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State-of-the-Art Pediatrics

July 2019

Improving Treatment for Children with Complex Anorectal Malformations 

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Author: Rebecca Rentea, MD | Director, Comprehensive Colorectal Center | Colorectal and Pelvic Reconstruction Surgery | Assistant Professor of Pediatric Surgery, UMKC School of Medicine        
    
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Column editor: Amita R. Amonker, MD | Pediatric Hospitalist | Assistant Professor of Pediatrics, UMKC School of Medicine 
 
When a malformation with long-term functional outcome management needs intersects with new technologic advances, more children can be helped. Anorectal malformations comprise a wide spectrum of diseases affecting boys and girls, and involving the distal anus and rectum, as well as the urinary and genital tracts. They occur in approximately 1 in 5,000 live births.1
 
While some defects can be minor and easily treated, some are very complex, difficult to manage, and are associated with other anomalies. These complex defects may have a poor functional prognosis, requiring a team approach to diagnosis and treatment during childhood and adolescence.  

At Children’s Mercy Kansas City, a comprehensive team of specialists whose focus is on meeting these patients’ needs long term is critical to improved outcomes. Pediatric specialties involved include urology, gastroenterology, gynecology, neurosurgery, surgery and psychology.

Instead of multiple appointments with different doctors who each have a separate plan of care, this team meets together to discuss each patient’s unique needs and create one, unified approach to treatment. This often translates into a reduced number of procedures and surgeries, as well as fewer office visits. The collaborative approach overall works to improve the child’s and their family’s quality of life.

Complex anorectal malformations treated in this setting typically include:

• Imperforate anus
• Hirschsprung's disease
• Cloaca
• Pelvic floor disorders

Visualizing Repairs

Even with an excellent surgical repair early in life, bowel control, urinary control and sexual function are all major concerns for these patients, their families and their medical team. Often, these patients require surgical reconstructions and specific recommendations regarding their anatomy as they grow and reach developmental milestones.

Visualizing the viability of tissue and making intra-operative decisions is of paramount importance to good healing and good long-term functional outcomes. Recently, I shared this experience with the American Pediatric Surgical Association:

• Complex pediatric colorectal reconstructions involve visual assessment of tissue perfusion. Traditionally, tissue perfusion has been measured via active bleeding and lack of discoloration.
• Indocyanaine Green fluorescence angiography, or the ICG-FA, can utilize fluorescent dye to assess tissue perfusion in real time. This technology has been utilized in the adult population for many years, but it has not been employed in the pediatric population until recently.
• ICG-FA is safe, is easily metabolized and requires no radiation exposure.

Children’s Mercy has implemented intraoperative use of ICG-FA in highly select cases, and is finding it to be more accurate at tissue perfusion assessment than the surgeon’s eye, ultimately changing intra-operative real-time decisions in complex pediatric colorectal surgery cases. We are one of only a select few children’s hospitals in the nation utilizing this technology for our patients.

Utilizing this technology, along with the skills of the Comprehensive Colorectal Center team, may improve lifelong outcomes for children with anorectal malformations. Indeed, we often see patients who benefit from long-term monitoring and assessment of these conditions as they grow and develop. The management of these children postoperatively is not “one and done.”

Our team also has developed educational materials on anorectal malformations and various other management options which are now available. We invite you to share these resources with your patients and their families: https://www.childrensmercy.org/colorectal/

References  

1. Anorectal Malformations. Levitt MA, Peña A. Advanced Orphanet J Rare Dis. 2012 7:98. https://doi.org/10.1186/1750-1172-2-33. 

2. Impact on Patient Care of a Multidisciplinary Center Specializing in Colorectal and Pelvic Reconstruction. Vilanova-Sánchez A, Rentea RM, Levitt MA. Front Surg. 2018 Nov 19;5:68.       

3. Diagnosis and Management of a Remnant of the Original Fistula (ROOF) in Males Following Surgery for Anorectal Malformations. Rentea RM, Halleran DR, Wood RJ. J Pediatr Surg. 2019 Feb 28. pii: S0022-3468(19)30116-2.