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2
 Between 4-6 weeks, the cloaca becomes the
common depository for the developing urinary,
genital and rectal systems.
 Than cloaca divided into an anterior urogenital
sinus and a posterior intestinal canal by the
urorectal septum.
 Two lateral folds of cloacal tissue join the
urorectal septum.
3
 Anorectal atresia occurs in one of every 4000 to
5000 newborns.
 Slightly more common in males.
 The most frequent defect-
◦ Male- anorectal atresia with
rectourethral fistula.
◦ Female- anorectal atresia with
rectovestibular fistula.
4
 MALES -
1. Rectobladder neck fistula
2. Rectourethral fistula
 Prostatic
 Bulbar
3. Imperforate anus with no fistula
4. Perineal fistula
5. Rectal Atresia
5
 FEMALES -
1. Rectovestibular fistula (most common)
2. Perineal fistula (2nd most common)
3. Cloaca (3rd most common)
 with a common channel longer than 3 cm
 with a common channel shorter than 3 cm
4. Imperforate anus with no fistula
5. Complex malformations
6
8
 Only true supralevator malformation
 Incidence- 10% of total male ARM.
 Rectum opens into the bladder neck
 Levator muscles, striated muscle complex, and
the external sphincter are poorly developed,
sacrum is often deformed and short, perineum is
often flat
 Poor prognosis
9
10
 Most frequent defect.
 Fistula may be located at the lower (bulbar) or the
higher (prostatic) part of the urethra.
 Immediately above the fistula, the rectum and
urethra share a common wall.
 Meconium through the urethra-unequivocal sign of
a rectourinary fistula.
11
 RECTOURETHRAL BULBAR FISTULA-
◦ Usually associated with good-quality muscles, a well-
developed sacrum, a prominent midline groove, and a
prominent anal dimple.
 RECTOURETHRAL PROSTATIC FISTULA-
◦ more frequently
◦ associated with poor-quality muscles, an abnormally
developed sacrum, a flat perineum, a poor midline
groove, and a barely visible anal dimple.
12
13
 Incidence - 5%
 Most patients have – well developed sacrum and
good muscles
 Good prognosis
 The rectum usually terminates approximately 2 cm
from the perineal skin.
 Rectum and urethra separated only by a thin,
common wall.
14
 50% pt also have Down syndrome.
 More than 90% of patients with Down syndrome
and imperforate anus have this defect.
 Down syndrome does not interfere with good
prognosis for bowel control.
15
 Extremely unusual defect in male patients (~1%).
 The lumen of the rectum is totally (atresia) or
partially (stenosis) interrupted.
 The upper pouch - dilated rectum
 The lower portion - small (1-2 cm) anal canal in
the normal location
 These two structures may be separated by a thin
membrane or by dense fibrous tissue.
 Excellent functional prognosis.
 Must be screened for a presacral mass.
16
 It is defined as a defect in which the rectum,
vagina, and urinary tract meet and fuse, creating a
single common channel.
 This defect should be suspected in a female born
with imperforate anus and small-looking genitalia
 On inspection- single perineal orifice.
 Length of the common channel varies from 1 to 7
cm.
18
 Frequently, the vagina is abnormally distended
and full of secretions (hydrocolpos)
 It compresses the trigone and frequently
associated with megaureters
 Hydrocolpos may become infected (pyocolpos)
and may lead to perforation and peritonitis
 Also associate with different degrees of vaginal
and uterine septation or duplication
 Rarely, patients may have cervical atresia
19
20
 Usually associated with complex defects
 Mobilization of the vagina is difficult.
 Some form of vaginal replacement is often needed
21
22
 This defect can be repaired with a posterior
sagittal operation without opening the abdomen.
 Usually associated with a well-developed sacrum,
a normal-appearing perineum, and adequate
muscles and nerves therefore, a good functional
prognosis.
23
24
 It is the most common defect in girls
 Excellent functional prognosis.
 On inspection - normal urethral meatus and a
normal vagina, with a third hole in the vestibule,
 About 5% of these patients will have two
hemivaginas with a vaginal septum.
