# VACTERL Association — GCMD Library living collection

Everything in the library about VACTERL association — built automatically from dossiers that name it.

Updated: n/a · 3 episodes · 39 cited statements

## Episodes
### Acute Management
- [Fetal and Newborn Management of Cloacal Malformations](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988) — podcast · 13:53 · [machine version](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988.md)

### Evidence & Research
- [Quick Literature Updates Episode 14](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776) — video · 4:46 · [machine version](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776.md)
- [Journal of Pediatric Surgery Article Review: March 2023](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854) — podcast · 11:42 · [machine version](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854.md)

## Chapters
- [0:00](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=0) Introduction and First Paper Overview (Ep 1)
- [1:03](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=63) VACTERL and Gynecologic Anomalies Study (Ep 1)
- [2:08](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=128) Pain Control After Nuss Procedure (Ep 1)
- [3:31](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=211) Anal Sphincter Reconstruction After Hirschsprung Pull-Through (Ep 1)
- [4:29](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=269) Closing Remarks (Ep 1)
- [0:00](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=0) Introduction and Episode Overview (Ep 2)
- [1:27](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=87) VACTERL and Gynecologic Anomalies in Anorectal Malformations (Ep 2)
- [4:52](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=292) Sphincter Reconstruction for Post-Hirschsprung Incontinence (Ep 2)
- [7:25](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=445) Contrast Enema Utility Before Stoma Reversal (Ep 2)
- [10:32](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=632) Summary and Closing (Ep 2)
- [0:00](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=0) Introduction and Prenatal Diagnosis of Cloacal Malformations (Ep 3)
- [3:25](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=205) Prenatal Counseling and Delivery Planning (Ep 3)
- [5:04](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=304) Immediate Postnatal Assessment and VACTERL Screening (Ep 3)
- [7:00](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=420) Hydrocolpos Management and Renal Protection (Ep 3)
- [9:15](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=555) Colostomy Creation and Placement Considerations (Ep 3)
- [10:24](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=624) Long-term Follow-up and Prognostic Factors (Ep 3)

