Tag Archive for: colorectal surgery

Everyone Poops Book Cover

2022: Pediatric colorectal and pelvic reconstructive surgery today

Everyone Poops book coverAdapted from Levitt MA. New and exciting advances in pediatric colorectal and pelvic reconstructive surgery – 2021 update. Semin Pediatr Surg. 2020 Dec;29(6):150992.

As demonstrated in the popular children’s book by Taro Gomi, Everyone Poops, the physiology of stooling is a focus of early childhood development and a subject of concern for both parents and children. References to pediatric colorectal problems go back many thousands of years. In fact, the Babylonian Talmud, written in the year 200 CE, recommends that “an infant whose anus is not visible should be rubbed with oil and stood in the sun… and where it shows transparent the area should be torn crosswise with a barley grain.” Surgical techniques to manage such patients have certainly evolved since that time, but the basic principles of care remain the same.

How we got here

The modern story of the care of patients with anorectal malformations (ARMs) began in the 1940s in Melbourne, Australia, when Henry Douglas Stephens worked to define the anatomy of children with ARMs by analyzing the anatomy of twelve deceased patients with these conditions. He continued to focus on this specialty for the rest of his career and published two books on the topic in 1963 and 1971. Prior to his groundbreaking work, the anatomy of such patients was only a concept that existed in surgeons’ minds – without anatomic precision – since no one had actually seen the anatomy. These concepts were depicted in the bible of pediatric surgery in North America, the Gross textbook, which in retrospect was both oversimplified and inaccurate.

During his autopsy dissections, Stephens came to the key anatomic conclusion that the puborectalis muscle (the sphincters) lay behind the urethra. He devised an operation based on this concept: first, the urethra was identified, then a space between it and the puborectalis was dissected, and then the rectosigmoid was pulled through that space. A small incision in the perineum for the pulled-through bowel was made, within which the new anus was created. The perineal dissection was a blind maneuver. During the same time period, William Kiesewetter in Pittsburgh proposed his version of the sacral abdominoperineal pull-through using similar anatomic principles.

Justin Kelly was one of Stephens’ trainees in Australia who learned how to do this operation. At Boston Children’s Hospital in the late 1960’s, he taught what he had learned from Stephens to the surgeons there, including another trainee, Alberto Pena. Pena and his fellow surgical residents benefitted from exposure to Kelly as well as master surgeon Hardy Hendren, who operated on patients across town at Massachusetts General Hospital. Hendren, the pioneer in the care of children with cloacal anomalies, passed away this year at the age of 96.

Pena completed his training in Boston and went to Mexico City in 1972 at the age of 34 to become the head of surgery at the National Pediatric Institute. He tells the story that when he asked his new pediatric surgery faculty to choose an area of specialization, no one chose colorectal, so he decided to take on that group of patients and thus embarked on his revolutionary colorectal career. Pena at first applied the technique he had learned from Kelly to repair anorectal malformations, but he became increasingly frustrated by the procedure. He felt that the maneuvers offered very poor exposure to the anatomy, and over time his incision grew longer and longer. In 1980, Pena’s collaboration with Pieter de Vries – who had come to Mexico City to work on these cases with Pena – culminated in the first posterior sagittal anorectoplasty [Figure 1]. That same year, Pena presented his findings at a meeting of the Pacific Association of Pediatric Surgeons.

imperforate anus repair

Figure 1: Original diagrams of the posterior sagittal approach to repair imperforate anus

This posterior sagittal approach opened a beneficial Pandora’s Box in surgery. It allowed for a true understanding of the pelvic anatomy and led to the care of many conditions that were previously – to use Pena’s words – “too difficult to reach from above (via laparotomy) and too difficult to reach from below (perineally).” This new approach influenced the repair of cloacal malformations, urogenital sinus, pelvic tumors, urethral problems, reoperations for imperforate anus and Hirschsprung disease (HD), a transpubic approach for complex genitourinary problems, and a comprehensive strategy for the management of cloacal exstrophy. In addition to his surgical contributions, Pena also conceived of the intervention that has arguably improved patients’ quality of life the most: a focused approach to the bowel management of fecal incontinence. Thanks to such bowel management programs, now available at many centers across the world, thousands of children are no longer wearing diapers and have said goodbye to their stomas. The impact of bowel management is perhaps comparable to that of intermittent catheterization for patients with urinary incontinence.

