CASE STUDY: BLADDER EXSTROPHY - PowerPoint Presentation

1. CASE STUDY: BLADDER EXSTROPHYPresenter: Taphinez Machibya, MDSupervisor: Yustina Tizeba,MD,MMED- Pediatric and Child Health

2. Case PresentationPATIENT’S Identity: Name: PENDO DANIELAge: 12 DAYSSex: FEMALEPlace of Birth: HOMEAddress: KABATINI Department: PEDIATRIC AND CHILD HEALTHPatient’s File no: 00-17-36Date of Admission: 12 FEBRUARY, 2020Time of Admission: 10.30 am, from Home

3. Main ComplainAbnormal swelling and opening in the lower abdomen since birth

4. HISTORY OF PRESENTING ILLNESSThe abnormality in the abdominal wall was seen by the mother after deliveryIt extends from the lower margins of umbilicus towards the genitaliaThe baby was able to suck, breath well, cry normal, and pass stool normally However there was spontaneous leakage of fluid from the swelling that caused wetting on the dressings throughout.No obvious bleeding was seen No history of convulsion, DIB,Loss of consciousness, abnormal bleeding or difficult in breastfeeding.

5. Pre natal HistoryMother is 21 yrs old, this was the 3rd pregnancy (P3L3)Ante - Natal Clinic ; 3 visitations in the last three months of pregnancy (October, November, December), received Folate tabs and SP.No Vaccination givenHx of use of Dawa TatuNo any hx of pregnancy complication (PPROM or PV bleeding)No hx of smoking,maternal Tobacco use,

6. Natal History Normal labour durationDelivered by SVD at homeBorn at term , 9 monthsCried immediately after birthSucked within 1 hr post deliveryNo yellowish or bluish discolorationOn inspection the mother noted abnormal swelling below the umbilicus that is coming from inside the abdomen with watery leakage, pinkish in color with no abnormal smell or bleeding.Body weight currently: 2.8Kgs

7. Post Natal History Cord dropped at 2nd day of lifeNo neonatal yellowish or bluish discoloration Able to breast feed up to dateNormal defecationAbnormal urine passage through the lesionNo any vaccination received

8. FAMILY - SOCIAL HISTORYIndex is the 3rd born in the family to her parents2nd born is male, 2 yrs doing fine 1st born is male , 4yrs, doing fine.The mother is the 1st wife 2nd wife has two children (2yrs F, and 6/12 old , M – both doing fine)No any history of same condition in the family or any other chronic diseases history. Both parents are peasantsHouse is iron roofed with wire meshed windowNo Health insurance for family membersFood availability is 2-3 meals per day, almost the same food of stiff porridge with fish , meat or vegetables and the father is the main provider of the family

9. On Examination Generally: Alert, active, pink skinnedNot pale, no jaundice, no DIB, No LL edemaNormal muscle bulkiness, tone, and power- 5/5 both ULs and LLsNormal upper and lower limbs anatomically Both reflexes normal per age (suckling, gag, moro , symmetrical tonic neck, rooting, grasping)

10. Per ABDOMENNormal contourMoves with respirationUmbilicus normal size and shapeAn opening lesion with protruding mass from inside the abdomen , suprapubic area, measuring of 9x6x4cm3, pinkish , non foul smelling, and watery fluid leakage throughoutNo obvious bleeding seenMargins of the lesion: upper roof at 1cm from umbilicus, extension to the Labia majora bilaterally, and distends to the superior aspect of the clitorisNo other mass or organomegally palpable Perforate anus with normotone sphincter

11. The Abdomen abnormality seen

12. Anterior view of the lesion

13.

14. The whole infant physical appearance

15. Cardiovascular system HR – 142 bpmApex beat at 4th intercostal spaceNo pre-cordial hyperactivityHeart sounds 1 and 2 heard with no murmursNo raised JVPPulse character: strong, regular – regular rhythm

16. Respiratory systemRR- 36 breathes per minuteChest moves symmetricallyNo any chest skin lesion notedTrachea centrally locatedResonant note on percussionNormal bronchial breathe sounds bilaterallyNo anterior lower chest wall indrawing

17. Central Nervous SystemAlert, fully consciousNormal scalp with normal fontanelesOpen eyes spontaneousPositive suckling reflexes Gag reflexes positiveGrasping reflexes normal Occipital – Frontal Circumference 34cm Can feel on touch

