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Questions and Answers
What is the primary function of the longitudinal muscle fibers in the context of bowel control?
What is the primary function of the longitudinal muscle fibers in the context of bowel control?
- Elevating the anus (correct)
- Assisting in the funnel shape of striated muscles
- Forming the internal sphincter
- Contracting against the wall of the rectum and anus
A patient presents with meconium per urethra, a flat perineum, and no pigmentation at the site of the anus. According to the classification of anorectal malformations, which type of anomaly is most likely?
A patient presents with meconium per urethra, a flat perineum, and no pigmentation at the site of the anus. According to the classification of anorectal malformations, which type of anomaly is most likely?
- Cloacal Anomaly
- Intermediate Anomaly
- High Anomaly
- Low Anomaly (correct)
In a high anorectal malformation, what is the significance of a cloacal anomaly?
In a high anorectal malformation, what is the significance of a cloacal anomaly?
- Normal sphincter contraction
- Absence of a perineal opening
- Presence of a common channel between the urogenital sinus and rectum (correct)
- Bulged membrane at the normal location of the anus
During the examination of a male newborn, an ectopic opening from the rectum is detected along the perineal raphe. According to the PENA classification, what type of anomaly is this?
During the examination of a male newborn, an ectopic opening from the rectum is detected along the perineal raphe. According to the PENA classification, what type of anomaly is this?
A newborn female is diagnosed with a vertebral abnormality alongside an anorectal malformation. What does this combination suggest?
A newborn female is diagnosed with a vertebral abnormality alongside an anorectal malformation. What does this combination suggest?
What is the primary purpose of daily dilation of the anal opening for 3-5 months after surgical correction of an anorectal malformation?
What is the primary purpose of daily dilation of the anal opening for 3-5 months after surgical correction of an anorectal malformation?
A surgeon is performing a PSARP procedure. What is the rationale for ensuring equal distribution of muscle fibers during the midline dissection?
A surgeon is performing a PSARP procedure. What is the rationale for ensuring equal distribution of muscle fibers during the midline dissection?
What is the most appropriate first step in the management of a newborn diagnosed with a high anorectal malformation?
What is the most appropriate first step in the management of a newborn diagnosed with a high anorectal malformation?
What information is gained from performing lower abdominal and perineal CT/MRI during the investigation of anorectal malformations?
What information is gained from performing lower abdominal and perineal CT/MRI during the investigation of anorectal malformations?
Which of the following is the most likely outcome of a delay in diagnosing anorectal malformations?
Which of the following is the most likely outcome of a delay in diagnosing anorectal malformations?
What is the primary significance of the 'bowel skin distance' measured in the midline sagittal plane during the evaluation of anorectal malformations?
What is the primary significance of the 'bowel skin distance' measured in the midline sagittal plane during the evaluation of anorectal malformations?
During a PSARP procedure, what is the recommended initial approach to finding the dissection plane?
During a PSARP procedure, what is the recommended initial approach to finding the dissection plane?
In managing anorectal malformations, what is the rationale for separating the bowel from the urinary tract or vagina during surgery?
In managing anorectal malformations, what is the rationale for separating the bowel from the urinary tract or vagina during surgery?
A clinician notes the absence of external sphincter contraction upon cutaneous stimulation of the anus in a newborn. What does this finding suggest?
A clinician notes the absence of external sphincter contraction upon cutaneous stimulation of the anus in a newborn. What does this finding suggest?
What is a potential complication of rectourinary fistulas following surgical repair?
What is a potential complication of rectourinary fistulas following surgical repair?
In the context of anorectal malformations, what is the most critical immediate goal of treatment following the diagnosis?
In the context of anorectal malformations, what is the most critical immediate goal of treatment following the diagnosis?
What factor primarily dictates whether an anorectal malformation is classified as a 'high' anomaly rather than a 'low' anomaly?
What factor primarily dictates whether an anorectal malformation is classified as a 'high' anomaly rather than a 'low' anomaly?
During a Posterior Sagittal Anorectoplasty (PSARP) for a male patient, what specific anatomical landmark confirms the correct dissection plane when addressing a recto-urethral fistula?
During a Posterior Sagittal Anorectoplasty (PSARP) for a male patient, what specific anatomical landmark confirms the correct dissection plane when addressing a recto-urethral fistula?
In managing a newborn girl with a suspected high anorectal malformation, which clinical finding would strongly suggest the presence of a cloacal anomaly?
In managing a newborn girl with a suspected high anorectal malformation, which clinical finding would strongly suggest the presence of a cloacal anomaly?