My personal journey in this field began in 1992, when I was an eager medical student and signed up for an elective in pediatric surgery with Alberto Pena. This experience changed my career trajectory in a very dramatic and positive way. Medicine was becoming increasingly complex, and fields such as neonatal care, transplantation, and cardiology were benefitting from a collaborative approach. It became clear that the colorectal field needed the same approach. As a comparative example, consider the project of building a bridge. How does such a project start? The cement layers do not show up one day and lay cement prior to the steel team deciding where to place the beams. The project must begin with all parties meeting together to develop a comprehensive plan. Amazingly, however, that type of collaborative planning does not often happen in the care of medically complex patients. It most assuredly needs to.

The value of multi-disciplinary care

Medical complexity requires integrated and collaborative care because all the anatomic structures that need to be managed are located right next to each other and because each has a unique and complex physiology that can affect the other systems. To achieve success, patients with anorectal malformations, Hirschsprung disease, fecal incontinence (related to a variety of conditions), and colonic motility disorders require care from a variety of specialists throughout their lives. These include providers in the fields of colorectal surgery, urology, gynecology, gastroenterology, motility, orthopedics, neurosurgery, anesthesia, pathology, radiology, psychology, social work, nutrition, and many others. Perhaps most important to the achievement of a good functional result, however, is a patient’s connection to superb nursing care. A complex colorectal operation takes about four hours to perform, but to get a good result, it takes an additional 96 hours of work, the vast majority of which involves nursing care. The value of good nursing partners to ensure successful surgery cannot be overemphasized. They have unique skills in identifying and solving problems, a willingness to get down in the weeds, and are always striving to fill the gaps in care.

What parents want and need

Having met many parents with newborns diagnosed with colorectal problems, I have made several observations. First, it seems that no parent has ever imagined that their child could have a problem with stooling – this is a physiologic ability that is taken for granted. When they are told about the problem with their baby, they are uniformly shocked that something like this could happen. Second, when discussing that their child will need surgery to correct their colorectal anatomy, parents don’t focus on the surgical technique and elegance of the reconstruction, as surgeons tend to do. Instead, parents dwell on whether the surgery will create a working reconstructed anatomy that will allow their child to stool without difficulty or embarrassing accidents. As surgeons we need to remember this. We always need to understand what it is that the family and patient wishes us to deliver, and we need to strive to achieve those goals. As proud of our surgical skills as we are, it is the functional outcome that matters most.

Where we are in 2022

In 2022, the advances in the field of pediatric colorectal and pelvic reconstruction are significant. They include new techniques and ideas that over time have made a dramatic and positive impact on the care and quality of life of children who suffer from colorectal problems. Here are a few such advances:

  1. Prenatal diagnosis of anorectal and cloacal malformations has been progressively improving. Perinatologists have learned to look for specific findings, such as a pelvic mass in a female with a single kidney, and consider that it could be a cloaca. Assessment of perineal anatomy, pubic bone integrity, sacral development, abnormalities of the radius bone, as well as cardiac, spinal, and renal anomalies may lead the clinician to consider that a fetus may have an anorectal malformation.
  2. Management of the newborn, particularly in the fields of newborn radiology and neonatal care, has dramatically improved as neonatal techniques have advanced. Specific to the colorectal patient have been advances in radiology such as assessments of hydronephrosis, 3D reconstruction of cloacal anomalies, and ultrasound-guided distal colostography. Further advances include improved techniques in the management of hydrocolpos and stoma care, to name a few.
  3. The treatment of associated urologic anomalies has diminished chronic renal disease, and proactive bladder management is reducing the need for bladder augmentations and renal transplantation.
  4. Understanding the gynecologic collaboration has helped clinicians define the Mullerian anatomy and better plan for menstruation and future obstetric potential.
  5. Prediction of continence, even in the newborn period, requires an understanding of the associated problems with the sacrum and spine. This knowledge has allowed clinicians to have more robust conversations with families about their child’s future.
  6. The decision of whether to do a newborn repair versus a colostomy must be guided by the surgeon’s experience and the clinical circumstances in which they find themselves.
  7. The defining of anatomy allows patients to be compared across medical centers, and for treatment options and outcomes to be uniformly analyzed. Keeping track of one’s outcomes and always striving to improve should be basic tenets of surgical practice.
  8. Recognizing the value of laparoscopy and knowing for which cases this approach should be applied. Morbidities associated with a laparoscopic approach for a rectum in an ARM patient well below the peritoneal reflection have been noted. In HD cases, laparoscopy can limit the stretching of the sphincters which occurs during the transanal rectal dissection.
  9. Development of a treatment algorithm for the management of cloacal malformations which considers the importance of their common channel and urethral lengths.
  10. Recognizing key complications after ARM and Hirschsprung surgery, knowing when and how to do a reoperation, determining the outcomes of such reoperations, and ultimately figuring out how to avoid complications altogether.
  11. Understanding the causes of fecal incontinence, the amount of incontinence that can be anticipated, and the surgical contributors to achieving continence.
  12. Development of bowel management programs in multiple centers and committing to following these patients in the long term.
  13. Learning the pathophysiology of motility disorders and developing treatment protocols, as a result of the vital collaboration between surgery and GI/motility. Medical treatments with laxatives, rectal enemas, botox injection of the anal canal, and surgical adjuncts such as antegrade colonic flush options and sometimes colon resections are key aspects of the treatment armamentarium.
  14. Developing a collaboration between colorectal surgery and urology allows the clinical team to know when the colon can be used for a bladder augment (which not only offers an augment option but also can improve antegrade flushes of the colon) or if the appendix can be shared between Malone and Mitrofanoff. The collaboration with urology to plan the management of both urinary and fecal continence during the same operation is a very valuable trend. This proactive planning has improved the lives of many patients and has reduced the numbers of operations they need as well as their hospital stays.
  15. Sacral Nerve Stimulation (SNS) has shown promise in the management of urinary incontinence and seems to have a role in improving fecal continence and promoting motility, as an adjunct to treatments for constipation.
  16. Development of dedicated colorectal centers that are integrated and collaborative allows a team to tabulate their results and strive for better outcomes. The value of a collaborative model for the care of such patients cannot be overstated, not only for patient convenience, but also for creating an integrated plan for their care. These exist now in many parts of the country and care is available in nearly every region, reducing a family’s need to travel great distances away from their home to access care.
  17. Establishing a transition to adult programs, as with congenital heart disease and cystic fibrosis. Colorectal surgeons are obligated to develop a transition plan for their patients as they enter adulthood.
  18. Using Basic Science to advance the field, including tissue engineering and genetics, will be revolutionary. We should be able to imagine the day when cloacal reconstruction could be based on a previously tissue-engineered segment of vagina, produced by the patient’s own stem cells. In addition, the genetics of anorectal malformations as well as Hirschsprung disease are being vigorously pursued which will impact parental counseling and potential therapies.
  19. Real time data used to follow outcomes is needed to keep track of complications as well. That information can be used to adjust protocols which will improve results.
  20. Development of international consortiums will help patients in a way that is not achievable by a single institution. Consortiums allow ideas to be spread rapidly which will dramatically affect how many patients can be helped and how quickly. The Pediatric Colorectal and Pelvic Learning Consortium, pcplc.org, is well on its way to achieving these goals.
  21. Bringing complex care to all corners of the world because there is a great deficiency in advanced colorectal care in the developing world. The care of colorectal patients in a resource-limited setting has unique challenges, but creative solutions by innovative surgeons in those areas have a led to dramatic improvements in care.
  22. Parent/patient organizations provide education, advocacy, and support for families at all stages of their child’s care. With internet access readily available, colorectal patients and their families can now access the welcoming environment of these organizations, and no longer feel as lost and alone as in previous years.