18. ENTNormal earsNormal throatNormal noseCan sense some soundsNo cleft lesions per oral or Throat lesions seen

19. Provisional diagnosisBLADDER EXSTROPHYDdx: Cloacal ExstrophyCloacal malformation

20. Management PlanIV ceftriaxone 500mg bid 5/7IV Metronidazole 250mg 8hrly 5/7Wound dressing with sterile gauze and exchange 4hrlyContinue breast feeding and Nursing careAbdominal USS (R/O other urogenital anomalies)ECHOFor Pediatrician reviewUrology / Pediatric surgery review and management (Referral system)

21. Vaccination plan;To be given BCG, OPV0* (*before 14 days) And counselled the parents on other vaccination plan follow up (EPI) completely

22. Abdominal USS ResultsBilateral hydronephrosisOther organs normal

23. ECHO resultsNormal heart shapeNormal cardiac activitiesNormal valves and vesselsConclusion: Normal Echo-Cardiological findings

24. Conclusion Wdx: Bladder Exstrophy with Bilateral HydronephrosisMEDICAL PLAN: Referral to Bugando Medical Centre for Surgical management of the patient.

25. Counselling sessionWellbeing Prognosis of the child post surgical management and psychological assurance (Good if done within 18 months)Counselling to the parents about genetical screening to avoid further recurrence of the same condition and other restrictions like Tobacco use for the future pregnanciesJoining iCHF or NHIF insurance for future medical management and agreed

26. Medical aspect and Short previewBladder exstrophy is a complex, rare disorder that occurs early on while a fetus is developing in the womb. As the bladder is developing, the abdominal wall does not fully form, leaving the pubic bones separated and the bladder exposed to the outside skin surface through an opening in the lower abdominal wall. Because the bladder and urethra are not closed, the bladder cannot store urine. Urine produced by the kidneys drains into this open area.

27. EPIDEMIOLOGYBladder exstrophy occurs in approximately 1 in every 50,000 live births and is slightly more common in males. The disorder may occur in varying degrees and may involve other organs including the bowel, external genitalia and pelvic bones.To USA Prevalence goes up to 3.3/100,000 births.For Classic bladder exstrophy, the Male to female ratio is 2.3:1 and as high as 6:1 in some studies.These conditions seem to be more common in Whites than in other races.

28. Bladder exstrophy diagnosis and evaluationExstrophy of the bladder can usually be diagnosed by fetal ultrasound before an infant is born. Bladder exstrophy is suspected when ultrasound shows that the baby’s bladder is not filling and emptying normally.Fetal imaging experts will look for several other indicators to confirm the diagnosis, including a low umbilical cord with an abdominal bulge below the cord insertion (representing the opened bladder halves, or bladder plate) and unclear male or female genitalia. Bladder exstrophy is not usually associated with other ultrasound findings (But not strictly) or chromosomal or genetic syndromes.However, for gender identification, an amniocentesis may be recommended.If bladder exstrophy is not prenatally diagnosed, the bladder defect is easily visible after birth.

29. Prenatal care and delivery of babies with bladder exstrophyA prenatal diagnosis of bladder exstrophy does not typically change prenatal care, routine delivery planning, timing or mode of delivery. 

30. PARENTAL COUNSELLING AND PSYCHOLOGICAL SUPPORTBladder exstrophy can be an overwhelming diagnosis for parents, but our aim as medical team should aim to support the parent from before birth through delivery and beyond.

31. TREATMENTTreatment for bladder exstrophy includes surgical repair. The goal of treatment is to optimize urinary control, to preserve normal renal function, and to optimize the appearance and function of the external genitalia. If left untreated, normal urine continence does not occur and normal sexual function is compromised.

32. Images showing both a female (left) and male (right) baby with an exposed bladder caused by bladder exstrophy.

33. Take Home messageEarly ANC visitation; as soon as the mother misses her period and discover she is pregnantAtleast once USS scanning in each pregnancyEarly and timely referral system

34. References Pediatric Medical Records Unit, Katavi Regional Referral HospitalTanzania STANDARD TREATMENT GUIDELINES AND ESSENTIAL MEDICINES LIST FOR CHILDREN AND ADOLESCENTS, First Edition October 2017 WHO Pediatric Pocket Book, 2013 (2nd Edition) Mediscape, Exstrophy and Epispadias; Elizabeth B Yerkes, MD (Feb 2,2019)An Illustrated Guide To Pediatric Surgery by Ahmed H. Al-Salem, 2014Atlas of Pediatric Surgical Techniques,Elsever. 1ST Edition,2010 by Dai Chung and Mike Chen

35. Discussion