Cincinnati Children’s Hospital
Medical Center Case Study:
case study describes work at Cincinnati Children’s Hospital Medical
Center that demonstrates the multidisciplinary, collaborative effort to
care for pediatric patients with anorectal malformations (ARM).
A male patient was born with an anorectal malformation and bilateral
pelviectasis. He was born to a G3P3 healthy female. After birth, it was
noted he had a high imperforate anus, bifid scrotum and hypospadias. He
had a divided colostomy performed on day of life two.
The family elected to travel to Cincinnati, Ohio to bring their child to
the Alberto Peña, MD, Colorectal Center at Cincinnati Children’s
Hospital Medical Center for care of the ARM and the urologic issues.
On initial assessment of the patient’s chart, it was apparent that he
required a combined approach to manage his anorectal malformation and
On initial assessment of all patients with anorectal malformations, we
look for associated defects. This not only gives us a good idea of other
anomalies, but can help us predict the future continence of the patient.
The screening process includes a voiding cystourethrogram and renal
ultrasound to assess the urinary system. The spinal cord is also
assessed because there is an association of anorectal malformations,
spinal anomalies, tethered cord and possible presacral masses. This can
all be seen with a spinal MRI. If needed, the heart will be assessed
with an echocardiogram if there is any suspicion of cardiac disease.
To look at the severity of the anorectal malformation, a high pressure
distal colostogram is obtained. This helps us plan our surgical
operation and give the parents a good idea of the type of operation the
baby will need.
Diagnosis of anomalies
This patient was identified as having a prostatic fistula on the distal
colostogram. There was enough colon to perform a posterior sagittal
incision without entering the abdomen. In our experience, patients with
an ARM and hypospadias have been at higher risk of associated urologic
issues and potential bladder dysfunction. This was found to be true with
this patient; he was found to have Grade 2 vesicoureteral reflux on his
VCUG. His renal function and kidneys looked normal.
On his screening MRI, he was found to have a shortened sacrum and a
tethered cord with a cord lipoma. The family was counseled at length
regarding the prostatic fistula and the long term prognosis of bladder
and bowel control.
We discussed the findings of the studies, including the implication for
decreased potential for bladder and bowel control, and the possibility
of clean intermittent catheterization and enemas in the future to stay
clean and dry.
Both urology and neurosurgery were consulted and an overall plan of care
was developed for the patient. Because of the severity of the tethered
cord, Francesco Mangano, DO, director of pediatric neurosurgery,
performed a release of his tethered cord and removal of the lipoma prior
to any other interventions.
Cystoscopy and repair of the ARM was performed by Belinda Hsi Dickie,
MD, PhD, pediatric surgeon from the colorectal team, eight weeks after
the tethered cord release. The cystoscopy was performed by our pediatric
urology team to delineate the anatomy. The repair of the ARM was all
done posterior sagittally with no need to enter the abdomen. The patient
recovered without incident, and was able to void after the catheter was
removed two weeks after surgery.
While he was still diverted from any stool near his perineum, our
pediatric urologists proceeded to repair his hypospadias and bifid
scrotum. Four weeks following this surgery, he underwent closure of his
colostomy. He has since recovered without incident and has returned home
with strict guidelines for follow-up of his urologic system and his
This case demonstrates the benefit of multidisciplinary evaluation that
all patients receive at the Peña Colorectal Center at Cincinnati
Children’s. All charts are reviewed and the trigger list helps ensure
that urologic conditions are not missed.
The patient described had a unique set of conditions that were addressed
efficiently in one evaluation. We have learned that a thorough
evaluation and treatment plan before surgery helps to minimize
anesthetics, improve family understanding and expectations, and may
result in better outcomes. A collaborative, multidisciplinary model
helps achieve these goals.
of upload: 14th July 2014