What Is Known/What Is New
What Is Known
- Rectal prolapse is often seen in children less than 4 years of age.
- Constipation is a common cause of rectal prolapse.
What Is New
- Due to newborn screening, cystic fibrosis is no longer a common cause of rectal prolapse.
- Signs of straining rather than stool consistency seems to be the largest contributor to rectal prolapse in children with constipation, suggesting that a strict toilet regimen maybe most beneficial for medical management.
- Patients who fail surgical management are most often older in age.
Rectal prolapse is a protrusion of rectal mucosa through the anal sphincter. The last data analysis and review of rectal prolapse and its risk factors, clinical characteristics, and management of rectal prolapse in children was published in 1988 (1,2). Since that time, there have been many medical advances and many published reports describing patients with medical conditions presenting with rectal prolapse that had previously not been described (3–7). Accordingly, the aim of the study was to perform a retrospective descriptive analysis of children diagnosed with rectal prolapse.
After approval from the Wayne State University Institutional Review Board, a retrospective chart review was conducted on children less than 18 years of age who were diagnosed with rectal prolapse from January 1999 to May 2014 at Children's Hospital of Michigan. Records were obtained from multiple sources: hospital admissions, emergency room, pediatric gastroenterology clinic, and pediatric surgery clinic. Information collected included patients’ demographics, history of present illness, anthropometrics, and management.
We found 158 patients who were diagnosed with rectal prolapse. The mean age at diagnosis was 3 years, 11 months (range = 2.5 weeks to 17 years). 93 (59%) were boys. Seventy-one (45%) were Caucasian, 36 (23%) African American, 10 (6%) Middle Eastern, 7 (4%) Hispanic, and 4 (3%) Asian.
Eight percent were diagnosed with failure to thrive. Constipation was the leading diagnosis associated with rectal prolapse (87 patients; 55%). The second most common was idiopathic (27 patients, 17%), as no underlying cause was confirmed. Only 4 patients (3%) were diagnosed with cystic fibrosis. For more details regarding diagnoses, see Table 1.
The average duration of prolapse was 1 year. The duration was slightly longer in patients older than 4 years of age, averaging to 1 year and 2 months. Patients diagnosed as idiopathic and were older than 4 years of age had the longest duration of symptoms calculating to 2 years and 7 months. Of these patients, 5 of the 9 had an underlying behavioral disorder.
In patients diagnosed with constipation, 52% (17 out of 33 patients) had hard consistency stools; the rest either described their stool as soft or mixed. The most common complaint in patients diagnosed with constipation was straining (66%). The workup for patients with constipation included sweat chloride testing (40% of patients), barium enema (18%), celiac screening (22%), thyroid hormones (11%), and colonoscopy (9%). More than two-thirds of patients who underwent this workup were seen in gastrointestinal (GI) clinic. Seven patients (28%) categorized as idiopathic had behavioral disorder. Of these, 1 patient was confirmed to be sexually abused, with concern of abuse in 3 additional patients. Solitary rectal ulcer syndrome was found in 2 patients while performing a lower endoscopy.
Documentation for manual reduction of prolapse was reported in 59 patients. Please see Figure 1 for details. Surgical referral was made to assist in reduction for 14 patients, which mostly required sedation and manual reduction. Two patients required reduction with sugar application.
Seventy-four patients followed up in either pediatric gastroenterology or surgery clinic after initial diagnosis. Resolution of rectal prolapse occurred in 33 of those patients by medical management alone, average age being 3 years. Eight patients required treatment of their underlying illness, such as gluten-free diet for celiac disease. The rest were prescribed a laxative or fiber supplement, with polyethylene glycol 3350 being the most common laxative, prescribed in 88% of patients. The average duration of prolapse was 2 months. Most patients were referred to surgery if they continued to have recurrent rectal prolapse despite medical therapy, with an average duration of 5 months with recurrent rectal prolapse. Eleven patients were scheduled for surgery following the first consultation either because of duration of prolapse or age of onset; shortest duration documented of 7 months and youngest age of 6 years.
Thirty-four patients (22%) required surgical management. The most common procedure was linear cauterization with a circumferential submucosal retention suture (cerclage), average age was 6 years. The second most common procedure included linear cauterization alone, with an average age of 8 years.
One patient required laparoscopic rectopexy after failing both linear cauterization and cauterization with cerclage. The onset of rectal prolapse for this patient was 7 years of age, requiring surgical management at 8 years of age, eventually undergoing rectopexy at 10.
The goal of this study was to reassess the clinical characteristics of rectal prolapse and to compare previous studies to determine if major changes have occurred in the presentation of rectal prolapse in the past few decades (1). In older studies, cystic fibrosis was a leading diagnosis associated with rectal prolapse. Zempsky et al (1) reported 11% of patients with the associated diagnosis of cystic fibrosis. This was the third leading cause in their chart review. Stern et al (8) reported that 18.5% of patients with cystic fibrosis presented with rectal prolapse, and 78% presented with prolapse before diagnosis. Recommendations at that time suggested performing sweat chloride tests on all patients presenting with rectal prolapse, as cystic fibrosis was a common risk factor. In our study, only 4 patients, or 3%, were diagnosed with cystic fibrosis. Similar findings were also reported in JPGN in 2015 by El-Chammas et al (9), which 3.6% of patients had associated cystic fibrosis with rectal prolapse. The implementation of cystic fibrosis newborn screening began in the October of 2007 for the State of Michigan. After implementation, patients are now diagnosed earlier in life and before clinical manifestations of the disease present itself. All of the patients diagnosed with cystic fibrosis in our study were born before 2007.
