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Anorectal malformation.pptx

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Anorectal malformation

Anorectal malformation.pptx

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Anorectal Malformations-
At birth assessment, evaluation &
management protocols
Dr. Arjun A. Pawar
MBBS, MS,
M. Ch. Pediatric Surgery,
DNB Pediatric Surgery,
FMAS,
FIAGES.
Consultant Pediatric and Neonatal Surgeon,
Pediatric Laparoscopic Surgeon
Pediatric Urologist
Divine Pediatric Surgery Centre.
Garkheda, Chhatrapati Sambhajinagar.
Overview
• Definition
• Embryology
• ANC diagnosis
• Clinical presentation of ARM- Male/Female
• Associated Anomalies/ Sundromes
• Evaluation at Birth
• Management Algorithms- Male /Female
• Classification
• Cloaca
• Pouch Colon
Anorectal malformations
• Complex group of congenital anomalies
resulting from abnormal development of the
hindgut, allantois and mullerian duct.
– Isolated ARM -40%
– Syndromic ARM -60%
• Incidence1:3000 live births
• Sex ratio: almost equal,
Embryology
Abnormal Hindgut Development:
Malfunction of the primitive streak
and tail bud in the dorsal part of the
embryo leads to ARM
The earliest morphological defect
– deficiency in the dorsal
component of the cloacal
membrane and the adjacent dorsal
cloaca.
Antenatal detection- USG/MRI
Most important signs :
o Absent target sign,
o Anomalous distal bowel wall and rectal fluid
o Presence of a dilated distal bowel/rectum
o Intraluminal meconium calcification or
o Entero lithiasis
Prenatal diagnosis of ARM remains
challenging in female fetuses with a low-
type imperforate anus
Antenatal USG of ARM