What is the primary rationale behind performing daily anal dilations for several months following surgical correction of an anorectal malformation?
What is the primary rationale behind performing daily anal dilations for several months following surgical correction of an anorectal malformation?
Which diagnostic imaging modality is most beneficial in evaluating the pelvic anatomy and the relationship of the rectal musculature in a complex anorectal malformation?
Which diagnostic imaging modality is most beneficial in evaluating the pelvic anatomy and the relationship of the rectal musculature in a complex anorectal malformation?
In the context of anorectal malformations, how does the presence of vertebral abnormalities in females influence the diagnosis and management?
In the context of anorectal malformations, how does the presence of vertebral abnormalities in females influence the diagnosis and management?
What surgical approach is typically preferred for the initial management of a high anorectal malformation discovered in a newborn?
What surgical approach is typically preferred for the initial management of a high anorectal malformation discovered in a newborn?
In the management of anorectal malformations, why is it important to carefully separate the bowel from the urinary tract or vagina during surgical repair?
In the management of anorectal malformations, why is it important to carefully separate the bowel from the urinary tract or vagina during surgical repair?
During the evaluation of a newborn with a suspected anorectal malformation, what does the lack of external sphincter contraction upon cutaneous stimulation of the anus suggest?
During the evaluation of a newborn with a suspected anorectal malformation, what does the lack of external sphincter contraction upon cutaneous stimulation of the anus suggest?
What is a potential long-term complication of rectourinary fistulas following surgical repair of anorectal malformations, particularly if the separation was incomplete?
What is a potential long-term complication of rectourinary fistulas following surgical repair of anorectal malformations, particularly if the separation was incomplete?
What implication does the VACTERL syndrome have in the context of anorectal malformations?
What implication does the VACTERL syndrome have in the context of anorectal malformations?
In a newborn with an anorectal malformation, what does the presence of meconium in the urine or at the urethral meatus typically indicate?
In a newborn with an anorectal malformation, what does the presence of meconium in the urine or at the urethral meatus typically indicate?
During the posterior sagittal anorectoplasty (PSARP) approach, it's mentioned that the midline dissection ensures equal distribution of muscle fibers on the left and the right. What is the primary functional outcome expected from this careful dissection?
During the posterior sagittal anorectoplasty (PSARP) approach, it's mentioned that the midline dissection ensures equal distribution of muscle fibers on the left and the right. What is the primary functional outcome expected from this careful dissection?
What is the rationale behind delaying the measurement of 'bowel skin distance' until after 24 hours of life in newborns with suspected anorectal malformations?
What is the rationale behind delaying the measurement of 'bowel skin distance' until after 24 hours of life in newborns with suspected anorectal malformations?
Flashcards
Continence Mechanism
Continence Mechanism
The normal continence mechanism relying on the internal and external sphincters.
Smooth Muscle Role
Smooth Muscle Role
Muscle that forms the internal sphincter, aiding in bowel control.
Striated Muscle Role
Striated Muscle Role
Complex of levator ani & external sphincter, crucial for bowel control.
Horizontal Muscles
Horizontal Muscles
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Longitudinal Muscle fibers
Longitudinal Muscle fibers
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Anorectal Malformations Cause
Anorectal Malformations Cause
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Typical Rectum Features
Typical Rectum Features
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Anorectal Malformations Fate
Anorectal Malformations Fate
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Meconium per urethra
Meconium per urethra
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Stenotic opening
Stenotic opening
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PENA classification
PENA classification
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Cloacal Anomaly
Cloacal Anomaly
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Bowel Skin Distance
Bowel Skin Distance
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Plain X-ray positions
Plain X-ray positions
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3-Staged procedures
3-Staged procedures
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Anus anomalies cause
Anus anomalies cause
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Rectum anomalies cause
Rectum anomalies cause
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Flat perineum/buttocks
Flat perineum/buttocks
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Well-formed perineum/buttocks
Well-formed perineum/buttocks
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No orifice
No orifice
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Rectourinary fistula
Rectourinary fistula
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VACTERL syndrome