Why it matters

Given all these wonderful advances, we must continue to reaffirm the key principles stated by Sir Dennis Browne that “the aim of pediatric surgery is to set a standard, not to seek a monopoly.” With an integrated approach to the care of this complex group of patients, great things can be achieved. I am hopeful that the caregivers and parent/patient group organizations who commit to the care of children with colorectal problems and understand the daily struggle of improving a patient’s quality of life will learn the skills and tricks necessary to achieve good results. If they do, they will help many children.

Finally, I will share a humorous piece written by my daughter, Jess Levitt, regarding the value of bringing order to chaos. Its message is particularly relevant to the care of children with colorectal problems in 2022, as we build on our efforts to improve, streamline, and transform the formerly chaotic process through collaboration and education. As we endeavor to advance this field, we need to remember what Dr. Pena often said: “It is not the unanswered questions, but rather the unquestioned answers that one must pursue.”

“A” must come before “B,” which must come before “C,” everybody knows that. But what if the Millercamps of this world did not have to sit next to the Millerchips when it comes to seating arrangements? Can Pat Zawatsky be called before Jack Aaronson when the teacher is taking attendance? Do those 26 letters that make up all the dialogue, signs, thoughts, books, and titles in the English-speaking department of the world need their specific spots in line? Everyone can sing the well-known jingle from A to Z, but not many people can tell you why the alphabet is the way it is.

For almost as long as humans have had the English language, they have had the alphabet. The good ol’ ABCs. However, the alphabet represents the human need for order and stability. I believe that the same thinking that went into the construct of time and even government went into the alphabet. Justifiably, lack of order leads to chaos. Knife-throwing, gun-shooting chaos, in the case of lack of governmental order. Listen to me when I tell you that there is absolutely no reason that the alphabet is arranged the way that it is. Moreover, the alphabet is simply a product of human nature and how it leads people to establish order for things that do not require it.

Now I know this sounds crazy, but bear with me. Only if you really peel away the layers of the alphabet will you find the true weight it carries. People organized the letters of our speech into a specific order simply because there wasn’t already one. Questioning this order will enlighten you on the true meaning of it. Really dig deep into the meaning behind the social construct that is the alphabet. Short and sweet as it may be, the order of the ABCs is much less than meets the eye. There is no reason that “J” should fall before “K”! Understand this. Very important as order is, it is only a result of human nature.

What’s next? X-rays become independent of Xylophones in children’s books of ABCs?

You know what the best part is? Zero chance you even noticed that each sentence in this essay is in alphabetical order.

– Jess Levitt

 

Marc Levitt plays with a patient

Reoperation of anorectal malformation repair restores continence, improves quality of life

Marc Levitt plays with a patient

Dr. Levitt has performed over 10,000 surgeries to address the wide spectrum of problems involving the colon and rectum — more than any other full time practicing pediatric surgeon in the world.

Patients with a previously repaired anorectal malformation (ARM) can suffer from complications which lead to incontinence. Reoperation can improve the anatomic result, but its impact on functional outcomes has previously been unclear.

Marc Levitt, M.D., chief of Colorectal and Pelvic Reconstructive Surgery at Children’s National, and Richard Wood, M.D., chief of Colorectal and Pelvic Reconstruction at Nationwide Children’s Hospital, co-led the study when they worked together in Columbus. They performed a retrospective cohort study, from 2014 to 2019, of patients with a previously repaired ARM who underwent another posterior sagittal anorectoplasty (PSARP) procedure, essentially redoing their first procedure. When results from the initial assessment were compared to 12 months after the redo surgery, they found that patients with fecal incontinence after an ARM repair can, with a reoperation, have their anatomy corrected, restoring continence for many and also improving their quality of life.

The study, published in the Journal of Pediatric Surgery, found that at one-year post-redo operation, 50 percent of the patients were on laxatives only, and 75 percent of those patients were completely continent. Overall, 77 percent of the patients were clean (1 or fewer accident per week) after their redo surgery and complication rates were low. Strictures were the most common complication seen after reoperations, as no dilations were performed, but were easily managed with a minor procedure. Surprisingly, 20 percent of patients with expected poor continence potential became fully continent on a laxative-based regimen after redo surgery. Traditionally, many of these children would not even be offered a redo surgery, given their perceived poor potential for bowel control.