25
26
 It is equivalent to the perineal fistula described in
the male patient.
 The rectum is well positioned within the sphincter
mechanism, except for its lower portion, which is
anteriorly located.
 The rectum and vagina are well separated
27
28
 Same therapeutic and prognostic implications as
described for male patients.
29
 Unusual and bizarre anatomic arrangements can
be seen.
 No general guidelines can be drawn for the
management of these patients.
 Each case must be individualized.
30
 The term vaginal fistula is frequently erroneously
used in patients who actually have a vestibular
fistula or a cloaca.
 A true vaginal fistula occurs in less than 1% of all
cases.
 It is not considered part of the proposed
classification.
31
 1. Urologic defects (approx 50%)
 2. Spinal and sacral defects (approx 30%)
 3. Tethered cord and other cord abnormalities
(approx25%)
 4. Cardiovascular malformations (approx 30%)
but only 10% have important hemodynamic
repercussions, requiring treatment
 5. Esophageal atresia (approx 5–10%)
32
 GOOD PROGNOSTIC SIGN-
◦ Well-developed sacrum
◦ Good-quality muscles
◦ Prominent midline groove
◦ Prominent anal dimple
 BAD PROGNOSTIC SIGN-
◦ Abnormally developed sacrum
◦ Poor-quality muscles
◦ Flat perineum
◦ Poor midline groove
◦ Barely visible anal dimple
33
34
 Clinical examination
 Investigation
 Treatment
 MALE :
• Meconuria
• Midline raphae
• Size and position of anal dimple
• Gluteal folds
• Good looking perineum
• Imperforate anus with no fistula
• Flat bottom, bifid scrotum
 FEMALE :
• Number of openings
• One/two/three/four
 Does the baby has a serious associated
defect that endangers life.?
 Does the baby need a colostomy or we
perform a primary repair of
malformation?
 During the first 24 hours :
the neonate should
• Take nothing orally
• Receive intravenous fluids
• And antibiotics
• Look for other associated defects
• Urological defects(50%)
• Spinal & sacral defects(30%)
• Tethered cord & other cord abnormalities(25%)
• Cardiovascular malformations(30%, but 10%
have serious hemodynamic effects)
• Esophageal atresia(10%)
• VACTERL anomalies
• Examination of spine
• Passage of nasogastric tube
• Central cyanosis
• Limb anomaly
• Examination of genitalia
• USG abdomen to R/O Hydronephrosis &
Megaureter
• USG pelvis in a female to R/O Hydrocolpos
• USG spine for tethered cord
• Xray spine & sacrum for anamolies
• Clinical evaluation for CVS defects( along with
Echocardiograghy) & Esophageal atresia
 After 24 hrs :
• Re evaluate
• Look for passage of meconium, if not we go for
investigation
 Babygram
 Invertogram(1930)
 Crosstable prone lateral radiography(1983)
 USG abdomen+pelvis(to rule out
genitourinary anomalies)
 AT BIRTH : Gas in stomach
 6-8 HRS : Gas reaches ileocaecal junction
 12-18 HRS: Gas reaches rectum
 ANOPLASTY :
• Cut back
 COLOSTOMY
 HIGH PRESSURE DISTAL COLOSTOGRAM AT 2-3
MONTHS OF AGE
 PSARP DONE AT 6MONTHS TO 1 YEAR OF AGE
 DILATION BY HEGAR DILATOR OF DIFFERENT SIZES
 DEFENATIVE SURGERY
 COLOSTOMY :
• High Sigmoid loop
• Divided colostomy
Surgical options :
• PSARP
• ASARP
• APPSARP
• APPT
• LAARP
Complications of posterior sagital anorectoplasty
Wound infection Dehiscence Retraction
Anal strictures
Constipation
 BOWEL MANAGEMENT :
 For constipation
 Fecal Incontinence
 95% successfull in keeping patients clean.
Other surgeries :
• ASARP
• APPSARP/APPT
• LAARP
Laparoscopically assisted anorectal
pull through (LAARP) for high ARM
• Advantages:
• LAARP allows the surgeon to treat a high
lesion like a low lesion.