## Statements
Every statement is attributed, typed, and timestamped; the link is the citation.
- Abdominal or pelvic cystic masses are found on prenatal ultrasound in 52% of cloacal malformation cases. (epidemiological) [Ep 3 · 1:21](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=81)
- Hydronephrosis is seen on prenatal ultrasound in approximately 49% of cloacal malformation cases. (epidemiological) [Ep 3 · 1:32](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=92)
- Intraluminal calcifications are a specific prenatal ultrasound finding for cloacal malformations, forming when urine mixes with meconium inside the body, indicating communication between bowel and urinary tract. (clinical) [Ep 3 · 1:44](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=104)
- Up to 70% of non-syndromic cloacal malformation patients have VACTERL association. (epidemiological) [Ep 3 · 2:25](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=145)
- VACTERL association is defined as having 3 or more of the following: vertebral defects, anorectal malformations, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb defects. (clinical) [Ep 3 · 2:41](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=161)
- Vaginal delivery is often safe and feasible for cloacal malformations. (clinical) [Ep 3 · 4:02](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=242)
- Delivery must occur at a specialized tertiary care center with a top-level NICU and subspecialists available. (guideline) [Ep 3 · 4:07](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=247)
- Immediate postnatal priorities include stabilizing breathing and circulation, obtaining IV access, starting antibiotics, and ensuring urine and stool can exit the body. (guideline) [Ep 3 · 4:27](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=267)
- Initial NICU stay typically lasts 2 to 3 weeks for stabilization and early procedures. (clinical) [Ep 3 · 4:50](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=290)
- Definitive reconstructive surgery for cloacal malformations is usually performed between 3 and 12 months of age. (guideline) [Ep 3 · 4:57](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=297)
- Passing a nasogastric tube immediately after birth checks for esophageal atresia or tracheoesophageal fistula, which occurs in 7-11% of babies with anorectal malformations. (clinical) [Ep 3 · 5:22](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=322)
- Cardiac defects occur in up to 40% of cloacal malformation cases, requiring early echocardiogram before considering abdominal surgery. (epidemiological) [Ep 3 · 5:40](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=340)
- The proper physical examination technique for cloacal malformations requires gently lifting the labia edges upwards and outwards toward the examiner's shoulders to visualize the single small orifice. (clinical) [Ep 3 · 6:07](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=367)
- The common misdiagnosis error is identifying a cloacal malformation as a rectovaginal fistula; true rectovaginal fistulas are extremely rare. (clinical) [Ep 3 · 6:31](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=391)
- Hydrocolpos is found in up to one-third of cloacal malformation cases and can be lethal if not managed quickly. (clinical) [Ep 3 · 7:00](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=420)
- Hydrocolpos results from massive distention of the shared vaginal structure with trapped fluid (urine and mucus) when the single exit opening is inadequate. (clinical) [Ep 3 · 7:05](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=425)
- The distended vagina in hydrocolpos compresses the bladder, blocking both ureterovesical junctions and causing severe bilateral hydroureteronephrosis. (clinical) [Ep 3 · 7:25](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=445)
- The vagina must be drained immediately in hydrocolpos, first attempting catheter placement confirmed by ultrasound, or creating a vaginostomy if catheterization fails or is inadequate. (guideline) [Ep 3 · 7:50](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=470)
- Vaginal drainage must be performed before considering other urologic surgeries like vesicostomy or nephrostomy tubes, as the hydroureteronephrosis is caused by vaginal compression of the bladder. (guideline) [Ep 3 · 8:13](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=493)
- Over 80% of cloacal malformation patients have associated urinary tract anomalies. (epidemiological) [Ep 3 · 8:46](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=526)
- Vesicoureteral reflux (VUR) is seen in approximately 50% of cloacal malformation patients. (epidemiological) [Ep 3 · 8:51](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=531)
- Hydronephrosis occurs in 30% of cloacal malformation patients. (epidemiological) [Ep 3 · 8:57](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=537)
- Between 50% and 75% of cloacal malformation patients will develop chronic kidney disease over their lifetime. (epidemiological) [Ep 3 · 8:57](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=537)
- Approximately 17% of cloacal malformation patients eventually progress to end-stage renal disease requiring dialysis or transplant. (epidemiological) [Ep 3 · 9:06](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=546)
- Colostomy creation is typically performed within 24 to 48 hours after birth as the second major surgical priority. (guideline) [Ep 3 · 9:22](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=562)
- The ideal colostomy placement is in the descending colon just past where it is naturally fixed down, which prevents prolapse and preserves distal bowel length for future reconstruction. (clinical) [Ep 3 · 9:38](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=578)
- Transverse colostomy should be avoided because it has higher risk of metabolic acidosis (due to greater fluid and electrolyte absorption) and higher prolapse risk. (clinical) [Ep 3 · 10:01](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=601)
- Low sigmoid colostomy should be avoided because it can interfere with future pelvic reconstruction and may require later relocation. (clinical) [Ep 3 · 10:12](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=612)
- Spinal ultrasound is performed early to screen for tethered spinal cord, which can affect nerve function to bladder and bowel. (clinical) [Ep 3 · 10:44](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=644)
- Sacral X-rays should be delayed until after 3 months of age because the bones are not fully calcified before then, making earlier X-rays uninformative. (clinical) [Ep 3 · 11:03](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=663)
- A common channel length of less than 3 centimeters is considered moderate complexity and is associated with better chance of achieving volitional bladder emptying. (clinical) [Ep 3 · 11:34](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=694)
- Over 50% of cloacal malformation patients have müllerian anomalies affecting the uterus, cervix, or vagina. (epidemiological) [Ep 3 · 12:05](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=725)
- Approximately 40% of cloacal malformation patients develop obstructive menstrual problems at puberty, causing significant pain and potential complications including infection or endometriosis. (epidemiological) [Ep 3 · 12:10](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=730)
- The foundation for long-term success in bowel function, bladder control, and gynecologic function is established in the first few days through proper initial triage, hydrocolpos drainage, and thoughtful colostomy placement. (opinion) [Ep 3 · 12:45](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=765)
- The concept of gynecologic association with anorectal malformation is relatively new, launched in 2005 with the creation of the first ever clinic at Cincinnati Children's Hospital. — Marc Levitt (clinical) [Ep 2 · 3:42](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=222)
- The sphincter reconstruction technique addresses Hirschsprung patients who had iatrogenic injury at their original pull-through, with overstretched sphincters leaving them fecally incontinent. — Marc Levitt (clinical) [Ep 2 · 5:34](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=334)
- Of 6 patients who underwent sphincter reconstruction, 4 have done extremely well, while 2 have Down syndrome with behavioral delay in their capacity to demonstrate successful bowel function. — Marc Levitt (clinical) [Ep 2 · 6:14](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=374)
- There is no mechanism to expect that distal to a stoma there is continuing disease or continuing problem that needs to be checked for before stoma closure. — Marc Levitt (opinion) [Ep 2 · 9:47](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=587)
- In patients with necrotizing enterocolitis, it is reasonable to get a contrast study before stoma takedown. — Todd (opinion) [Ep 2 · 10:15](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=615)