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Anorectal malformation.pptx

  • 1. Anorectal Malformations- At birth assessment, evaluation & management protocols Dr. Arjun A. Pawar MBBS, MS, M. Ch. Pediatric Surgery, DNB Pediatric Surgery, FMAS, FIAGES. Consultant Pediatric and Neonatal Surgeon, Pediatric Laparoscopic Surgeon Pediatric Urologist Divine Pediatric Surgery Centre. Garkheda, Chhatrapati Sambhajinagar.
  • 2. Overview • Definition • Embryology • ANC diagnosis • Clinical presentation of ARM- Male/Female • Associated Anomalies/ Sundromes • Evaluation at Birth • Management Algorithms- Male /Female • Classification • Cloaca • Pouch Colon
  • 3. Anorectal malformations • Complex group of congenital anomalies resulting from abnormal development of the hindgut, allantois and mullerian duct. – Isolated ARM -40% – Syndromic ARM -60% • Incidence1:3000 live births • Sex ratio: almost equal,
  • 4. Embryology Abnormal Hindgut Development: Malfunction of the primitive streak and tail bud in the dorsal part of the embryo leads to ARM The earliest morphological defect – deficiency in the dorsal component of the cloacal membrane and the adjacent dorsal cloaca.
  • 5. Antenatal detection- USG/MRI Most important signs : o Absent target sign, o Anomalous distal bowel wall and rectal fluid o Presence of a dilated distal bowel/rectum o Intraluminal meconium calcification or o Entero lithiasis Prenatal diagnosis of ARM remains challenging in female fetuses with a low- type imperforate anus
  • 7. Clinical Presentation Absent anal opening Abdominal distension Vomiting Passing stool from introitus/vagina Inborn Patient: • Diagnosis during detail examination
  • 8. Male ARM Local Examination: Presence or absence of the anus or fistula, Presence and exact position of the fistula, Midline raphe , scrotum & penis Urethral meatus - Meconium Development & Symmetry of buttocks VACTERL association - examination of the entire child is mandatory
  • 9. Male ARM- Local examination
  • 11. ARM with Anal dimple
  • 13. High ARM with flat bottom & Meconuria
  • 14. Absent anus with flat bottom & Bifid scrotum
  • 15. Low ARM with Fistula in scrotum
  • 16. Low ARM with meconium pearl
  • 23. Female ARM-Local Examination • Presence or absence of the anus, • Key step- Determining the number of orifices in perineum 1 orifice - Cloaca 2 orifice - RVF, Vaginal atresia, UGS, ARM without fistula 3 orifice - Vestibular Fistula, Perineal fistula, Ante posed anus, Perineal groove 4 orifice - Perineal canal, Acquired RVF
  • 24. Size of fistula Development of buttocks Symmetry of buttocks Presence of a presacral mass. VACTERL- association
  • 25. Female ARM- Absent Anal Opening
  • 26. Cloaca – Single perineal opening
  • 27. Cloaca – Single perineal opening
  • 28. Cloaca – Single perineal opening
  • 32. Recto-vaginal Fistula – 2 perineal opening
  • 33. Vaginal Atresia- 2 perineal opening
  • 34. Urogenital sinus- 2 perineal opening
  • 35. Vestibular fistula- 3 perineal opening
  • 36. Vestibular fistula- 3 perineal opening
  • 39. Perineal fistula- 3 perineal opening
  • 40. Anterior ectopic anus/ Perineal fistula- 3 perineal opening
  • 41. Perineal groove- 3 perineal opening
  • 42. Perineal canal- 4 perineal opening
  • 43. Acquired RVF- 4 perineal opening
  • 44. Systemic malformations associated with ARM PUV Bladder exstrophy UDT Renal duplications ,Hypospadias, Bifid scrotum -MMC, Diastematomyelia Hydrocephalus Encephalocele Hemivertebra ASD, PDA, Coarctation of Aorta PPHT CDH TEF, DA,
  • 45. Chromosomal Anomalies Associated with ARM  Trisomy 21 (Downs syndrome)- • Flattened face • Small head, Short neck, Protruding tongue, Poor muscle tone • Upward slanting eye lids (palpebral fissures) • Unusually shaped or small ears, Excessive flexibility • Broad, short hands with a single crease in the palm • Relatively short fingers and small hands and feet • Brushfield's spots, Short height  Trisomy 13 (Patau syndrome)  Trisomy 18 (Edwards syndrome)  Cat-eye syndrome- ARM, coloboma of the iris Coloboma of the choroid and/or optic nerve, microphthalmia,and variable external ear deformities
  • 46. Genetic syndromes with ARM VACTERL Association –vertebral, anorectal, cardiac, tracheoesophageal, renal, limb anomalies (atleast 3) Caudal Regression Syndrome-replaced with “caudal dysgenesis sequence” affect the lower back (including the spinal cord), limbs, genito-urinary tract, and the gastrointestinal tract. Currarino Syndrome- Sacral agenesis , Anorectal malformation, Presacral mass
  • 47. FG Syndrome-Mental retardation, large head, imperforate anus, congenital hypotonia and partial agenesis of corpus callosum Sirenomelia- legs are fused together Townes-Brocks syndrome (TBS), renal-ear-anal-radial syndrome Pallister Hall Syndrome hypothalamic hamartoma (panhypopituitarism), polydactyly (central), imperforate anus, and respiratory tract anomalies (bifid epiglottis and/or other laryngeal anomalies)
