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Perinatal Asphyxia with Adrenal Haemorrhage and Shock

Dr. Dinakara Prithviraj, , Dr. Vivek Chail,Dr. Dr. Manjunath CB, Rasheed Dr. Bhagya, Dr. Swagata M, Aravind N
Neonatal ICU, Dept of Pediatrics, Dept of Radiology. Vydehi institute of medical Sciences  and research centre.Whitefield, Bengaluru Phone no : 080-28413385-89 ext 273
Email drdinakar.nishanth@gmail.com, vchail@gmail.com

Live term male baby born by LSCS Ind – failed induction and fetal distress on 10.7.10 at 3:10 pm in peripheral hospital weighing 3 kgs. Baby was flaccid and required ambu bagging and chest compressions for 3-4 mins. Following this baby had improved.APGAR not known.
Baby shifted to mother’s side, was feeding well.On the 2nd day, baby developed weak cry, poor feeding and reduced activity and was admitted in medical college NICU. Baby developed hypoglycemia with convulsions. It was corrected with glucose administration ,  ,the baby was given Inj calcium gluconate(2ml/kg). Still convulsions persisted phenobarbitone  even with this medications convulsions persisted ,then phenytoin was given. Inspite of all these the convulsions still persisted, and the baby was put on midazolam infusion. Noted gradually baby was becoming pale, having irregular respiration, Spo2 was fluctuating, given supplemental oxygen,
Reports showed:

USG brain – normal study
Hb: 19.7g/dl; HCT: 60.7%; WBC: 21,300c/Cumm; P72, L20, E03; 
Platelets: 1.56 lakhs/cumm ; PS :microcytic normochromic blood picture
S.calcium:6.5mg/dl ; Blood urea :52mg/dl ; S.creatinine0.7mg/dl,
PT:3 mins ;APTT >5mins 2secs(prolonged)

Inspite of all the above measures baby continued to have convulsions and hypoglycemia. The baby was discharged against medical advice and was admitted in our hospital .
At the time of admission ; baby appeared pale, HR -200/min, RR-40-68/min irregular breathing, BP-30/20 mmHg, GRBS- 30 mg/dl, Temp- Hypothermia, CFT>5 secs, Icterus up to lower thighs, pupils mildly dilated,reacting to light, anterior fontanalle at level.
Systemic examination: cardia – tachycardia, no murmur, feeble peripheral pulse ; respiratory – irregular respiration ; abdomen – soft, distended abd girth 36 cm, noted bilateral huge mass near the flanks ?renal ; cns – weak cry, hypotonic ,Baby was developing multifocal convulsions.
Hypoglycemia corrected .Iv fluids started, 10%dextrose , Shock treated with 20 ml/kg NS. Baby put on Bubble CPAP with FiO2 60%, pressure 4 cm of H2O. Calcium gluconate 2 ml/kg. Ionotrops started
Reports – Hb:17.8g/dl , HCT:51.8% , WBC:15400c/cumm , Platelets:1.3 lakhs, CRP:negative , Urea:49mg/dl, S.creatinine:0.56 mg/dl, Na:133.9mg/dl, K:3.7mg/dl, Cl:106mg/dl. LFT: normal study. S.calcium:8.6 mg/dl. TSH: 4.07micro IU/ml.           Bilirubin: 14 mg/dl ;
USG Brain – normal study
USG Abdomen – B/l huge adrenal hemorrhage Right side 3.6 x 3.1cm;;. Left side 1.5x1.3cm.
At this juncture , HIE with b/l adrenal hemorrhage and consumptive coagulopathy was suspected.
Baby was treated with 2 mg iv Inj Vit K. Group specific fresh frozen plasma started and continued for 3 days. Baby passing urine & stool adequately. Blood glucose monitored meticulously and maintained within normal limits
Ionotrops gradually tapered and stopped. Removed from Bubble CPAP, started supplemental O2. Electrolytes were monitored alternate days which were normal. Phototherapy was given for 6 days. SpO2 was normal and O2 stopped. No evidence of external hemorrhage like petechiae, purpura , internal hemorrhage like hematuria, nasal, oral, GI bleeding.
Baby’s general condition improved, convulsions controlled, inj eptoin stopped low dose of phenobarbitone 3 mg?kg HS dose continued for 1 month. BP and blood glucose were maintained. Since S bilirubin and hemoglobin within normal limits (10mg/dl) and (15g/dl) respectively, so at this point we were forced to suspect cystic neuroblastoma or duplicating renal collecting system.
USG abdomen repeated once in three day, showed some echo texture in adrenal gland bilaterally.
Urinary VMA sent – normal
CT scan – b/l adrenal hemorrhage
Repeat USG showed left side adrenal hemorrhage which was decreasing in size, right side showed gradual organization.            
Eye test – normal; Hearing test – Normal
ECG- normal ; ECHO – Normal ; Repeat USG brain :Normal

