Case presentation, baby with hyperglycemia & brain cyst. By dr Iman Ghabn, El Nasr hosp, Port said, Egypt

1. Case presentation
Dr.Eman Aly Mohamed
Specialist of pediatrics & neonatology
EL NASR N.I.C.U

2.  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.

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

4.  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 thighs
 Face ex.: no apparent abnormal features
except low hair line.
 Mouth ex.: normal.

6. 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.

7.  Abdomen is lax.
 Loss of subcutaneous fat.
 Loss of skin elasticity.
 Liver (++) below costal margin ,soft
consistency, rounded border .
 Passes urine&stools

8.  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

10.  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

11.  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.

14. Series
Series
Series
Series
Series
Series
Series
Series
Series
Series
Series
Series
Series
PM
AM
AM
AM
AM
AM
AM
PM
PM
PM
PM

15.  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

16.  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)

17.  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

18.  Marked hepatomegally with
diffuse increased parenchymal
density
 Conclusion: signs suggestive of
diffuse parenchymal liver disease
for lab.correlation&biopsy.

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

25.  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.

26.  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.

27. 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.

28.  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%

29. 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

30. 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)

31.  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

32.  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.

33.  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.

36. After shunt Before shunt