• No need to divide the muscle complex from
below.
• Immediately after the procedure strong and
symmetric contraction of the sphincter around
the neoanus can be seen.
• It also avoids complication and multiple
procedures associated with colostomy.
• More rapid return of bowel function
• Improved cosmetic appearance
• Shorter postoperative recovery
• Decreased postoperative complications
REFERNCES :
• Paediatric surgery-Diagnosis & management by
P.Puri & M Hallworth
• Ashcraft’s paediatric surgery 5th ed.
• Holshneider anorectal malformations in children
THANK YOU

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Ano-rectal Malformations copy.pptx

  • 1.
  • 2. 2
  • 3.  Between 4-6 weeks, the cloaca becomes the common depository for the developing urinary, genital and rectal systems.  Than cloaca divided into an anterior urogenital sinus and a posterior intestinal canal by the urorectal septum.  Two lateral folds of cloacal tissue join the urorectal septum. 3
  • 4.  Anorectal atresia occurs in one of every 4000 to 5000 newborns.  Slightly more common in males.  The most frequent defect- ◦ Male- anorectal atresia with rectourethral fistula. ◦ Female- anorectal atresia with rectovestibular fistula. 4
  • 5.  MALES - 1. Rectobladder neck fistula 2. Rectourethral fistula  Prostatic  Bulbar 3. Imperforate anus with no fistula 4. Perineal fistula 5. Rectal Atresia 5
  • 6.  FEMALES - 1. Rectovestibular fistula (most common) 2. Perineal fistula (2nd most common) 3. Cloaca (3rd most common)  with a common channel longer than 3 cm  with a common channel shorter than 3 cm 4. Imperforate anus with no fistula 5. Complex malformations 6
  • 7.
  • 8. 8
  • 9.  Only true supralevator malformation  Incidence- 10% of total male ARM.  Rectum opens into the bladder neck  Levator muscles, striated muscle complex, and the external sphincter are poorly developed, sacrum is often deformed and short, perineum is often flat  Poor prognosis 9
  • 10. 10
  • 11.  Most frequent defect.  Fistula may be located at the lower (bulbar) or the higher (prostatic) part of the urethra.  Immediately above the fistula, the rectum and urethra share a common wall.  Meconium through the urethra-unequivocal sign of a rectourinary fistula. 11
  • 12.  RECTOURETHRAL BULBAR FISTULA- ◦ Usually associated with good-quality muscles, a well- developed sacrum, a prominent midline groove, and a prominent anal dimple.  RECTOURETHRAL PROSTATIC FISTULA- ◦ more frequently ◦ associated with poor-quality muscles, an abnormally developed sacrum, a flat perineum, a poor midline groove, and a barely visible anal dimple. 12
  • 13. 13
  • 14.  Incidence - 5%  Most patients have – well developed sacrum and good muscles  Good prognosis  The rectum usually terminates approximately 2 cm from the perineal skin.  Rectum and urethra separated only by a thin, common wall. 14
  • 15.  50% pt also have Down syndrome.  More than 90% of patients with Down syndrome and imperforate anus have this defect.  Down syndrome does not interfere with good prognosis for bowel control. 15
  • 16.  Extremely unusual defect in male patients (~1%).  The lumen of the rectum is totally (atresia) or partially (stenosis) interrupted.  The upper pouch - dilated rectum  The lower portion - small (1-2 cm) anal canal in the normal location  These two structures may be separated by a thin membrane or by dense fibrous tissue.  Excellent functional prognosis.  Must be screened for a presacral mass. 16
  • 17.