The Division of Colorectal & Pelvic Reconstructive Surgery at Children’s National is the first in the mid-Atlantic region to fully integrate surgery, urology, gynecology and gastroenterology into one cohesive program for children. Dr. Levitt is a world-renowned surgeon who has performed over 10,000 surgeries to address the wide spectrum of problems involving the colon and rectum — more than any other full time practicing pediatric surgeon in the world.

This study shows that redo surgeries are a safe and effective option for patients with fecal incontinence after an anorectal malformation repair. The authors hope that the findings will lead to the ability to help more patients who suffer from complications and/or incontinence after a prior repaired ARM and who can benefit from an improvement in their colorectal anatomy.  After a reoperation, patients can expect to have improved quality of life because the outcome gives them more freedom and less worry about soiling accidents.

To access the full article published in the Journal of Pediatric Surgery click here.

Marc Levitt plays with a patient

Evidence to eliminate burdensome postop practice after imperforate anus repair

Marc Levitt plays with a patient

The study was co-led by Marc Levitt, M.D., who launched the division of Pediatric Colorectal and Pelvic Reconstructive Surgery at Children’s National Hospital in late 2019.

A prospective randomized controlled trial has given pediatric colorectal specialists the first evidence to reconsider a standard postoperative care practice: Routine anal dilations following a primary posterior sagittal anorectoplasty (PSARP), an operation to reconstruct a child born with imperforate anus. This treatment has been the standard of care following PSARP for more than thirty years and was believed to help prevent strictures after surgery for anorectal malformations (imperforate anus). However, it requires parents and caregivers to perform this uncomfortable procedure on their child daily, which can have a significant psychological impact on the child. Prior to this trial, a quality of life assessment found that postoperative dilations were the most stressful part of these patients’ care for both patient and parents.

“The PSARP procedure, performed for the first time in 1980, improves the lives of children born with imperforate anus by providing a safe and effective reconstruction technique,” says Marc Levitt, M.D., who led the study with co-author Richard Wood, M.D., of Nationwide Children’s Hospital, before joining Children’s National Hospital as chief of the division of Colorectal and Pelvic Reconstructive Surgery. “We are thrilled to have evidence that one of the top postoperative challenges for parents – a twice daily anal dilation for several months after the surgery is completed – can potentially be eliminated for most kids with no impact on their recovery.”

“We also found that if a stricture, or scar, develops, which occurs in only about 10 percent of cases, it can easily be managed with a minor operative procedure done at the same time as colostomy closure, which in most cases they already need. So, if a family had to choose between daily dilations for months or a one in 10 risk of needing a minor surgical procedure, they can now make that choice and avoid routine dilations.”

The prospective single institution randomized controlled trial was conducted between 2017 and 2019 and included 49 patients. The abstract of the results was accepted for presentation at the British Association of Paediatric Surgeons Annual International Congress, 2020, and its manuscript is to be published.

“The clinical benefit of routine dilation had never been studied in a formal way, it had been accepted as surgical dogma. Our cohort, who underwent a randomized controlled trial, gave us the ability to look at this practice in an evidence-based way,” Dr. Levitt says. “Revising this practice could be a real game-changer for parents and kids with anorectal malformations.”

ARM index webinar

Colorectal team presents virtual conference sessions

The 6th Annual Alex Pediatric Surgery Congress and 1st Nile of Hope Hospital Congress conference, in cooperation with Colorectal Team Overseas (CTO), provides updates in colorectal, urogenital disorders and pelvic reconstructions in pediatrics. The Children’s National Hospital colorectal team was due to present at the conference in Alexandria, Egypt, in April 2020, but due to the global COVID-19 pandemic the event was indefinitely postponed. Despite this, Marc Levitt, M.D., Colorectal and Pelvic Reconstructive Surgeon at Children’s National, and Founder and Head of the CTO, presented educational sessions virtually to Egyptian surgeons throughout the month of April.

Anorectal malformation case presentation

Surgeons assemble in a virtual Zoom session for a case presentation on anorectal malformations by Marc Levitt, M.D., and the Children’s National colorectal team.