  • 49. Krickenbeck Classification of ARM Krickenbeck Castle in Westphalia, Germany- 2005 to provide Uniform terminology worldwide in ARM to avoid confusion and also standard surgical procedure nomenclature
  • 50. Aims of the initial assessment Threefold: 1. To determine the level of the malformation 2. To determine the integrity of sphincters and their nerve supply. 3. To document any associated anomalies that may affect survival. Decision- Primary perineal operation vs perform a colostomy (Staged Repair)
  • 51. Evaluation at Birth Blood investigations for fitness for surgery X-Ray – 1.Chest & Abdomen + Pelvis Erect view 2.Spine - Lateral view with Pelvis 3.Prone Cross table lateral view X- Ray USG Abdomen and KUB details sos USG spine & Skull if spine anomaly MCU if PUV or HUN 2D- ECHO- ASD, VSD,PDA, TOF,TGV,HLHS Sos Genitogram for cloaca
  • 55. 3 minutes prone position, femur overlapping, anal marker, X-ray beam on greater trochanter, after 18 to 24 hrs Prone Cross-table Lateral X –ray
  • 56. Prone Cross-table Lateral X –ray (For males & females without fistula) P C I A
  • 61. Ultrasonography- USG Abdomen + Pelvis, Detail USG-KUB, sos USG Spine, sos USG Skull 2D-ECHO- ASD, VSD, PDA, TOF, TGV, HLHS Sos Radiopaque contrast studies of the fistula Genitogram – Cloaca, UGS Anesthesia fitness
  • 62. Initial Management Algorithm During the first 24 h, the baby should receive intravenous fluids and antibiotics. NBM, AG charting  The presence of Associated defects should be investigated
  • 65. Transverse colostomy- Cloaca And Long seg HD Divided descending colostomy-Ideal- ARM High sigmoid loop colostomy- ARM Loop ileostomy - pouch colon Advantages & Disadvantages
  • 66. Divided descending colostomy Male ARM- Ideal High sigmoid loop colostomy
  • 67. Divided sigmoid Colostomy- Male ARM/Vestibular Fistula
  • 69. Colostomy On table- distal colonic washout- warm saline Start feeds- once stoma functional Start DC washouts on POD-3 using warm saline OD (At home-Warm water with pinch of salt added)
  • 70. Definitive Repair Blood investigation- Hemoglobin, Sr. creatinine, Sr. proteins 2D-Echo MCU Pressure Augmented Distal Colostogram sos Cystoscopy, CGS for cloaca and RVF Renal Scintigraphy- EC Scan , DMSA sos MRI Pelvis for complex urological anomaly to look for Sphincter muscle complex (SMC) development
  • 74. Definitive repair- Male ARM Male ARM –PSARP- at 3 to 5 months Anal dilatation started on POD-14 Colostomy Closure – 6 weeks after definitive surgery  Continue anal dilatation & taper it gradually as per protocol  Continue management of associated anomalies simultaneously
  • 75. Definitive repair- Female ARM • Female with colostomy- ASARP/PSARP at 3 to 5 months of age. • Female without colostomy : Planned for primary ASARP / PSARP before 6 months of age  Start on rectal washout-Use IFT no-8/9/10  Ensure adequate decompression of colon  Rectal dye study before repair ( Dilatation)  NBM for 5days-IV fluids /TPN/ Antibiotics.
  • 81. PSARP- on follow up after-2 months
  • 82. ASARP
  • 83. Cut back anoplasty in Low ARM
  • 84. Cut back anoplasty in Low ARM
  • 85. Complications associated with ARM Long Term- 1.Chronic constipation 2.Fecal incontinence(25%) 3.Urinary incontinence in cloaca (40%) ↓ Requires evaluation , management, long term follow-up
  • 86. Determine Sacral ratio = BC/AB Preoperatively to predict prognosis
  • 87. Normal SR- 0.74 & 0.77  S4,5-Normal  SR < 0.5/0.4- Incontinence/poor prognosis  S3,4,5 abnormal – Variable continent  S1,2 abnormal – Incontinent  Hemi sacrum- Unpredictable continence  Asso. MMC- Poor Prognosis
  • 89. Cloaca  Transverse colostomy sos cutan. vagionostomy sos vesicostomy  Definitive repair-1 yr- PSARP with PUM/TUM PSARVUP  Colostomy Closure
  • 91. Pouch Colon- Type 1 & 2- -Disconnection of fistula and Coloplasty of 15 cm f/b end colostomy- -Definitive repair Type 3 & 4- -Disconnection of the fistula & Excision of the pouch with an end colostomy- -Definitive repair with or without diversion colostomy
  • 92. One-stage vs. three-stage repair in anorectal malformation with rectovestibular fistula Omid Amanollahi and Saman Ketabchian1 Afr J Paediatr Surg. 2016 Jan-Mar; 13(1): 20–25. Recommends - One stage Single-stage repair versus traditional repair of high anorectal malformations, functional results’ correlation with Kelly’s score and postoperative magnetic resonance imaging findings A.N. Gangopadhyayaa , Vijayendra Kumara Ann Pediatr Surg 9:108–113 c 2013 Annals of Pediatric Surgery. Clinical assessment using the Kelly score was similar for the single-stage operation and the staged procedure, and this was supported by MRI findings. Therefore, we recommend the single-stage procedure to achieve a better outcome in intermediate and high anorectal malformation