Impaired growth outcomes in children with congenital colorectal diseases.

BACKGROUND: Cloaca, Hirschsprung disease, and anorectal malformations (CHARM) are congenital anomalies of the hindgut. Small series have suggested that children suffering from one of these anomalies may be at risk for growth impairment. We sought to expand on these findings in a comprehensive cohort, hypothesizing that patients with Medicaid insurance or African-American (AA) race would be at higher risk for poor growth. METHODS: Following Institutional Review Board (IRB) approval, single-institution retrospective review of children with CHARM anomalies was performed (2009-2016). Body mass index (BMI) value Z-scores were obtained using the 2006 World Health Organization (age 0-24 mo) and 2000 Centers for Disease Control (CDC) (age >2 y) growth charts and calculators (statistical analysis system). Patient factors and BMI Z-scores were analyzed with descriptive statistics and Fisher's exact test. RESULTS: One hundred sixty-six patients (Cloaca n = 16, Hirschsprung disease [HD] n = 71, anorectal malformation [ARM] n = 79) were identified. The BMI Z-score distribution for the entire CHARM cohort was lower than controls (P < 0.0001). HD and ARM BMI Z-scores were also lower versus controls (P < 0.0007, P < 0.0037). Requiring more or less than the average number of surgeries did not impact BMI Z-score [P = non-significant (NS)]. Patients with Medicaid had lower Z-scores versus private or commercial insurance (P < 0.0001). AA race BMI Z-score distribution was lower than controls (P < 0.0002), but there was no statistical difference in BMI Z-scores when comparing AA versus non-AA CHARM patients (P = NS). CONCLUSIONS: Patients born with CHARM anomalies are at risk for impaired growth. Furthermore study is warranted to identify modifiable risk factors contributing to this impairment. Longitudinal follow-up should include interventions to mitigate these risks.

Low-cost instrumentation for the diagnosis of Hirschsprung's disease.

In Hirschsprung's disease, the internal anorectal sphincter fails to relax in response to rectal distension, which strongly indicates the absence of rectoanal inhibitory reflex (RAIR). Hirschsprung's disease is a very common case particularly encountered in the newborns in our region. Development of a manometric system targeted specifically for the diagnosis of this disease at a reasonable cost is an urgent need identified by our regional colorectal surgeons. These surgeons indicated that commercially available anorectal manometers are too expensive to acquire. Therefore, in our research we tried to develop a low-cost single balloon-transducer system, which only provides information about RAIR, and hence diagnoses the Hirschsprung's disease. The hardware part of our instrumentation is made of a latex balloon, pressure transducer, amplifier, and A/D converter circuits, which all collects the pressure readings and sends the data to the computer. The manometer system software, programmed based on Delphi, displays these readings and patient information on a computer screen. This designed system was successful enough to perform manometric recording of RAIR in the anorectal ampulla of rabbits and rats.

[Value of anorectal manometry in assessment of constipation in childhood]. / Stellenwert der anorektalen Manometrie bei der Abklärung der Obstipation im Kindesalter.

We studied retrospectively 210 anorectal manometries of constipated children. Of the 87 patients with an anal fissure or a functional constipation, 83 had normal sphincter relaxation. All of the 23 patients with Hirschsprung's disease lacked the sphincter relaxation, as well as 22 of the patients with a dysganglionosis. Eleven patients with innervation defects showed pathologic sphincter contractions. Anorectal manometry is a valuable tool to differentiate between innervation defects and constipation of other etiologies.

Assessment of anorectal motility measurements in the management of adult megacolon.

Anorectal motility was studied in 23 patients with adult megacolon. A balloon was used for rectal distension, and electrical signals from the anal canal or rectal mucosa were monitored with a silver/silver chloride electrode. Intraluminal pressure was measured by a pressure transducer linked to the vicinity of the electrode via a polythene tube. Responses to rectal distension provided a means of distinguishing patients with adult Hirschprung's disease from ones with idiopathic megacolon. Some patients with the latter condition showed an elevation of mean anal canal pressure relative to that in normal subjects. There was no statistically significant difference in slow-wave frequency in the anal canal between patients and adult megacolon and normal subjects. The fall in anal canal pressure associated with rectal distension was correlated with a decline in the amplitude of slow-wave electrical activity. The measurement of anorectal motor parameters was of value in the management of adult megacolon.

Manometric assessment of anorectal pressures in Hirschsprung's disease after Rehbein's operation with and without anorectal myectomy.

Anorectal manometric studies were performed on 15 patients with Hirschsprung's disease before surgery, on eight patients after Rehbein's procedure and seven patients after Rehbein's procedure with anorectal myectomy. The results were compared with those of 45 normal subjects. High anal resting pressure as well as an absence of the anorectal reflex proved to be responsible for obstructive symptoms in Hirschsprung's disease. Out of eight patients treated according to Rehbein's procedure, new internal sphincter relaxation was found in two, and anal resting pressures were significantly higher than those of the normal controls or of the patients treated according to Rehbein's procedure plus anorectal myectomy. It was demonstrated that anorectal myectomy is a more definitive procedure to restore the anal resting pressure to normal. Good postoperative continence can be achieved not only by the new internal sphincter relaxation but also by good propulsive movement which can overcome the remaining functional obstruction.