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Learn more about this complex colorectal disorder that affects approximately 1 in 5,000 live births from USA pediatric surgeon, Jeremy Fisher, MD. 

Hirschsprung Disease is a complex colorectal disorder affecting approximately 1 in 5,000 live births. It’s a defect that is typically identified in the early days of a child’s life, if they’re unable to pass meconium in the first 24 to 48 hours after birth. Meconium is a newborn’s first poop that is made of protein, fats, and intestinal secretions like bile. Children who do not pass stool as expected may be suspected of having Hirschsprung Disease. 

“Simply put, Hirschsprung Disease can cause difficulty passing stool. Children affected by this condition often develop constipation or partial or total obstruction of the bowels, leading to discomfort and pain,” says Jeremy Fisher, MD, pediatric surgeon with University Surgical Associates. “The condition involves missing nerve cells in the muscle in part of all of the large intestine. These cells are called ganglion cells, and their job is to allow the rectum to relax. Without them, the muscles in the bowel are constantly contracting and lose their ability to move stool through the intestine and out of the body.”  

Suspicion of Hirschsprung disease is first explored with a contrast enema, which is an x-ray study using a water-soluble contrast agent to capture images of the colon and rectum. If an abnormality or suggestion of disease is found, a biopsy of the lining of the rectum is performed to confirm the diagnosis.  

“Hirschsprung Disease exists on a spectrum – some have a small area affected. The lower rectum is involved for everyone with the condition, and it can move as high as the colon and small intestine,” Dr. Fisher says. 

Who is Affected? 

There are no racial or ethnic backgrounds that make developing Hirschsprung Disease more likely. Anyone can be born with this birth defect, although it is four times more common in males than females. While typically diagnosed at birth, the condition is sometimes found later – where a small area of the rectum is involved and becomes more noticeable as the child gets older and experiences issues with constipation. Because constipation is a such a common childhood ailment, it can take a while to identify the root cause of symptoms.  

In addition to the main symptoms of constipation and bowel obstruction, children who are diagnosed later are also at risk for Hirschsprung enterocolitis, an inflammatory condition and infection of the colon that causes fever, abdominal distention, diarrhea, and sepsis.   


“The only treatment for Hirschsprung Disease is surgery, where the section of the rectum or colon that isn’t functioning properly is removed. When found at the newborn stage, many children will have this procedure before they leave the hospital,” says Dr. Fisher. “The number one goal is to remove the affected area and put things back together so the child can poop normally. Although a temporary stoma is sometimes necessary, the vast majority are ultimately able to go to the bathroom through the anus.” 

Typical recovery for this surgery is a few days in the hospital, usually less than a week depending on other factors. Before discharge home, a child’s bowels must be functioning as expected. Parents are also educated about how to look out for enterocolitis or infection that can develop in the digestive tract after surgery. While in the past surgeons have believed that surgical repair corrected Hirschsprung Disease completely, it’s now understood that many children with the condition may have ongoing issues with bowel function including incontinence and constipation. 

Dr. Fisher notes that Hirschsprung Disease is complex and multifaceted and can negatively impact a person’s quality of life. Because these issues often continue into adolescence and adulthood, routine monitoring or being followed by a specialist is critical to ensure they don’t develop or continue to struggle with the physical and psychosocial issues related to the disease.   

“Pediatric surgeons are trained to treat this disease and it is an essential pediatric surgical condition. However I do believe that more experience and a greater volume of cases means a higher level of expertise – leading to better outcomes,” says Dr. Fisher. “The experience I gained during my fellowship and training under one of the leading experts in the disease means I’m well equipped not only to perform the initial surgery if needed, but to provide a nuanced assessment and advanced care for individuals who are still struggling with long term symptoms.” 

If you or your child is struggling with lingering symptoms of Hirschsprung Disease, compassionate and effective care is available. To schedule a consultation with Dr. Fisher, call University Surgical Associates at 423.267.0466.  