  • 18.  It is defined as a defect in which the rectum, vagina, and urinary tract meet and fuse, creating a single common channel.  This defect should be suspected in a female born with imperforate anus and small-looking genitalia  On inspection- single perineal orifice.  Length of the common channel varies from 1 to 7 cm. 18
  • 19.  Frequently, the vagina is abnormally distended and full of secretions (hydrocolpos)  It compresses the trigone and frequently associated with megaureters  Hydrocolpos may become infected (pyocolpos) and may lead to perforation and peritonitis  Also associate with different degrees of vaginal and uterine septation or duplication  Rarely, patients may have cervical atresia 19
  • 20. 20
  • 21.  Usually associated with complex defects  Mobilization of the vagina is difficult.  Some form of vaginal replacement is often needed 21
  • 22. 22
  • 23.  This defect can be repaired with a posterior sagittal operation without opening the abdomen.  Usually associated with a well-developed sacrum, a normal-appearing perineum, and adequate muscles and nerves therefore, a good functional prognosis. 23
  • 24. 24
  • 25.  It is the most common defect in girls  Excellent functional prognosis.  On inspection - normal urethral meatus and a normal vagina, with a third hole in the vestibule,  About 5% of these patients will have two hemivaginas with a vaginal septum. 25
  • 26. 26
  • 27.  It is equivalent to the perineal fistula described in the male patient.  The rectum is well positioned within the sphincter mechanism, except for its lower portion, which is anteriorly located.  The rectum and vagina are well separated 27
  • 28. 28
  • 29.  Same therapeutic and prognostic implications as described for male patients. 29
  • 30.  Unusual and bizarre anatomic arrangements can be seen.  No general guidelines can be drawn for the management of these patients.  Each case must be individualized. 30
  • 31.  The term vaginal fistula is frequently erroneously used in patients who actually have a vestibular fistula or a cloaca.  A true vaginal fistula occurs in less than 1% of all cases.  It is not considered part of the proposed classification. 31
  • 32.  1. Urologic defects (approx 50%)  2. Spinal and sacral defects (approx 30%)  3. Tethered cord and other cord abnormalities (approx25%)  4. Cardiovascular malformations (approx 30%) but only 10% have important hemodynamic repercussions, requiring treatment  5. Esophageal atresia (approx 5–10%) 32
  • 33.  GOOD PROGNOSTIC SIGN- ◦ Well-developed sacrum ◦ Good-quality muscles ◦ Prominent midline groove ◦ Prominent anal dimple  BAD PROGNOSTIC SIGN- ◦ Abnormally developed sacrum ◦ Poor-quality muscles ◦ Flat perineum ◦ Poor midline groove ◦ Barely visible anal dimple 33
  • 34. 34
  • 35.  Clinical examination  Investigation  Treatment
  • 36.  MALE : • Meconuria • Midline raphae • Size and position of anal dimple • Gluteal folds • Good looking perineum • Imperforate anus with no fistula • Flat bottom, bifid scrotum  FEMALE : • Number of openings • One/two/three/four
  • 37.
  • 38.  Does the baby has a serious associated defect that endangers life.?  Does the baby need a colostomy or we perform a primary repair of malformation?
  • 39.  During the first 24 hours : the neonate should • Take nothing orally • Receive intravenous fluids • And antibiotics • Look for other associated defects
  • 40. • Urological defects(50%) • Spinal & sacral defects(30%) • Tethered cord & other cord abnormalities(25%) • Cardiovascular malformations(30%, but 10% have serious hemodynamic effects) • Esophageal atresia(10%) • VACTERL anomalies
  • 41. • Examination of spine • Passage of nasogastric tube • Central cyanosis • Limb anomaly • Examination of genitalia
  • 42. • USG abdomen to R/O Hydronephrosis & Megaureter • USG pelvis in a female to R/O Hydrocolpos • USG spine for tethered cord • Xray spine & sacrum for anamolies • Clinical evaluation for CVS defects( along with Echocardiograghy) & Esophageal atresia
  • 43.  After 24 hrs : • Re evaluate • Look for passage of meconium, if not we go for investigation
  • 44.  Babygram  Invertogram(1930)  Crosstable prone lateral radiography(1983)  USG abdomen+pelvis(to rule out genitourinary anomalies)
  • 45.
  • 46.  AT BIRTH : Gas in stomach  6-8 HRS : Gas reaches ileocaecal junction  12-18 HRS: Gas reaches rectum
  • 47.
  • 48.
  • 49.
  • 50.
