1. Pediatric Case Presentation
Reporter: Intern 張智閑
Supervisor: CR賴馥蘋
Date: 2016/5/20

2. 2016/5/13

3. Chief Complaint
Small for gestational age and imperforate anus was found after birth (2016/5/12)

4. Present Illness MOTHER 32 y/o female Amniocentesis: normal
Level II sonar: normal, no oligohydroamnio or
polyhydroamnios
No gestational diabetes mellitus (GDM), pregnancy-induced hypertension (PIH) or preeclampsia
TORCH survey for IUGR

5. Past History Birth History: Apgar score: 8→9 Feeding: NPO
G1P1A0
Cesarean section due to IUGR w/ fetal distress
Gestational age: 38+2 weeks
Newborn: delay of initial crying (-), premature rupture of membrane (-)
Apgar score: 8→9
Feeding: NPO
Vaccination: (-)
Growth and Development:
Body weight: 1765gm (<3th%), body length: 43.9 cm (<3th%),
head circumstance: 29.5 cm (<3th%)
Ponderal index=2.09(<10th%)

6. Family History

7. Physical Examination Vital signs: T/P/R: 35.3/152/36, BP: 86/51 mmHg
Consciousness: clear,
Appearance: fair looking
Head: Conjunctiva: not anemic, Sclera: not icteric
Throat: not injected
Neck: supple, no lymphadenopathy
Chest: symmetric expansion, subcostal retraction (-)
Breathing sounds: bilateral clear, no crackles
Heart sound: no murmur
Abdomen: soft, not distended, bowel sounds: normoactive
Liver/Spleen: impalpable / impalpable
Extremities: freely movable, pitting edema (-)
Skin: turgor fine, no rash, no meconium stain
Anus: imperforate, sphincter reflex (-), no visible fistula

8. Laboratory Data

9. Impression Imperforate anus Survey for associated anomaly
→ NPO with nasogastric tube decompression
Consult Pediatric surgeon

10. VACTERL defect Vertebral defect Anal defect Cardiac defects
Tracheal Esophageal fistula defect
Renal defect
Limb defect
OEIS 1 in 40W

11. X ray
VACTERL可以看出VTEL

12. Cardiac echo
PDA and inter-atrial shunt
1)Situs solitus, levocardia
2)No chamber dilation
3)A patent ductus arteriosus with size: 0.3cm; L to R shunt, with PG: 12mmHg
4)Good LV systolic function with LVEF: 65.9%
5)An inter-atrial left to right shunt, size: 0.365cm
6)Left arch, no COA
DVD N530
Min-Ling Hsieh / Min-Ling Hsieh
A patent ductus arteriosus with size: 0.272cm; L to R shunt, with PG: 16mmHg

13. TE Fistula

14. Renal Echo

15. Renal Echo

16. Spinal echo

17. X ray
VACTERL可以看出VTEL

18. 2016/5/13 Operation Operative diagnosis :
1. Anorectal malformation, high type
2. Megacolon(?)
Operative procedure :
Double barrel colostomy　
area of vestige of external sphincter
fistula 2mm in diameter 2.8cm from the vestige

19. Post-OP D3

20. Final Diagnosis
Anorectal malformation
vestibular fistula type

21. Imperforate Anus

22. Overview Incidence (1) 1/4,000 live births (2) Male > female
Embryology
(1) Urorectal septum anomaly
(2) Cloaca → urogenital sinus + rectum
Nelson Textbook of Pediatrics.19 th edition, 2011: Chapter 336 Surgical Conditions of the Anus and Rectum

23. Type Low type: fistula to perineum High type: fistula to urinary tract
Normal sacrum, good muscles
Good prognosis
High type: fistula to urinary tract
Undeveloped sacrum and muscles
Poor innervation
Poor prognosis
Nelson Textbook of Pediatrics.19 th edition, 2011: Chapter 336 Surgical Conditions of the Anus and Rectum

24. Nelson Textbook of Pediatrics
Nelson Textbook of Pediatrics.19 th edition, 2011: Chapter 336 Surgical Conditions of the Anus and Rectum

25. Type Perineal fistula Vestibular fistula Cloaca
The most benign of ARM
<10% associated with urologic defect
Gynecologic anomalies are rare
Vestibular fistula
Cloaca
Pediatric Gastrointestinal and Liver Disease, 5st edition

26. Approach to the patient
Careful inspection of the perineum to check fistula
Cross-table lateral veiw X-ray
Nasogastric tube to identify esophageal atresia
Urinalysis: provide enough data in 80-90% of male patients to determine the need for a colostomy
Meconium in the urine: do colostomy
Radiography not reliable during first 16~24hr -> bowel not distended
Nelson Textbook of Pediatrics.19 th edition, 2011: Chapter 336 Surgical Conditions of the Anus and Rectum

27. minimal posterior sagittal anal rectal plasty
改cross-table view 1 27

28. Outcome Fecal & urine incontinence and constipation
Nelson Textbook of Pediatrics.19 th edition, 2011: Chapter 336 Surgical Conditions of the Anus and Rectum

29. Operation Low High Minimal Posterior Sagittal Anorectoplasty (MPSARP)
1-2 days after birth
High
Colostomy: 1-2 day after birth
Pull-through (Peña PSARP): after 4-8 weeks
Cloaca: after 4-6 months
Take-down: 1-3 months after pull-through
The three main goals of treatment are
to allow passage of stool (ie, relieve obstruction), to place the rectal pouch on the perineum in good position, and to close the fistula.
Low defects:
Minimal PSARP with anal dilation
(daily for 3-5 months to prevent stricture formation and to allow for growth)
place the fistula within the limits of the ext. sphincter.
High defects:
Colostomy decompresses the bowel and provides protection during the healing of the subsequent total repair.
(先以下結腸造口術解除大腸阻塞的現象)
If the patient grows well and has no other associated defects (GI or cardiac) that require treatment → readmitted at 4-8 weeks
Very young infant → need more experience
Performing the definitive repair at 1 m/o has important advantages for the patient.
The time of cloaca repair: 3 m/o 1 29 29 29

30. Take home messages Evaluation: physical examination & urine analysis
Low type v.s. High type: perineal fistula
VACTERL: (Vertebral, imperforate Anus, Cardiac defect, TracheoEsophageal fistula, Renal agenesis, radial Limb dysplasia)
Survey
Operation
Outcome: Fecal & urine incontinence and constipation

31. Thanks for your attention!