Xu Hongmin, Gagnon Marie-helene, Ali Sumera, Linam Leann
Final Pr. ID: Poster #: EDU-039
The purpose of this educational exhibit is to:
1.Review the embryology, classification, and imaging features of anorectal malformations (ARMs).
2.Review the indications and techniques of Contrast-enhanced colosonography (CeCS) in pediatric patients with ARMs.
3.Provide sample cases and clinical courses of patients with specific ARMs.
Anorectal malformations(ARMs) are a spectrum of congenital anomalies involving abnormal development of the distal rectum and anus, leading to an interruption or misconnection of the normal passage between the rectum and the perineum. The condition occurs in about 1 in 5,000 live births and can range from mild to complex. Children with ARMs require preoperative imaging to assess the presence and location of a fistula. Identifying the location of these fistulas is crucial for surgical planning.
Contrast-enhanced colosonography (ceCS) has emerged as a valuable tool in diagnosing pediatric ARMs, offering superior anatomical visualization and assessment compared to traditional imaging techniques like fluoroscopy, providing more sensitive and specific evaluation of fistula with real-time contrast filling. CeCS avoids ionizing radiation, and ultrasound contrast agents have a high safety profile with low incidence of adverse events compared to other contrast agents.
Contrast-Enhanced colosonography (CeCS) technique should include survey greyscale ultrasound examination of the pelvis to assess for adequate window. Lumason, the ultrasound contrast agent, is prepared and injected into a bag of saline, with a ratio of 1:500 mL. The mucous fistula is examined, and a small Foley catheter is inserted by the radiologist, then balloon is inflated to create a seal. Contrast is then instilled by gravity drip, and perineal structures are imaged through three standard approaches- transabdominal, transperineal and posterior sagittal approaches.
As well as reviewing the topics above, this review will also provide examples of ARM cases demonstrating high, intermediate, and low fistulas seen on CeCS.
Overall, CeCS enhances the diagnostic accuracy and management of pediatric ARMs. By providing detailed anatomical insights and allowing for accurate classification, it plays a critical role in guiding treatment strategies and improving long-term outcomes for affected patients.
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Authors: Xu Hongmin , Gagnon Marie-helene , Ali Sumera , Linam Leann
Keywords: Anorectal Malformation, Contrast Enhanced Ultrasound, Pre-Surgical Planning
Wepking Kelly, Gill Kara, Riedesel Erica
Final Pr. ID: Poster #: CR-068
Currarino’s Syndrome (CS) is a unique form of caudal regression syndrome characterized by triad of sacral dysplasia, anorectal malformation, and presacral mass. Additional congenital malformations of the genitourinary tract and spine may be present.
Currarino's Syndrome has a variety of clinical presentations - from asymptomatic to severe chronic constipation - depending on associated malformations. Early identification and adequate treatment prevents eventual serious complications.
Plain radiograph of the sacrum is the first diagnostic step. Ultrasound or pelvic/spinal MRI is used to better evaluate for presacral mass. Fluoroscopic enema studies may be used for further evaluation of anorectal malformation. Additional imaging of the GU tract with US and VCUG is suggested due to risk for associated GU anomaly or vesicoureteral reflux.
We present two cases of Currarino’s Syndrome which demonstrate classic imaging findings on plain film radiograph, ultrasound, and MRI.
Case 1 presented in infancy with severe chronic constipation. Abdominal radiograph demonstrated classic "scimitar" sacrum. Fluoroscopic enema demonstrated high grade anal stenosis. US and MRI of the spine and sacrum revealed large anterior sacral meningocele.
Case 2 presented at birth with imperforate anus. Abdominal radiograph demonstrated classic "scimitar" sacrum. MRI of the spine and sacrum revealed small anterior sacral meningocele. Additional GU anomalies were discovered including vesicoureteral reflux and uterine didelphis.
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Authors: Wepking Kelly , Gill Kara , Riedesel Erica
Keywords: Currarino's Triad, Sacral dysplasia, Presacral mass, Anorectal malformation, Case Report
Belachew Bethelhem, Silva De Campos Meneses Marcus Otavio, Moreira Vidal Lorenna
Final Pr. ID: Poster #: EDU-090
Abstract:
Sacral development is a key determinant of bowel and bladder function in children with anorectal malformations (ARM) and sacral agenesis. Accurate radiologic assessment of the sacrum provides essential prognostic information, guiding both surgical planning and long-term management. We aim to review the imaging-based metrics used to evaluate sacral development, highlighting their significance in predicting continence outcomes.
ARM result from abnormal development of the hindgut and cloacal membrane between weeks 6 and 9 of gestation. These malformations can vary significantly, ranging from minor anal displacement to complex defects that involve the rectum, vagina, and urethra. Both the anorectal structures and the sacrum develop from the caudal mesoderm during the same period, which means that sacral anomalies, such as hypoplasia or agenesis, often occur alongside ARM. Due to this close embryologic origin, the sacrum can provide important insights into the prognosis of ARM, and specific metrics can offer objective information for assessment.
The sacral ratio (SR), derived from anteroposterior (AP) and lateral pelvic radiographs, is the most widely used quantitative measure. It stratifies patients into prognostic categories, with SR ≥ 0.7 indicating good continence potential and SR < 0.4 associated with poor outcomes. However, SR is subject to variability due to patient positioning, pelvic tilt, and image quality.
Additional metrics such as the sacral index (SI) and sacral curvature (SC) offer complementary insights. SI reflects sacral width and height and has been correlated with ARM severity. SC provides a qualitative measure of sacral shape and is associated with continence outcomes. These measurements are often limited by the constraints of conventional radiography and the technical challenges of MRI, including non-isotropic data and restricted field of view.
This educational exhibit will provide a comprehensive review of sacral measurements in ARM and sacral agenesis. We encourage a standardized and multimodal approach to sacral assessment, and provide information for a deeper understanding of:
- Sacral and anorectal malformations embryology
- Key radiologic metrics (SR, SI, SC) and their interpretation
- Limitations and considerations in measurement techniques
- The prognostic significance of sacral development
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Authors: Belachew Bethelhem , Silva De Campos Meneses Marcus Otavio , Moreira Vidal Lorenna
Keywords: Anorectal Malformation, Sacrococcygeal, Xray