Anorectal malformations

1. Dr. Anvesh amiti

2.  Baby details: b/o Uma maheswari
 TERM
 DOB-15/9/18
 TOB-7.58AM
 APGAR-8/10, 9/10
 B.wt-2.83 kg
 HC- 34cm
 Length-47cm

3.  Mother details:
 G2P2L2A4
 Spontaneous conception.
 Total 4 spontaneous abortions were done, years 2010,2011,2015,2016
required D&C
 Non consaguineous marriage
 Married life 8 years
 K/c/o Bronchial asthma on MDI therapy
 1st baby was born in 2011 at Aditya hospital, surgery done for
intussusception was done at 5 months of age and no other health
issues

4. Antenatal scans
 1 scan done on 8 weeks 3 days scan was normal study
 2nd scan done On 21 weeks no anamolies were detected

5.  Baby was born through elective LSCS (ind: prev LSCS) at Aditya
hospital, chennai
 Baby cried at birth
 Noticed no patent anal opening and single umbellical artery hence
reffered to SRMC for further management
 Baby was received and kept on nil per oral started IV fluids

7.  Pead surgery opinion was taken and planned for colostomy
 Usg (kub) done s/o normal study
 2D echo done s/o 4 mm ASD with L to R shunt
 1 PRBC transfusion given
 Ortho opinion was taken for skeletal anamoly , right side L5 hemi
vertebrae
 Hence baby kept as VACTERL association

8.  X ray for done skeletal abnormality

9.  Colostomy was done under general anesthesia on 17/9/2018
 Septic work up done and started Piptaz and Amikacin
 On 2nd POD started feeds and gradually increased
 Culture s/o no growth hence antibiotics were stopped

10. DISCUSSION

11. Introduction and epidemiology
 Anorectal malformations comprise a wide spectrum of diseases
which involve the distal anus and rectum as well as the urinary and
genital tracts
 Defects range from the very minor to those complex, are often
associated with other anomalies
 Anorectal malformations occur in approximately 1 newborn per
5000 live births

12. Embryology
 Parts of the cavity of the yolk sac are enclosed withinembryo to
form the primitive gut.
 Primitive gut is in free communication with the rest of theyolk sac
 Part of the gut cranial to this communication is the foregut,caudal
is hindgut and in-between is the midgut.

13.  The part of the hind gut caudal to attachment of
allontoicdiverticulum is called cloaca
 The cloaca soon divides into the broad ventral part and narrow
dorsal part separated by urorectal septum
 The ventral part is called the primitive urogenital sinus and dorsal
part is called primitive rectum
 Uro rectal septum grows toward the cloacal membrane and
eventually fuses with it
 The cloacal membrane is divided into ventral urogenital
membrane and dorsal anal membrane

18. Rectal atresia
 . Rectal atresia is a rare type ofanorectal malformation
(ARM)constituting 1-2% of the ARM.Male:

19. Rectal duplication
 Rectal duplications form only 5% of alimentary tract duplications
 The majority of rectal duplications are located in theretrorectal
space as a cystic mass

20. Rectal ectasia
 Rectal ectasia is defined as a state of massive dilatation of the rectum and
sigmoid colon.
 lt may be primary (presenting at the time of birth) or secondary
(developing later as a result of distal obstruction or inadequate
evacuation).
 Balloon-like rectum, colonic inertia, megarectum,terminal fecal reservoir
syndrome, pseudo-Hirschsprung's disease and rectal inertia

21. Persistant cloaca
 Persistent cloaca represents the most complexdeformity in female
anorectal, vaginal, andurogenital malformations.
 It is defined as a defect in which the rectum, oneor two vaginas
and the urinary tract converge into one common channel.
 It is very rare and occurs in 1250,000 newborns.

22. Associated malformations
CVS anomalies(30-35%): TOF, VSD, TGA, HLHS (ref :jiaps 2015 jan-
mar 20(1); 10-15)
GI malformations(5-10%): tracheoesophageal fistula, duodenal
atresia, annular pancreas, malrotation, Hirschprung disease
Vertebral malformations(4-9%): lumbosacral anomalies, spinal
dysraphism, tethered spinal cord, Currarino triad
Urogenital tract anomalies(40-50%): Renal agensis, VUR, Renal
dysplasia, cryptorchidism, bicornate uterus, uterus didelphys,
vaginal septum etc

23. Investigations

24. Invertogram

25. Cross table view

26. Common mistakes in interpRetation X Ray
1. Insufficient time for gas to reach the terminal bowel
2. Active contraction of the levator a ani/sphinctermuscle complex
can push the gas shadow higher.
3. Distortion by x-ray magnification resulting in the appearance of a
longer gap between the gas within terminal colon and skin.
4. Meconium plug in the terminal gut may producerroneously high
shadow if the gas does notdisplace the meconium.
5. Gas in vagina may mistaken for gas in bowel

27. Pressure augmented colostogram
 The investigation involves the injection of water-soluble contrast medium
into the distal limb ofthe colostomy using a balloon catheter.
 The balloon is inflated and traction applied tothe catheter to occlude the
stoma.
 Water-soluble contrast medium is then injectedunder constant gentle
pressure until a fistula isfilled, and visualized with the aid of fluoroscopy.
 The presence of a fistula is initially indicated by theappearance of a nipple
of contrast arising from theterminal colon.

29. Ultrasonography
 Intial reports involved scanning from the anal dimple and measuring the
distance between the terminal colon and the skin at the anal dimple site
DISADVANTAGES
1. Application of too much pressure at the anal dimple distorting the
distance to the terminal colon
2. The colon being difficult to visualize if decompressed or if a large fistula
present

30. Ct scanning
 CT provides increased detail, particularly of the osseous structures, as well
as being able to demonstrate the pelvic floor muscles
 There is difficulty in distinguishing meconium from the rectal wall and
adjacent muscles due to limitations in contrast

31. MRI
 Improved contrast resolution allows easydifferentiation of
meconium from the rectal wall andlevator musculature.
 In the neonatal period, meconium is easilyvisualized on MRI as
hyper intense material onT1-weighted imaging, due to its lipid
content.
 MRI can accurately detect the level of the bowel, and the state of
the pelvicfloor musculature.

32. Managment

33.  IVF & evaluate associated defects
1. NG tube to exclude EA
2. ECHO for cardiac evaluation
3. USG abdomen to evaluate renal defects
4. Spinal defects evaluation with Xray
 If meconium seen on the perineum , perineal fistula exists & if in
urine rectourinary fistula exists
 If air in the rectum locatd below the coccyx, baby in good
condition and no significant associated defects can perform
PSARP without colostomy

34.  Colostomy allows for a future high pressure distal colostogram
 PSARP at 2 to 3 months later will provide the neonate gaining weight
appropriately
 Advantages of definitive repair early at 2-3 months of age:
1. Less time with abdominal stoma
2. Less size discrepancy between proximal and distal bowel
3. Easier anal dilatation
4. Acquired local sensation

37. Post operative anal dilatations
 Started after 2 wks post operative with that fits gently into the anus
 Then twice daily with increase size weekly until the rectum reaches the
desired size
 Once desired size reached the colostomy can be closed
 After that the parents follow a tapering dilatation schedule that is once a
day for 1 month, twice a week for 1 month, once a week for month and
once a month for 3 months

38. Post operative complications
1. Wound infection
2. Anal strictures
3. Rectal prolapse
4. Constipation
5. Fecal incontinence
6. Neurogenic bladder

39. THANK YOU