VACTERL syndrome
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Treatment goals
Treatment goals
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Position for PSARP
Position for PSARP
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Study Notes
Normal Continence Mechanism
- Bowel control relies on smooth and striated muscles
- Smooth muscle refers to the internal sphincter
- Striated muscle refers to the complex of the levator ani and external sphincter
- Striated muscles are funnel-shaped, originating from the pubis, pelvic rim, and sacrum
- These muscles converge at the perineum, merging with the internal and external sphincters
- The striated muscle complex contains horizontal and longitudinal muscles
- Horizontal muscles contract against the rectum and anus
- Longitudinal muscle fibers elevate the anus
Anorectal Malformations: Anomalies of the Anus and Rectum
- Anomalies of the anus: Caused by abnormal growth and fusion of embryonic anal hillocks, rectum normally developed and sphincters usually intact, proper treatment allows the sphincter to function normally
- Anomalies of the rectum: Caused by faulty cloaca division into the urogenital sinus and rectum via the urorectal septum,internal sphincter and striated muscle complex are hypoplastic, surgical repair results in varying degrees of continence
Classification of Anorectal Malformations
- Low anomalies predictors: Meconium per urethra, a flat perineum and buttocks, no pigmentation or dimple at the anus site
- High anomalies predictors: Stenotic opening in the perineum, a bulged membrane seen at the normal anus location, a well-formed perineum and buttocks
- Cloacal anomaly is the most severe high deformity with a common channel between the urogenital sinus and rectum and a single perineal opening
PENA Classification of Anorectal Malformations
- Male malformations: Perineal fistula, Rectourethral fistula (bulbar, prostatic), Rectovesical fistula, Imperforate anus without fistula, and Rectal atresia
- Female malformations: Perineal fistula, Vestibular fistula, Persistent cloaca (<3 cm or >3 cm common channel), Imperforate anus without fistula, and Rectal atresia
Clinical Pictures of Anorectal Malformations
- Low anomalies clinical signs: Ectopic opening from the rectum can be detected in the perineal raphe (males) or lower vagina, vestibule, or fourchette (females)
- High anomalies clinical signs: No orifice or fistula, meconium in the urethral meatus, urine, or upper vagina, absence of external sphincter contraction with cutaneous stimulation of the anus
Complications of Anorectal Malformations
- Associated anomalies such as VACTERL syndrome occur in up to 70% of cases with high anomalies
- Delay in diagnosis leads to excessive large bowel distention and perforation
- Rectourinary fistula leads to reflux of urine into the rectum and colon resulting in absorption of ammonium chloride and acidosis
- Colon contents reflux into the urethra, bladder, and upper tracts resulting in recurrent pyelonephritis
Associated Anomalies Information
- Anomalies of vertebrae and UT occur in 2/3 of all patients with high anomalies and 1/3 of males with low anomalies
- Vertebral abnormalities in females indicate a high imperforate anus
Investigations for Anorectal Malformations
- Plain X-ray Position: Cross-table lateral film and Invertogram
- Plain X-ray Timing: After 24 Hours
- Bowel skin distance is measured in the midline sagittal plane
- <15 mm suggests a low anomaly
-
15 mm indicates a high anomaly
- Lower abdominal & Perineal CT & MRI: Defines pelvic anatomy and location regarding rectal musculature
- Lumbosacral spine MRI: Identifies spinal cord anomalies such as a tethered filum terminale
Treatment Goals
- Relieve obstruction and allow the passage of stool
- Place the rectal pouch on the perineum, positioned correctly within the muscle complex
- Separate the bowel from the urinary tract/vagina by closing the fistula
Treatment Methods
- Low Anomalies: Single-stage primary repair or delayed repair
- High Anomalies: 3-staged procedures: Colostomy and mucous fistula formation, posterior sagittal anorectoplasty (PSARP) after 4-6 weeks, closure of the colostomy after several months
- Approach: Perineal
- Timing: In the newborn period
- Method: Use a muscle stimulator to determine the site of the sphincter complex, anteriorly placed anal opening is completely mobilized and transferred to a normal position
Post-operative Care
- The anal opening is dilated daily for 3-5 months to prevent stricture formation and allow for growth
Surgical Management - Posterior Sagittal Anorectoplasty (PSARP)
- Position: Jack knife position (Prone)
- Primary PSARP in a male operative image for recto bulbar fistula
Steps for surgery
- Midline dissection ensuring distribution of muscle fibers on the left and the right
- Rectumis identified and stay silk sutures are placed
- Rectum is opened above the position of the fistula
- Rectum is opened further until the fistula is seen, marked with a silk stay suture
Considerations for dissection plane
- Start on the lateral walls of the rectum and then follow the plane anteriorly and dissect off the urethra
- Dissection should be on the wall of the rectum
Additional Steps
- The muscle complex has to be demonstrated
- The rectum is tacked during the closure of the posterior sagittal incision to prevent future rectal prolapse
- The anoplasty is performed using a standard 16-suture technique
- The rectum is trimmed
- The anoplasty is complete
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