Paediatrics



Cincinnati Children’s Hospital Medical Center Case Study: Anorectal Malformation
 

This 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).

Clinical history

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.

Our approach

On initial assessment of the patient’s chart, it was apparent that he required a combined approach to manage his anorectal malformation and urogenital malformation.

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.

Surgical course

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 bowel management.

Summary

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.

 Date of upload: 14th July 2014

 

                                  
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