Introduction: Tethered cord syndrome(TCS) is a form of an occult spinal dysraphism which include low lying conus, tight filum terminale, lipomeningomyelocele, split cord malformations, dermal sinus tracts, and dermoids. This congenital disorder has been associated with musculoskeletal, neurological, and gastrointestinal abnormalities.
Methods: This was a retrospective review of the prospectively collected Nationwide Inpatient Sample(NIS) database from 2003-2012. NIS supplied hospital- and year-adjusted weights allowed for accurate assessment of incidence of TCS, as well as cardiac and gastrointestinal(GI) and genitourinary(GU) anomalies.
Results: 13,470 patients were identified with TCS diagnosis. 5,488(40.7%) of TCS patients have at least one additional anomaly. Secondary anomalies most frequently presented in the following systems: spine(24.48%), cardiac(6.27%), urinary (5.37%), GI(4.55%), nervous(3.16%), GU(2.48%), and bone(1.01%). 1,691(12.55%) of TCS patients had more than one body system anomaly. Of patients with more than one associated anomaly, the most common combination of body system anomalies were GI and cardiac(4.55%), urinary and GI(4.26%), and urinary and cardiac(4.19%). The most common spinal associations were: 13.65% spina bifida, 0.39% Klippel-Feil, and 0.12% with torticollis. The most common neurological and musculoskeletal anomalies were: 13.45% with any VACTERL association, 3.58% with Rubenstein-Taybi syndrome, 0.42% with hydromyelia, 0.16% with chromosome 22 defect/deletion. 23.8% of TCS patients with microcephalus also had patent ductus arteriosus and 18.0% of TCS patients with reduction deformities of the brain presented with atrial septal defect. The most common specific anomalies were spina bifida, large intestine atresia, Rubenstein-Taybi syndrome, and atrial and ventral septal defects.
Conclusions: This study provides a nationwide prospective on congenital anomalies and concurrent conditions present in the tethered cord syndrome patient population in the United States. 40.7% of TCS patients have at least one associated anomaly reported. The most common congenital anomalies studied were spina bifida, urogenital with or without cardiac septal defects, cytourethral anomaly or cystic kidney disease with or without large intestinal atresia.
Patient Care: This research will help inform physicians on the most commonly occurring congenital anomalies that are present in patients with tethered cord syndrome to help counsel patients and improve overall patient care
Learning Objectives: By the conclusion of this session, participants should be able to: 1) Describe the importance of identifying the top concurrent anomalies present in tethered cord patients in the United States, 2) Discuss, in small groups, the most common concurrent anomalies for each body system