestational age : 34 w. (+/-) 2 w. was referred to our NICU from other hospital by : Severe respiratory distress. Cyanosis. Persistent vomiting. Persistent hyperglycemia.

History

Chest xray CBC Blood glucoseTreatment: Antibiotics: cefotax,unictam N.G feeding &O2 by head box.Response to treatment was poor with persistent hyperglycemia and refer to our NICU .

Investigations done in the referring hospital as mentioned in report

Admission date : 28/12 . The baby was severely hypoactive and

depressed reflexes. Central cyanosis Waisted baby with loss of s.c fat of

abdominal wall &both thighsFace ex.: no apparent abnormal features

except low hair line.Mouth ex.: normal.

Examination on admission to our nicu

Chest ex.: Tachypnea, R.R 65/min Intercostals &subcostal retractions Fair air entery on both sides of chest wall Fine consonating &coarse cripits (bilateral)Heart ex.: H.R 135 beat /min. S1&S2 normal. No abnormal sounds.

Chest & heart ex.

Abdomen is lax. Loss of subcutaneous fat. Loss of skin elasticity. Liver (++) below costal margin ,soft consistency, rounded border .

Passes urine&stools

Abdominal ex.

Lower limbs ex . : Lax, no oedema Loss of subcutaneous fat over both

thighs , no deformities. Upper limb ex. : Mild hypertonia,, fisting of both hands no

deformities. Back&spines: Normal ,no deformities ,no masses External genitalia: normal appearance

Limbs ex.

Length: 46cm.Weight: 1.9kg.on admission , birth weight

was 2.5 kg.Chest circumference: 29cm. Skull circumferernce: 33 cm. on admission to our nicu. 34 cm. on stay in our unite Anterior fontanelle: soft,slightly

depressed,3x5 cm. in size

Measurements

Plain x ray (chest&heart): bronchopneumonic patches Blood glucose: hyperglycemia Acetone in urine: absent Serum insulin: 2 micro unit /ml. Normal : 6 -24 micro unit /ml. C-peptide : 0.6ng /ml. Normal( 0.9-4) ng/ml.

Investigations

Plain x ray chest &heart

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Profile of blood glucose analysis

C.B.C : W.B.C count: 5.7 x 10 3/cubic mm R.B.C count: 2.9 x 10 3/cubic mm Hgb: 10.4 g/L H.c.t.: 30.7 Plt. 35 x 10 3/mm3 Differential count: - Lymph 26.2 % - Mon 21. 5 % - Gra 52.3 %Another C.B.C: normal after blood

transfusion

Sodium : 126 mmol/L (135-150 mmol/L)

Potassium: 4.5mmol/L (3.5 -4.5 mmol/L)

Calcium: 10.4 (8.1-10.4mg)

Serum electrolytes:

Kidney functions test: Urea : 18 normal :(15-45mg %) Creatinine: 0.7 normal(0.3-1.3mg %)Liver functions test:

SGPT(ALT): 15 normal up to 45u/ml. SGPT(AST): 39 normal up to 40 u/ml. S. bilirubin : total : 5.5 normal up to 1.2n.g direct: 0.8 normal up to 0.25n.g

Marked hepatomegally with diffuse increased parenchymal density

Conclusion: signs suggestive of diffuse parenchymal liver disease for lab.correlation&biopsy.

C.T (liver) :

C.T liver

C.T liver

M.R.I. liver

M.R.I Livernormal

CT brain was ordered because of association of central abnormalities with cases of neonatal hyperglycemia

Contrast C.T. Brain

Large defined cystic lesion of c.s.f density is seen at RT. Tempro-parietal region connected to the atrium of the rt. Lateral ventricle

It is seen surrounded by mild degree of interstitial oedema (csf permeation)

It measures about 4x3.5 cm.

Contrast C.T.Brain:

No evident related soft tissue masses.

It exerts mild mass effect in the form of compression upon the third ventricle &minimal leftward shift of the mid line structures

Conclusions: Signs cope with large RT.tempro-parietal proncephalic cyst.

Cont. C.T brain

Neurosurgical consultation:Supratentorial arachnoid cyst attached to the RT. Lateral ventricle &effaced if less likely (pitocyst-AC)for M.R.I&contrast

M.R.I Brain :The same finding as C.T.

8 days after admission, the baby starts to develop subtle convulsions in the form of (pedaling & recycling ) alternating with tonic clonic convulsions of both upper limbs ,respond to phenobarbitone .

In addition to 3 attacks of generalized tonic clonic convulsions associated with hypoglycemia as a complications of insulin infusion respond rapidly to I.V. GL. 10%

Respiratory: C.P.A.P by nasal bronge with pressure

5 mm.Hg, oxygen 21% for 3 days. The baby was Shifted to H.B.

5L/min.for 1 day. Antibiotics : Unictam+cefatriaxone for 7 days

followed by Vancomycin +gentamycin Nebulizer with ventolin

Management

Glucose : We start with GL.10%with Na &K&Ca , Then reduce gl. concentration to 7%

and then to 5%. We modulate G.I.R according to

results of blood glucose starting with 7mg/kg/min. till 5.5 mg/kg/min.

A.A (panamin g. 0.5 gm./kg/day) lipids (lipovenous 0.5 gm./kg./day)

I.V.ifusion of fluids :

With persistent hyperglycemia even with G.I.R 5.5mg/kg/min.

we start insulin infusion in a dose of 0.02 u/kg/hour.

in spite of this minimal dose of insulin the baby developed hypoglycemia

so we have to increase G.I.R to 7.5 mg/kg/min. with insulin infusion till stabilization of blood glucose

Insulin infusion:

With stabilization of the baby ,we start gradual nasogastric feeding followed by complete oral feeding .

As soon as oral feeding is completed blood glucose returns to normal values.

Nasogastric feeding

Blood transfusion :15 ml./kg. Plasma transfusion :15 ml/kg. I.V. phenobarbitone followed by oral phenobarbitone.

Surgical drainage of cyst &CSF by shunt operation.

C.T brain after shunt operation

C.T brain after shunt operation

After shunt Before shunt

Thank you