## Host summaries
Recaps by a host of what the experts said — not the host's own clinical position.
- Cloacal malformations occur only in females with an incidence of approximately 1 in 50,000 births. — The host summarizing the discussion [Ep 3 · 0:21](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=21)
- In cloacal malformations, the GI tract, urinary system, and gynecologic tract all exit through a single opening due to failure of separation during development. — The host summarizing the discussion [Ep 3 · 0:21](https://library.globalcastmd.com/watch/fetal-and-newborn-management-of-cloacal-malformations-13988?t=21)
- The study by Ahmad et al. examined 384 females with anorectal malformations using the Pediatric Colorectal and Pelvic Learning Consortium database. — Ellen Encisco summarizes what Dr. Alex Halpern said [Ep 1 · 1:03](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=63)
- About 27% of patients with anorectal malformations had a gynecologic anomaly. — Ellen Encisco summarizes what Dr. Alex Halpern said [Ep 1 · 1:22](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=82)
- About 46% of females with anorectal malformations had VACTERL association, defined as having the anorectal malformation plus two of: vertebral, cardiac, tracheoesophageal, renal, or limb anomaly. — Ellen Encisco summarizes what Dr. Alex Halpern said [Ep 1 · 1:22](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=82)
- Having VACTERL association was associated with more gynecologic anomalies compared to patients without VACTERL association. — Ellen Encisco summarizes what Dr. Alex Halpern said [Ep 1 · 1:39](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=99)
- The association between VACTERL and gynecologic anomalies was especially true when renal anomalies were found. — Ellen Encisco summarizes what Dr. Alex Halpern said [Ep 1 · 1:49](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=109)
- The association between VACTERL and gynecologic anomalies was especially true when patients had recto vestibular or rectal perineal fistulas. — Ellen Encisco summarizes what Dr. Alex Halpern said [Ep 1 · 1:49](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=109)
- For females with anorectal malformations and VACTERL association, especially renal anomalies, clinicians should look carefully for gynecologic anomalies. — Ellen Encisco summarizes what Dr. Alex Halpern said [Ep 1 · 1:57](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=117)
- Thoracic epidural and intercostal nerve cryoablation are two options for pain control in patients undergoing a Nuss procedure for pectus excavatum. — Alex Halpern summarizing a resource [Ep 1 · 2:30](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=150)
- Dr. Tsai and his team from Penn State conducted a retrospective chart review of kids who underwent Nuss procedure between 2002 and 2020, comparing outcomes and costs of intercostal nerve cryoablation and epidural. — Alex Halpern summarizing a resource [Ep 1 · 2:42](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=162)
- The intercostal nerve cryoablation group had lower rates of PCA use compared to thoracic epidural. — Alex Halpern summarizing a resource [Ep 1 · 2:56](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=176)
- The intercostal nerve cryoablation group had lower total morphine milligram equivalent requirement compared to thoracic epidural. — Alex Halpern summarizing a resource [Ep 1 · 2:56](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=176)
- The intercostal nerve cryoablation group had shorter length of stay in the hospital compared to thoracic epidural. — Alex Halpern summarizing a resource [Ep 1 · 2:56](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=176)
- The intercostal nerve cryoablation group had longer operative times compared to thoracic epidural. — Alex Halpern summarizing a resource [Ep 1 · 3:07](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=187)
- The intercostal nerve cryoablation group had higher total hospitalization cost compared to thoracic epidural. — Alex Halpern summarizing a resource [Ep 1 · 3:07](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=187)
- Both intercostal nerve cryoablation and thoracic epidural have pros and cons, with cryoablation showing decreased total opioid use and decreased length of stay but increased operating room times and increased total cost. — Alex Halpern summarizing a resource [Ep 1 · 3:14](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=194)
