CASE PRESENTATION

Dr.Yazeed OwiwiPediatric Surgery Department

FCPS-II Trainee, PGR-III

CASE HISTORY

20th Dec, 2010

Saira

- 20 months old girl

Hirsutism -

Moon face -

Central obesity-

BMI 21.6 >95th centile -

BMI

HEIGHT 76 CM

WIGHT 12.5 KG

InvestigationBlood CP*

*S/biochemistry BSR monitoring*

*Testosteron level• *Serum cortisol levels

• Dexamethasone suppresion test *

24 hour urinary cortisol*

*Plasma ACTH level *Ultrasound abdomen

*CT Abdomen

Results

Blood cpSerum electrolyte

WNL Urea/Creatinine

BSRLFTs

Testosteron level 11.8 nmol/L ( female: 0.3-3.0 nmol/L )

Serum Cortisol 8:00 am 14 ug/dl 8:00 pm 29 ug/dl

Normal 2.3 -19.9 ug/dl ) )

Dexamethason Suppressive Test -ve

Plasma ACTH 22.20 pg/dl( Normal value 10-100

pg/dl )

24 Hrs urinary Cortisol 27.9 ug/dl( ( Normal value 43-

176 ug/dl

Ultrasound Abdomen

Right supra renal mass with evidance of focal calcification

CT scan Abdomen

Well defined lobulated soft tissue mass lesion -- approximatly cm in right supra renal region.

--Its arrising from right adrenal gland--Multiple calcific foci.

--Suggestive of Neuroblastoma.

Further Investigation

24 hr urinary VMA 4.2 mg/24 hours( Normal value <

13.6)

Surgery were planned..…

Pre-operative manegment

normal * Blood pressure.

* Serum electrolyte, no hypokalemia *BSR, 89 mg/dl normal

*Pt shifted to PICU prior to surgery.

*2 pints of blood crossed matched and prepared.

*Pt NPO at 3 am on day of operation .

Peri-operative manegment

*N/g tube placed .

* 2 wide bore cannula placed .

*Prophylactice antibiotic given .

*Inj. Hydrocotisone 25mg given followed by 25mg/24 hour in infusion form.

*Inj hydralazin on hand .

Open Right Adrenalectomy

Post-operative manegment

--Tissue biopsy sent for histopathology.

--N/G tube removed 1st post-op day.

--Inj. Hydrocortisone given in tapering form.

--Switch to oral coticosteroid 7th post-op day.

--Stitch removed at 10th post op day.

Histopathology

Adrenal Cortical Neoplasm There is marked pleomorphism and

scattered mitosis. However to establish malignant nature of the lesion, the evidence of metastasis is mandatory!

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