Many case reports have recently published unique medical conditions presenting with rectal prolapse (2–6,10,11). We found 2 cases of patients presenting with rectal prolapse that were eventually diagnosed with celiac disease. Both had resolution of the prolapse after initiation of a gluten-free diet. Another case report published by Errazuriz et al (12), describes 2 patients presenting with rectal prolapse and diarrhea, who eventually were diagnosed with celiac disease. As malabsorption is not only a clinical manifestation of celiac disease but also a risk factor for rectal prolapse, it is not surprising to see this association. Thus, we recommend screening patients for celiac disease if clinically relevant.
A large percentage of patients in our study had a diagnosis of neuropathic disorders or anatomical abnormalities with history of surgical corrections. Our results are similar to other studies (1). These patients are at high risk for rectal prolapse, as they tend to have pelvic floor weakness because of ineffective innervation, lack of musculature support, or anatomical variants (13).
In our study, the mean age of patients presenting with rectal prolapse was 3 years and 11 months. This is similar to what has been reported in previous studies. It is known that patients less than 4 years of age are at a higher risk of prolapsing mainly because of the difference in anatomy; vertical and low position of rectum, mobility of sigmoid, and immature development of levator ani muscle (14). After the age of 4 years, the sigmoid and rectum take on its adult shape and shifts posterior; thus, decreasing the amount of pressure applied directly to the rectum (15). For this reason, there may be spontaneous resolution in many cases where good results have been attributed to laxative treatment of constipation.
Only half of the patients diagnosed with constipation described their stools as hard in consistency. The most common complaint was straining. Straining leads to increased intra-abdominal pressure onto the rectum, increasing the chances of prolapsing (13). Inadequate sitting position does not allow the normal orientation for defecation, and prevents ease of stool movement. We believe that correct toilet sitting position is as, if not more, effective in management of these patients compared with prescribing laxatives.
Fourteen patients required reduction in the hospital setting. A majority of reduction was successful by digital pressure with sedation. Sedation was required because of patient's anxiety and pain. It is known that the longer a prolapse persists, the more difficult it is to reduce because of progressing edema of the prolapsed mucosa (2). Because of this, reduction should be attempted as soon as possible. Often steady and firm digital pressure to the prolapsed mucosa will reduce the prolapse (2). Some may need additional treatment, as was the case in 2 of our patients who required sugar application, which draws in water, reducing the edema of the prolapsed mucosa (16).
Only 49% of patients had documented resolution of their prolapse. More than likely this is because of the nature of being in a tertiary care center with a larger proportion of complex patients. Polyethylene glycol 3350 was the most common medication prescribed in patients with resolution, but was also the most prescribed for all patients presenting with prolapse. Due to this, it is difficult to conclude what or if a certain laxative is more superior to others. On the basis of the clinical characteristics of patients with prolapse, age of onset seems to be the major factor in predicting the success of treatment.
Twenty-two percentage of patients failed medical management of their rectal prolapse and required surgical management. The most common surgical procedure performed was linear cautery with cerclage. Linear cauterization alone was the second most common. This is similar to other centers who surgically manage recurrent rectal prolapse. Linear cautery entails placing multiple linear tracks along the rectal mucosa by cauterization. This leads to an inflammatory reaction resulting in fibrosis and shortening the mucosa causing adherence to the perirectal tissue (17). Linear cautery with cerclage, also known as Thiersch's anal cerclage, entails placing a circumferential submucosal suture at the anal opening, thus reducing the circumference of the anal opening and decreasing the chance of prolapse. One of our patients required laparascopic rectopexy because of failure of both linear cautery and lineary cautery with cerclage. In this procedure, a suture is placed between the rectum and sacral prominence, which prevents further rectal prolapse.
There is wide variability in the type of procedure performed for recurrent rectal prolapse, especially in the pediatric population because of its low prevalence (17). At this time, it is thought that the type of surgical procedure is decided based upon the surgeon's previous training and own experience (18,19).
In our population, the patients who required multiple surgical procedures were older than 4 years of age. This corresponds to other studies in which the prognosis of rectal prolapse tends to be worse in older children (17). In addition, of those who failed the first surgical procedure, many had underlying factors that predispose them to poor complications, such as anorectal abnormalities. Taking this into consideration, more than likely one of the major factors in surgical failure is not based on surgical performance or the type of procedure, but rather the patient's medical history and clinical characteristics involving the prolapse.
Interestingly enough, in this study, patients with behavioral disorder or social stressor seem to have a high recurrence rate and duration of prolapse. Others have reported similar findings. Marceau et al (20) found 50% of patients with rectal prolapse younger than 50 years of age suffered from severe psychiatric disease. Van Heest et al (21) found a larger proportion of mentally challenged patients with rectal prolapse refractory to medical and surgical intervention. Some suggest the constipating effects of psychiatric medications may be a factor in the high prevalence of rectal prolapse (22). Hill et al suggests using multidisciplinary approach in this cohort, including behavioral and physical therapy (23). We recommend obtaining a thorough history of the prolapse, including a social history so that appropriate referrals for social and mental health services can be made.
Our study has the limitation of its retrospective nature. Secondly, with its limitation to a tertiary care center and large surgical center, our data may be skewed with a description of cases more complicated than what is typically seen in the medical field.
In conclusion, this retrospective analysis demonstrates that rectal prolapse and its characteristics have changed. Cystic fibrosis is no longer a common diagnosis, now with the implementation of newborn screening. Different diseases have now been reported with rectal prolapse previously unknown; such as celiac disease. Straining is the most common complaint, suggesting appropriate sitting techniques are essential in management.
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