The video conferences allowed surgeons and experts to come together and foster the global collaboration that benefits colorectal teams and patients worldwide. The first session included 70 pediatric surgeons from Egypt and grew to over 128 attendees in the last session. The presentations spanned a variety of topics and can be accessed at the links below:

Joining Dr. Levitt in the discussion were members of the Children’s National colorectal nursing team, including Julie Choueiki, Program Manager, Justine Garofalo, CPNP, Meghan Mesa, Tara Garbarino, CPNP, and Katherine Worst, CPNP-AC. The integrated Children’s National colorectal team elevates the significance of the nursing role in caring for complex patients. For example, cases in the Bowel Management Program require hours of ongoing nursing care. The team demonstrated the partnership that benefits children when surgeons include and value nursing presence in the care of colorectal patients.

Moving forward, the team will bring continued virtual, telehealth collaboration and education. Doing so will expand the potential for more colorectal patients to receive the care they need.

Colorectal Textbook cover

Pediatric Colorectal and Pelvic Reconstructive Surgery textbook now available

Colorectal Textbook cover

The cover of the new Pediatric Colorectal and Pelvic Reconstructive Surgery textbook, edited by Marc A. Levitt, M.D., and Alejandra Vilanova-Sánchez, M.D.

The first edition of the Pediatric Colorectal and Pelvic Reconstructive Surgery textbook, edited by Marc A. Levitt, M.D., and Alejandra Vilanova-Sánchez, M.D., is now available.

The textbook provides comprehensive coverage of the anatomical and physiological aspects of complex colorectal and pelvic malformations presented in a practical and clinically focused way. Some of the topics explored include surgical protocols, the benefits of high-level collaboration between surgical services when treating these anomalies, treatment algorithms and care of complications.

The book also includes content on:

  • Evaluation and management of the newborn
  • Surgical interventions of the newborn, and when a primary repair versus a staged approach is required
  • The value of laparoscopy and when to use it
  • The importance of a transition program to adulthood

The Pediatric Colorectal and Pelvic Reconstructive Surgery, 1st edition textbook can be purchased here, and will benefit colorectal teams worldwide.

About the Editors

Marc Levitt

Marc Levitt, M.D., leads the colorectal program at Children’s National Hospital and is editor of the new Pediatric Colorectal and Pelvic Reconstructive Surgery textbook.

Marc Levitt, M.D., currently leads the colorectal program at Children’s National Hospital, the first in the mid-Atlantic region to fully integrate surgery, urology, gynecology and gastroenterology into one cohesive program for children. He has been the driving force around the world in enhancing the care of children with colorectal and pelvic reconstructive needs through the development of specialized, integrated and collaborative surgical centers. He is internationally recognized as specializing in conditions affecting the newborn, pediatric and adolescent population affected with anorectal malformations (imperforate anus), cloacal malformations, Hirschsprung disease, as well as a variety of conditions leading to fecal incontinence, such as spinal conditions and functional constipation. Dr. Levitt has written three textbooks, and has authored over 200 scientific articles on these subjects.

Dr. Levitt is the founder of the Colorectal Team Overseas (CTO), which is a group of international providers that travel to the developing world to provide care and teaching for patients with colorectal needs. He co-founded the creation of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC), which is an organization of collaborating colorectal centers across the globe.

Alejandra Vilanova-Sánchez, M.D., is a pediatric surgeon in the urogenital and colorectal unit at the University Hospital La Paz, Madrid. After finishing her training, she completed a fellowship in Pelvic Reconstruction Surgery at the Center for Colorectal and Pelvic Reconstruction at Nationwide Children’s Hospital. Her focus was on complex colorectal and pelvic surgery involving the gynecological and urological systems. Dr. Vilanova-Sánchez is a member of the Spanish Association of Pediatric Surgeons, European Pediatric Surgical Association (EUPSA) and ARM-net. She is a frequent speaker in international meetings and she has organized several national and international meetings on the topic of pediatric colorectal care. She participates annually in surgical brigades collaborating with nonprofit organizations, Colorectal Team Overseas and Helping Hands for Anorectal Malformations International, where she helps patients with colorectal conditions around the world.

child writing question marks on chalkboard

Test your knowledge of pediatric colorectal and pelvic reconstructive surgery!