Baby was in NICU for 11 days   
Discharged with syp phenobarbitone for 1month in gradual decrement
Advised parents about the adrenal crisis symptoms
(vomiting, diarrhea, lethargy, apnea, not accepting feeding, irritability, not gaining weight, sudden shock like features)

Followed up after 1 month

vitals and blood glucose – normal
EEG- normal .Blood glucose normal. Clotting factors -normal. Blood pressure-normal Electrolytes normal
No h/o vomiting/diarrhea/shock in the baby
Phenobarbitone stopped gradually.
USG abd:-Left side – normal
Right side – organizing and size is decreasing

At 2 months 

USG abd: - Left side – normal
Right side – size of hemorrhage is decreasing. Mild calcification noted with
Organization
T3, T4, TSH :normal , PT, APTT :normal. Electrolytes normal.

At 3 1/2months

Other vitals and blood glucose – normal
USG abd left side – normal
right side – well organization noted. Mild calcification noted, size 1.5x1.3 cm
PT, APTT: normal. Electrolytes normal.. Neck control started attaining

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Discussion:
Neonatal adrenal haemorrhage is a well-known clinical entity. Birth trauma, prolonged labor, hypoxia, asphyxia, shock, septicemia and hemorrhagic disorders are known to predisposing factors. The right adrenal gland is involved 3–4 times more than the left due to its greater likelihood of compression between the liver and spine. Since the right adrenal vein usually drains directly into the inferior vena cava, compression is likely to induce venous pressure changes. The clinical presentation is variable; infants may be asymptomatic, with the diagnosis made incidentally. Clinically common symptoms seen are poor feeding, vomiting, persistent jaundice, anemia, and abdominal mass though there are cases where it is an incidental finding.

Ultrasonography is the modality of choice for initial and follow-up evaluation of a flank mass in a neonate for the following reasons: (a) the small patient size, (b) the relatively large size of the normal adrenal gland in this age group, and (c) the lack of ionizing radiation.
Ultrasound examination can be repeated frequently without causing any harmful effect to neonates.

In neonates, the normal adrenal glands are clearly visualized at ultrasound and consist of a hypoechoic cortex and a thin, echogenic medulla. Radiological appearance varies depending on the age of hematoma. While acute hematomas appear echogenic on ultrasound, they liquefy with time and assume a cystic appearance. They shrink and decrease in size and may calcify with aging. Generally, it spontaneously undergoes resorption within 4-6 weeks. Thus, ultrasound follow-up is of great importance for ruling out neuroblastoma. Although there is no specific ultrasound sign for adrenal hematoma, demonstrating a decrease in lesion size and observing echo changes that are expected to take place in relation with the age of the lesion may suffice for the diagnosis of adrenal hematoma by ultrasound without any need for further imaging. In general, development of the lesion over time is routine and calcification of the adrenal gland is usually noted approximately 2 weeks after the hemorrhage or even as early as 1 week after hemorrhaging begins. The diagnosis of an adrenal hemorrhage has been made when an echolucent mass was found and then disappeared on follow-up ultrasound studies. Color Doppler sonography may be useful in some cases of neonatal adrenal haemorrhage that are characterized by diminished or absent blood flow in contrast to neuroblastoma. Thus, ultrasound follow-up is of great importance for ruling out neuroblastoma.

Differential diagnoses of cystic lesions near or at the adrenal gland include adrenal hemorrhage, adrenal cyst, adrenal abscess, neuroblastoma, pulmonary sequestration, bronchogenic cyst, enteric cyst, splenic cyst, and cystic lymphangioma. Some cysts arising from the upper pole of the kidney may be similar in the image investigation, including duplication of the renal collecting system, hydronephrosis, multicystic dysplastic kidney, Wilms’ tumor, and cystic nephroma. Since neuroblastoma is a relatively frequent neonatal malignancy, it is certainly important to distinguish between neonatal adrenal haemorrhage and neuroblastoma with colour Doppler sonography being useful in some cases of neonatal adrenal haemorrhage that are characterized by diminished or absent blood flow in contrast to neuroblastoma.

References

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CT ABDOMEN (PLAIN AND CONTRAST) AND ULTRASOUND ABDOMEN IMAGES


DAY OF ADMISSION USG

REVIEW USG AFTER 1 WEEK

USG AFTER 25 DAYS

CT ABD PLAIN

CT ABD CONTRAST

CT ABD CONTRAST

CT ABD CONTRAST

CT ABD CONTRAST


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