  • 52.  COLOSTOMY  HIGH PRESSURE DISTAL COLOSTOGRAM AT 2-3 MONTHS OF AGE  PSARP DONE AT 6MONTHS TO 1 YEAR OF AGE  DILATION BY HEGAR DILATOR OF DIFFERENT SIZES  DEFENATIVE SURGERY
  • 53.  COLOSTOMY : • High Sigmoid loop • Divided colostomy
  • 54.
  • 55.
  • 56. Surgical options : • PSARP • ASARP • APPSARP • APPT • LAARP
  • 57.
  • 58.
  • 59.
  • 60.
  • 61.
  • 62.
  • 63.
  • 64. Complications of posterior sagital anorectoplasty Wound infection Dehiscence Retraction Anal strictures Constipation
  • 65.
  • 66.
  • 67.
  • 68.  BOWEL MANAGEMENT :  For constipation  Fecal Incontinence  95% successfull in keeping patients clean.
  • 69.
  • 70. Other surgeries : • ASARP • APPSARP/APPT • LAARP
  • 71. Laparoscopically assisted anorectal pull through (LAARP) for high ARM • Advantages: • LAARP allows the surgeon to treat a high lesion like a low lesion. • No need to divide the muscle complex from below. • Immediately after the procedure strong and symmetric contraction of the sphincter around the neoanus can be seen.
  • 72. • It also avoids complication and multiple procedures associated with colostomy. • More rapid return of bowel function • Improved cosmetic appearance • Shorter postoperative recovery • Decreased postoperative complications
  • 73. REFERNCES : • Paediatric surgery-Diagnosis & management by P.Puri & M Hallworth • Ashcraft’s paediatric surgery 5th ed. • Holshneider anorectal malformations in children THANK YOU

Editor's Notes

  1. Vacterl anomoly can be associated with klippel feil and golden har syndrome. Among vacterl anomoly vertebral defetcs are 80% wich constitues hypoplasa, hemi vertebrae,scoliosis,etc.. Anal atresia and imperforate anus, Cardiac defects 75% wich has VSD as MC, ASD, TOF…, TEF, Esophageal atresia, Renal agenesis, Radial anomoly
  2. Hydrocolpos means distension of vagina due to fluid accumulation proximally. Obstruction might be bcos of imperforate hymen, transverse vaginal septum. Tetherd cord means pulling of cord making it taught due to attachment to the canal. They are called tethered cord syndrome(TCS) or occult spinal dysraphism sequence which consists on multiple neurological defects that relate to malformation of spinal cord. Like tight filum terminale, dermoid cyst, cystocele, diastematomeylia(split cord malformation), lipomeningomyelocele.
  3. Invertogram is obselete nowadays bcos of many disadvanatges like faulty techique, aspiration by baby in inverted position. Rectal gas to skin length less than 1cm ,more than 1 cms are all obselete. Imp feature to remember is both the GT shud superimpose
  4. Level of PC line denotes the bladder neck, verumontanum, pelvic reflection from rectum to prostate, external OS of cervix. I line corresponds to upper surface of bulb of urtehra in amle and upper limit of perineal body n the level of triangular ligament in female. Errors due to insufficient time, meconium plug, gas escaping thru fistula, inapprop placement of anal marker.
  5. After 4 weeks we go for cologram. #8 foley catheter is introduced in the mucous fistula 2-3cms n balloon infalted with 1-2ml water. Then we inject around 100ml of water sol contrast. Initially the contrast stops at PC line creating a line. This represents the muscle tone of levator ani. Not to confuse with high arm. Now inject with more pressure to overcome the tone. Not possible in RVF, bcos it immediately leaks and we cannot create pressure. In high fistula we have to go by APPSARP/APPT.
  6. For the imaging study, your child will not be sedated and the procedure will last approximately 15 minutes. The team will inject a special dye into the distal stoma, and use other tools to help push the dye into the final segment of the colon and rectum. This procedure gives the surgeon a very clear image of the defect and surrounding anatomy. This procedure is done in an outpatient setting. The surgical team will be able to proceed with your child’s final repair surgery after the distal colostogram.