Dr.Venkatesh Kumar M, Dr. Vinitha Prasad, Dr. Stefy, Dr.Shela , Dr.C.Jayakumar,
AIMS,Kochi.
Sixteen year old girl ,3rd child of Non consanguinous marriage developmentally normal and immunised upto age presented with non-projectile,non-bilious and non blood stained vomiting of 3 days duration associated with pain in the right lumbar region and high grade intermittent fever of 2 days.
She was initially treated on OP basis with oral antibiotics.
H/O culture positive UTI at 8 years of age.
USG Abdomen done outside showed B/L moderate hydronephrosis,multiple hyperechoeic foci in Left kidney-?calculi and was given Cotrimoxazole prophylaxis for 1 month.
She was then referred to AIMS for further evaluation.
USG KUB done here showed Right PUJ obstructive calculus(1cm),Right prominent pelvis,Urinary bladder showing free floating echoes.
CT Abdomen-B/L kidneys showing cortical irregularity,scarring,striate nephrogram with thin tubular irregular and deformed calyces likely to be secondary to chronic pyelonephritis.
Right PUJ obstruction.
Mild diffuse urinary bladder thickening possibly due to cystitis.
DMSA scan- % DMSA uptake in left kidney: right kidney= 52:48%
Lost followup for next 4 years.
At 13 years,she had recurrent abdominal pain once in a month for which she was managed symptomatically on OP basis.This time DTPA scan done showed decreased renal clearence from right kidney comparing to the left kidney and was on follow up later with Urology.She has not attained menarche till now(Sixteen years of age).
She was pale
SMR showed Tanner stage IV.
Auxology showed normal growth parameters.
Systemic examination was within normal limits.
Differential diagnosis considered were:
1.Chronic pyelonephritis.
2. Urinary tract infection
3. Acute appendicitis
4. Enteric fever
5. Ovarian torsion
Investigations:
CBC: TC-10.43 ku/ml,N-72.9%,L-17.9%,Hb-13 g/dl,Plt-243 ku/ml.
LFT:SGOT-22.8 IU/L,SGPT-45.8 IU/L,ALP-140 IU/L,
Total protein-7.4 gm/dl,S.Albumin-3.8 gm/dl,S.globulin-3.62 gm/dl,
Total bilirubin-0.80 mg/dl, Direct bilirubin -0.37 mg/dl.
RFT: Urea-17.3 mg/dl,Creatinine- 0.68 mg/dl, Na+-136.8 mmol/L,K+- 2.7 mmol/L
CRP: 157.98 mg/L(elevated)
URE showed 4-6 pus cells/hpf, 5-8 RBC’s/hpf, Urine C/S- E.Coli(> 1 lakh colonies)
USG abdomen-B/L kidneys showing raised cortical echogenicity with poorly maintained corticomedullary differentiation.B/L renal calculi present.
She was started on Inj.Ceftriaxone and Potassium correction was given.
Gynecology consultation showed imperforate hymen and advised MRI Abdomen & Pelvis.
MRI Abdomen & Pelvis:•Dysgenetic cervical canal with no definitive cervical canal and multiple septated hemorrhagic cysts at the site of lumen- likely loculated hematometra.
No definite external os.
Absent proximal 2/3rd of vagina.
•Overall appearance of dysgenetic cervix with atretic proximal vagina.
•B/L partial PUJ obstruction ( right > left).
Pediatric surgeon suggested cervical reconstruction surgery.
Enterovaginoplasty was performed.
Intraoperative findings: •Proximal vaginal atresia present.
•Uterine didelphys noted with left hemiuterus appearing rudimentary.
•Right hemiuterus appeared to have atretic cervix.
•On dividing above cervix, lumen could be noted with blood clots inside and both ovaries were present.
•Hemorrhagic cyst in the right ovary was drained.
•Colovaginoplasty done with proximal end anastomosed to the vaginal plate and distal end to uterus(Anti peristaltic).
Impression: Urinary Tract Infection with Mullerian duct anomaly-UTERINE DIDELPHYS.
Discussion:
•Class –I : Mayer-Rokitansky-Kuster-Hauser syndrome(Complete agenesis or hypoplasia)
•Class- II: Unicornuate uterus with or without rudimentary horn.
•Class III: Didelphys uterus.(Complete or partial duplication of cervix,vagina and uterus)
•Class IV: Bicornuate uterus(Uterine septum extending from fundus to cervical os)
•Class V: Septate uterus(Complete or partial midline septum present within a single uterus)
•Class VI: Arcuate uterus(small septate indentation present at the fundus)
•Class VII: DES exposure(T shaped uterus).
Carry home message: It should be kept in mind that regular menstrual history taking in older girls and SMR checking would help us rule out conditions like mullerian duct anomalies