Clinical and Radiologic Characteristics of Caudal Regression Syndrome in a 3-Year-Old Boy: Lessons from Overlooked Plain Radiographs
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Caudal regression syndrome (CRS) is a rare neural tube defect that affects the terminal spinal segment, manifesting as neurological deficits and structural anomalies in the lower body. We report a case of a 31-month-old boy presenting with constipation who had long been considered to have functional constipation but was finally confirmed to have CRS. Small, flat buttocks with bilateral buttock dimples and a short intergluteal cleft were identified on close examination. Plain radiographs of the abdomen, retrospectively reviewed, revealed the absence of the distal sacrum and the coccyx. During the 5-year follow-up period, we could find his long-term clinical course showing bowel and bladder dysfunction without progressive neurologic deficits. We present this case to highlight the fact that a precise physical examination, along with a close evaluation of plain radiographs encompassing the sacrum, is necessary with a strong suspicion of spinal dysraphism when confronting a child with chronic constipation despite the absence of neurologic deficits or gross structural anomalies.
Abstract
Caudal regression syndrome (CRS) is a rare neural tube defect that affects the terminal spinal segment, manifesting as neurological deficits and structural anomalies in the lower body. We report a case of a 31-month-old boy presenting with constipation who had long been considered to have functional constipation but was finally confirmed to have CRS. Small, flat buttocks with bilateral buttock dimples and a short intergluteal cleft were identified on close examination. Plain radiographs of the abdomen, retrospectively reviewed, revealed the absence of the distal sacrum and the coccyx. During the 5-year follow-up period, we could find his long-term clinical course showing bowel and bladder dysfunction without progressive neurologic deficits. We present this case to highlight the fact that a precise physical examination, along with a close evaluation of plain radiographs encompassing the sacrum, is necessary with a strong suspicion of spinal dysraphism when confronting a child with chronic constipation despite the absence of neurologic deficits or gross structural anomalies.
참고문헌 (14)
- Loening-Baucke V., "Prevalence, symptoms and outcome of constipation in infants and toddlers", J Pediatr, 146, pp.359~363 (2005)
- Duhamel B, "From the mermaid to anal imperforation : the syndrome of caudal regression", Arch Dis Child, 36, pp.152~155 (1961)
- Pang D, "Sacral agenesis and caudal spinal cord malformations", Neurosurgery, 32, pp.755~778 (1993)
- Aslan H, "Prenatal diagnosis of caudal regression syndrome : a case report", BMC Pregnancy Childbirth, 1, pp.8~ (2001)
- Kokrdova Z, "Caudal regression syndrome", J Obstet Gynaecol, 33, pp.202~203 (2013)
- Singh SK, "Caudal regression syndrome--case report and review of literature", Pediatr Surg Int, 21, pp.578~581 (2005)
- Zaw W, "Caudal Regression Syndrome in twin pregnancy with type II diabetes", J Perinatol, 22, pp.171~174 (2002)
- Shah JR, "Caudal regression syndrome with sacral rib : MRI features", Acta Radiol, 47, pp.862~864 (2006)
- Krenova Z, "Caudal regression syndrome in one of dizygotic twins", Fetal Pediatr Pathol, 29, pp.419~423 (2010)
- Sharma P, "Clinico-radiologic findings in Group II caudal regression syndrome", J Clin Imaging Sci, 3, pp.26~ (2013)
- Duncan MA, "Caudal regression syndrome : a case report", Med Univ, 16, pp.74~77 (2014)
- Bekkali NL, "Magnetic resonance imaging of the lumbosacral spine in children with chronic constipation or non-retentive fecal incontinence : a prospective study", J Pediatr, 156, pp.461~465 (2010)
- Tabbers MM, "Evaluation and treatment of functional constipation in infants and children : evidence-based recommendations from ESPGHAN and NASPGHAN", J Pediatr Gastroenterol Nutr, 58, pp.258~274 (2014)
- Diel J, "The sacrum: pathologic spectrum, multimodality imaging, and subspecialty approach", Radiographics, 21, pp.83~104 (2001)