- The Bokova et al. study is a multi-institutional one-year outcome study of anal sphincter reconstruction for patulous sphincter in 6 patients. — Cecilia Gigena summarizes what Dr. Alex Halpern said [Ep 1 · 3:48](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=228)
- Of the 6 patients in the sphincter reconstruction study, 2 had Down syndrome and underwent redo pull-through with anal sphincter reconstruction. — Cecilia Gigena summarizes what Dr. Alex Halpern said [Ep 1 · 4:00](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=240)
- Four patients in the sphincter reconstruction study received only anal sphincter reconstruction without redo pull-through. — Cecilia Gigena summarizes what Dr. Alex Halpern said [Ep 1 · 4:00](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=240)
- The 4 patients who received only anal sphincter reconstruction achieved voluntary bowel movements with higher productivity and confidence. — Cecilia Gigena summarizes what Dr. Alex Halpern said [Ep 1 · 4:12](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=252)
- Anal sphincter reconstruction appears to be an option for overstretched anal sphincters in patients with Hirschsprung disease after a pull-through. — Cecilia Gigena summarizes what Dr. Alex Halpern said [Ep 1 · 4:12](https://library.globalcastmd.com/watch/quick-literature-updates-episode-14-7776?t=252)
- VACTERL is an association defined by at least 3 anomalies in the vertebral, anorectal, cardiac, tracheoesophageal, renal, or limb systems. — The host summarizing the discussion [Ep 2 · 2:00](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=120)
- In a study of 834 females with anorectal malformations, patients with ARMs and VACTERL association had more gynecologic anomalies, especially in recto vestibular and rectal perineal fistulas. — The host summarizing the discussion [Ep 2 · 2:24](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=144)
- In ARM patients with VACTERL association, when one of the associated anomalies was in the renal system, there was an even higher risk of having an associated gynecological anomaly. — The host summarizing the discussion [Ep 2 · 2:45](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=165)
- Besides cloaca, rectal perineal and recto vestibular fistulas in ARM patients also have an association with gynecologic anomalies. — Cecilia Gigena summarizing the discussion [Ep 2 · 3:21](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=201)
- By sphincter reconstruction, the sphincters are tightened, which allows patients to feel the stool coming and therefore improve their bowel control. — The host summarizing the discussion [Ep 2 · 5:53](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=353)
- In a Dutch study of 224 patients 3 years old or younger needing stoma reversal between 1998 and 2018, 10% had strictures prior to reversal, and 95% of those with strictures had necrotizing enterocolitis. — Cecilia Gigena summarizing the discussion [Ep 2 · 8:13](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=493)
- Only one stricture case was not in a NEC patient; it was a patient treated for duodenal atresia who had a volvulus afterwards with ischemic injury of the bowel. — Cecilia Gigena summarizing the discussion [Ep 2 · 8:46](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=526)
- Of all patients in the stoma reversal study, only 68% had a contrast enema, which detected 92% of the strictures. — Cecilia Gigena summarizing the discussion [Ep 2 · 8:57](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=537)
- Most patients who develop strictures after stoma creation are those with vascular problems, particularly NEC patients. — Cecilia Gigena summarizing the discussion [Ep 2 · 9:07](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=547)
- In patients with intestinal atresia, there is a chance of having two strictures, so if an atresia is found during antegrade enema, a retrograde enema should also be performed to check for a second stricture. — Cecilia Gigena summarizing the discussion [Ep 2 · 9:24](https://library.globalcastmd.com/watch/journal-of-pediatric-surgery-article-review-march-2023-13854?t=564)

## Changelog
- Oct 12: Published again automatically — condition is back above threshold
- Oct 12: 3 items added automatically
- Oct 12: 5 items no longer name VACTERL association
- Sep 7: Unpublished automatically — folded or below threshold
- Sep 7: 5 items added automatically

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