Anorectal Malformations
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Quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "If the surgeon does not have a clear and reliable image that shows the rectum located below the coccyx, he or she should never approach a patient posterior sagittally without a colostomy and without a distal colostogram. The distal colostogram, which is by far the most valuable study in defining the anorectal anatomy, can be done in patients with anorectal malformations only when the patient already has a colostomy. We have seen catastrophic complications during the performance of posterior sagittal operations in male patients who did not have a distal colostogram." quote from "P…
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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - 1. For all anal stenosis (and rectal atresia) cases you must check for a presacral mass. 2. For any such presacral mass, always make sure there is no dural component (pelvic plus spinal MRI) and, if present, involve neurosurgery. 3. If Currarino syndrome, genetic testing is warranted for the patient and first-degree relatives. 4. For management, removal of the mass is key which will likely solve much of the constipation. 5. If the anus is narrow that must be treated; an anal canal sparing technique can accomp…
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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "It is crucial not to chase the anteriorly directed fistula toward the scrotum, as it runs parallel to the urethra. Attempting to trace this fistula surgically could lead to significant complications, including spongiosum bleeding and urethral injury. Instead, the focus should be on mobilizing the distal rectum and avoiding dissection of the anterior rectal wall to avoid a urethral injury."
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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "while the AP view might misleadingly suggest an underdeveloped sacrum, the lateral view typically provides a more accurate representation. The pelvic tilt can distort the appearance of the sacrum and coccyx in the AP view, leading to a falsely low sacral ratio. The lateral view, however, allows for a more reliable calculation by minimizing distortions caused by patient positioning"
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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "A recent study from the ARM-Net registry highlights the need for sufficient screening and vigilant management of high-grade VUR in ARM patients. This study underscores the fact that despite normal kidneys on US, a significant proportion of patients may still have high-grade VUR, necessitating prophylaxis. All patients with ARM should have a newborn screen renal US and, in most, a follow-up VCUG should be done to assess for VUR."
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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "When the common channel is <3 cm, it is possible to mobilize the UG complex as a unit to the perineum, the TUM. In general, a urethral length of >1.5 cm is required to avoid urine leakage and to avoid pulling down the bladder neck out of the urogenital diaphragm with the mobilization. When the length of the common channel is >3 cm or the urethra is <1.5 cm, a UG separation is utilized, leaving the common channel untouched and adding to it the native urethra. With the UG separation, the vagina and…
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Quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "For patients with uterine abnormalities, obstruction of menses is possible, and surgical correction may become necessary. Pregnancies in patients with vaginal anomalies are often considered high risk, and delivery options need to be discussed with maternal fetal medicine. The pelvic floor muscles are often not normally developed or have been iatrogenically altered, making vaginal delivery after pregnancy more traumatic. This is especially true if the perineal body was divided during reconstruction. Most pati…
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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "Low ARMs, like recto-vestibular and perineal fistula, tend to be associated with congenital rectal dilation above the fistula. The dilated rectum is prone to constipation, and early bowel management is recommended. Bowel management in this population utilizes laxatives and fiber to keep the rectum empty and avoid distention of the dilated rectum. The pelvic floor and sphincter muscle may be more robust in these distal malformations; however, incontinence is still possible, especially in the setting of an ass…
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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "Up to 10% of female infants with ARM have associated gynecologic anomalies, including an absent vagina in less than 1% of cases. Vaginoscopy is helpful to characterize the fistula and check for a vagina. An absent vagina may not be immediately apparent in the setting of a recto-vestibular fistula, especially if the fistula is mistaken for a vagina and a rectovaginal fistula is assumed to be present. In fact, absence of the vagina is only recognized about 50% of the time prior to anorectoplasty. When an absen…
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quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "Constipation is a constant sequela and should be treated aggressively. (This is true also for male patients with perineal fistulas.) We have learned that the lower the defect, the greater the chance of constipation. We have also learned that constipation is a self-perpetuating and self-aggravating condition that eventually produces severe megacolon, chronic faecal impaction, and overflow pseudo-incontinence; it must be vigilantly avoided."
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quotes from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "The repair of cloacas with longer common channels (>3 cm) represents a real technical challenge and requires a great deal of experience in the management of these cases. For that kind of patient, the pediatric surgeon and/or pediatric urologist should have experience in the management of the urinary structures, including bladder reconstructions, bladder neck reconstructions, ureteral reimplantations, bladder augmentation, and Mitrofanoff procedures, as well as vaginal replacements using rectum, colon, or small bowel." "If the common channel is longer than 5 cm, we recommend …
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quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "When a baby is born with cloaca, the surgeon must keep in mind that approximately 50% of these patients suffer from a very giant vagina full of fluid (“hydrocolpos”). The hydrocolpos may compress the trigone, interfering with the drainage of the ureters and therefore provoke bilateral megaureters and hydronephrosis. All babies with a cloaca should have a complete urologic evaluation at birth, including an ultrasound of the kidneys and ultrasound of the pelvis. The baby should not be taken to the operating room without this evaluation. If the baby suffers from hydrocolpos, it is m…
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