Is Multi-disciplinary care the future of medicine?

Most families who have a baby born with an anorectal malformation have never heard that such a condition could happen and experience psychological shock

The Babylonian Talmud from 2000 years ago describes that if an infant whose anus is not visible is born, they should be rubbed with oil and stood in the sun, and where it shows transparent should be torn crosswise with a barley grain, representing an early description of anoplasty

The first PSARP (posterior sagittal anorectoplasty) was performed in 1980, making modern functional colorectal reconstruction techniques much more recent than other congenital surgeries like esophageal atresia and diaphragmatic hernia repairs from the 1950s

Before modern techniques, anorectal malformation patients survived but suffered from constipation and fecal incontinence due to poor surgical outcomes that rescued the anus but did not create function

The goal of colorectal reconstruction is not only anatomic repair but creating a functional anus that allows the patient to attend normal school and wear normal underwear

Alberto Pena, the father of pediatric colorectal surgery who performed the first PSARP in 1980, initially provided all aspects of care alone including colorectal surgery, urology, gynecology, psychology, and social work

Dr. Levitt trained with Alberto Pena as a medical student in 1992 and recognized he could never provide that level of complex care alone, which led to conceiving the collaborative care model

Babies with anorectal malformations are almost always full-term and healthy with no prenatal expectation of the condition

When families learn their baby has an anorectal malformation, their primary question is whether their child will be normal at age 4 during potty training, not about surgical technique elegance

The mission of colorectal surgery is achieving good functional results, not just anatomic reconstruction, as anatomic correction without function is inadequate (analogous to correcting scoliosis but leaving the child unable to walk)

The bladder, gynecologic system, and rectum all fill the pelvis with intertwined anatomy, making collaboration between colorectal, urology, and gynecology essential

Children's National is the World Center for Cloacal Care, having performed 32 cloacal reconstructions in the last 4 years, while most centers see one cloaca every 5 years

Cloacal malformations may be prenatally diagnosed through identification of large pelvic mass, hydronephrosis, or missing kidney on ultrasound

Hydrocolpos (vagina filled with urine) in newborns with cloaca can depress respiration, cause kidney failure, and cause hydronephrosis, requiring immediate management

Patients with anorectal malformations often have associated neurologic problems, may have a single kidney or chronic kidney disease, and may have abnormal gynecologic anatomy including duplicated Mullerian systems

Sacrum and spine quality are used to predict continence outcomes and allow confident prognostic discussions with families on the first day of life

A patient with a favorable malformation, good quality sacrum, and normal spine will have continence in 4 years after reconstruction

A patient born with myelomeningocele will not be continent in 4 years, but bowel management programs can achieve functional outcomes

In a cloaca, the rectum, vagina, and bladder all form one confluence with a single exit, and the surgical goal is to create three separate openings

The term 'cloaca' comes from the French word meaning sewer, and birds have cloacas that combine fecal and urinary output

The Malone procedure involves taking the appendix, connecting it to the belly button, and using it as a conduit to flush the colon once daily, resulting in no bowel movements for 24 hours and allowing a clean child who can wear normal underwear without requiring continence or sphincters

The center performs approximately 100 Malone procedures per year

In patients with myelomeningocele requiring both urinary and fecal management, bladder augmentation can be performed using a segment of colon, which simultaneously makes the bladder bigger and the colon shorter and easier to empty, solving both problems in one operation

An appendix can be split to create both a Malone channel (for colon flushing) and a Mitrofanoff channel (for bladder catheterization) from a single appendix

There are 600 newborns with anorectal malformations born per year in the United States

Pediatric surgery fellows perform about 14 anorectal malformation cases in 2 years of training, and a general pediatric surgeon does about one case per year

Low surgical volume is one reason for poor outcomes and the need for reoperations in anorectal malformation surgery, supporting the case for regional centers of expertise

In the UK, biliary atresia and bladder exstrophy are specifically concentrated at certain centers and cannot be operated on at other centers due to recognized expertise benefits of the collaborative model

The colorectal program provides care from 20-week fetal diagnosis through adulthood, including a new transition program with Erin Tel in collaboration with MedStar for adult care

Fifteen years ago there was one collaborative colorectal center in Cincinnati, and now such centers have spread throughout the world

If a patient does not have an appendix, one can be surgically created for the Malone procedure