Discover the benefits of pectus excavatum surgery from Lisa Smith, MD, pediatric surgeon with University Surgical Associates.

Whether or not pectus excavatum surgery is the right choice is a question that every individual or family has to make when it comes to correcting chest wall deformities. The decision to have surgery to correct pectus excavatum or carinatum is difficult one, and there’s no doubt that treatment is complex. Yet, according to Lisa Smith, MD, pediatric surgeon with University Surgical Associates, the benefits of surgery often outweigh the rigors of recovery. In addition to the corrected appearance of the chest wall, these procedures also help children and adults live a more active life.

“I believe there’s a large group of young adults who have been told or are being counseled that if they’ve made it into adulthood without having surgery to correct their pectus excavatum that they probably don’t need surgery,” says Dr. Smith. “I don’t believe that’s true, and in fact, many people don’t even realize what an impact their deformity is having on their exercise capacity and their ability to adequately perform routine activities.”

Pectus excavatum is a congenital chest wall deformity where the chest cartilage grows abnormally, producing a caved-in or concave appearance in the anterior chest wall. It’s the most common chest wall deformity in children and represents about 90 percent of cases. Pectus carinatum is thought to occur by the same mechanism, but the chest bows out. Pectus excavatum occurs in an estimated 1 in 300-400 births, and males are more likely to have the condition.

a year unlike any other

According to Dr. Smith, many more adults reached out to her for consultation about pectus excavatum and carinatum correction during 2020 than typically do during a normal year. The change of routine brought on by theCOVID-19 pandemic provided an opportunity for many to seek treatment that might otherwise be too busy to take time for recovery. And although things looked a little differently than in years past, Pectacular!, University Surgical Associates’ chest wall reconstruction program, continued to make improvements in the methods used to treat these condition and to refine the recovery process.  

One such method was to change pain management protocols, moving away from a catheter-based system that had to be constantly maintained to a long-acting anesthetic injection. What started as a means to provide effective and safe care to reduce possible coronavirus exposures led to the discovery of a combination of pharmaceutical medications that offered even better pain control.

Dr. Smith notes that her passion for the Pectacular! program and the people who go through the process continues to grow as she witnesses the life-changing results for those who thought they had no good options for treatment.

“Until you change the mechanics of the chest wall and how it moves, people with these deformities will continue to have complications of the heart working harder than it should to keep up. My patients are blown away at the difference in how they feel after surgery,” says Dr. Smith. “Even though it’s a difficult surgery and long recovery, there’s a great payoff at the end –allowing individuals the opportunity to breathe and perform physically like they’ve never done before.”

If you’re considering chest wall reconstruction for yourself or your child, we understand that you have questions – and we want to help in any way possible. 

For more information visit our Pectacular! page or call (423) 267-0466 to schedule a consultation. 


A: Diverticula are pockets that develop in the colon wall, usually in the sigmoid or left colon,but can involve the entire colon. Diverticulosis describes the presence of these pockets. Diverticulitis describes inflammation of these pockets. The major symptoms of diverticular disease are abdominal pain (usually in the lower left abdomen), diarrhea, cramps, change of bowel habits and, occasionally,rectal bleeding.

It is believed that a low-fiber diet over the years creates increased colon pressure and results in pockets or diverticula. Diverticular disease is usually treated by diet and occasionally medications to help control pain, cramps and changes in bowel habits.Increasing the amount of dietary fiber (grains, legumes, vegetables) reduces complications. 

Diverticulitis requires more intense management. Mild cases may be managed without hospitalization, but this is a decision made by your physician. Treatment usually consists of oral antibiotics, dietary restrictions and possibly stool softeners. Severe cases require hospitalization with intravenous antibiotics and strict dietary restraints. Most acute attacks can be relieved with such methods. Surgery is reserved for recurrent episodes, complications or severe attacks.

 

USA Colorectal Surgeon Dr. Shauna Lorenzo-Rivero,M.D.


Find more information about Dr. Lorenzo and USA Colorectal surgery here

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