Treatment of patients with caudal regression syndrome: a systematic review of the literature

Abstract

Objective. To analyze and summarize the data of modern literature on the issues of surgical treatment and natural course of the spine and spinal cord pathology in patients with various types of caudal regression syndrome (CRS).Material and Methods. A systematic review of the literature on the issue of treatment of the spine and spinal cord pathology in patients with CRS was performed. Selection criteria were: articles for the period 2002–2022, original studies of populations/patients with various forms of CRS with a description of treatment methods and long-term results of treatment or observation. A total of 28 articles on the treatment of various forms of CRS with the described results of treatment of 212 patients were analyzed: 29 patients with CRS in combination with open neural tube defects and 183 patients with closed forms of CRS. Evaluation criteria included number of patients, gender, type of spinal cord pathology, type of sacral agenesis, presence of the spine and lower extremities deformities, concomitant pathology, operations performed and their complications, and results.Results. The studied patients underwent the following surgeries on the spine and spinal cord: untethering of the spinal cord, correction and stabilization surgeries on the spine, plasty of the spinal cord herniation, plasty of the terminal meningocele, and removal of the presacarial volumetric mass. The greatest number of complications occurred after operations on the spine and sacrum. The majority of patients (67 %) with sacral agenesis by the end of the follow-up period (average 14 years) walked independently or with the help of devices, and a minority of them (33 %) could not walk. More than half of patients with CRS (67 %) had a neurogenic bladder, urinary incontinence, or suffered from a chronic urinary tract infection. Fecal incontinence and constipation were less common (46 %).Conclusions. Patients with CRS have a good potential for improvement/recovery of walking and pelvic organ dysfunction. This is extremely important to timely carry out multimodality treatment of patients with CRS who have neurosurgical, orthopedic, urogenital and colorectal problems in CRS, and to start early motor rehabilitation and physiotherapy.