Is Multi-disciplinary care the future of medicine?

Most families who have a baby born with an anorectal malformation never heard that was something that could happen and are in 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 reconstruction techniques much more recent than other congenital surgeries like esophageal atresia and diaphragmatic hernia from the 1950s.

Before modern techniques, anorectal malformation patients survived but suffered from constipation and fecal incontinence due to poor surgical technique, unlike esophageal atresia patients who did not survive without good repair.

Alberto Pena, who performed the first PSARP in 1980, is considered the father of pediatric colorectal surgery.

In 1992, Alberto Pena was functioning as a lone provider handling colorectal surgery, urology, gynecology, psychology, and social work for his patients.

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

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.

A good anatomic reconstruction without a good functional result accomplishes very little, analogous to correcting scoliosis but leaving the child unable to walk.

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

Cloacal defects may be prenatally diagnosed, particularly when there is a large pelvic mass, hydronephrosis, or a missing kidney identified on ultrasound.

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

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

The quality of the sacrum and spine are used to predict continence and allow confident counseling of families even 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.

The word 'cloaca' comes from French meaning 'sewer,' and birds have cloacas that combine poop and pee together in a single exit.

The Malone procedure involves taking the appendix, connecting it to the belly button, and using it to flush the colon once daily, resulting in no bowel movements for the next 24 hours.

The center performs approximately 100 Malone procedures per year.

The Malone procedure does not require continence or sphincters; as long as poop comes out at a specific time with no poop for 24 hours afterward, the child can wear normal underwear.

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

Pediatric surgery fellows perform about 14 anorectal malformation cases in 2 years of training.

A practicing pediatric surgeon performs about one anorectal malformation case per year.

Insufficient case volume is one reason for the high number of redo operations seen in colorectal surgery.

In the UK, biliary atresia and bladder exstrophy are specifically focused at certain centers and cannot be operated on in other centers because the country recognized the expertise available through the collaborative model.

In collaborative surgery for patients with myelomeningocele and both urinary and fecal incontinence, a piece of colon can be used for bladder augmentation while simultaneously making the remaining colon shorter and easier to empty, solving both problems in one operation.

The appendix can be split to create both a Malone and a Mitrofanoff procedure, which is only possible with a collaborative model.

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