Imaging evolution from “presyrinx” to syrinx in patient with spinal lipoma

Background: The evolution of syrinx formation has rarely been documented. Here, we report a patient whose “presyrinx” evolved on successive magnetic resonance (MR) images to a mature syrinx. Case Description: A patient had a lipoma and tethered cord at birth. At 3 weeks of age, he had undergone a partial removal of the lipoma and untethering of the spinal cord. At age 6, the thoracic MR images showed edema within the gray matter of the cord at the T7 level, consistent with a “presyrinx.” In addition, subsequent MR studies (i.e., at age 7) showed a small cavity in the right posterior horn of the cord accompanied by further expansion throughout the right-sided gray matter. Despite repeated cord untethering at age 7, the T7 parenchymal cord change evolved into a mature syrinx by age 10. Conclusion: An infant with a lipoma/tethered cord, despite two instances of cord detethering (i.e., ages 3 weeks and 7 years), showed continued MR evolution of a “presyrinx” to a mature syrinx by age 10.

Spinal dysraphism with tripedus morphology: A case report

This is a rare case report of a 5-month-old child with a complex spinal dysraphic state, and an accessory limb (tripedus morphology), accessory genitalia, and anal dimple. The child was brought to the hospital with an accessory limb arising from the back. On clinical examination, an accessory limb arising from the lower back with a partially developed foot with the presence of toes and nails was noted. Spinal MRI was advised which revealed dysraphic features including spina bifida with the low lying and posteriorly tethered cord with diastematomyelia along with a supernumerary appendage attached to the vertebral column having rudimentary bones resembling those of extremities. The presence of an accessory limb with spinal dysraphism is quite a rare anomaly. The condition can be treated by surgical intervention and involves excision of the accessory limb with adequate dural and paraspinal muscle cover.

Spinal cord detethering without laminectomy or laminotomy

Background: Tethered cord syndrome occurs when there is abnormal tension on the distal spinal cord, which limits its elevation as patients grow. This results in stretching of the neural elements and microvasculature, resulting in both direct and ischemic injury.[7] Animal studies suggest that impairment of oxidative metabolic pathways may contribute to neuronal injury.[7] Associated conditions include myelomeningocele, lipomyelomeningocele, intraspinal lipomas, diastematomyelia, thickened/fatty filum terminale, and trauma.[2] Tethering may be asymptomatic or result in a variety of symptoms including lower extremity weakness/sensory deficits, bowel/bladder dysfunction, scoliosis, pes cavus, and back/leg pain.[6] Early surgical intervention has been shown to improve outcomes and may be performed prophylactically or to prevent symptom progression.[1,3] More specifically, retrospective studies demonstrate that surgical intervention in patients under the age of 2 years is associated with improved outcomes.[5] In some cases, detethering may result in clinical improvement.[3] Case Description: We present a case of a 6-month-old male with a low-lying conus medullaris, lumbar syrinx, mildly abnormal urodynamic studies, and asymmetric utilization of his lower extremities observed during the evaluation of a Y-shaped gluteal cleft. He underwent elective spinal cord detethering via the safe and effective, minimally invasive technique described in the video. The patient’s parents gave informed consent for treatment and video recording. Institutional review board approval was deemed unnecessary. Conclusion: Given the variety of surgical techniques used for cord detethering, this video may assist other surgeons in developing techniques that require little to no compromise of the developing bony spinal column while achieving sufficient release of the spinal cord.[4]

A rare case of fetus in fetu in the sacrococcygeal region: CT and MRI findings

Background Fetus in fetu is a rare condition in which a malformed fetus is found in the body of a living twin. The retroperitoneum is the most common location of this condition. However, the sacrococcygeal region is a rare site of the disease. The presence of vertebral bodies and limbs differentiates FIF from teratoma. Imaging modalities are important for diagnosing FIF. Case presentation A 12-months old boy was hospitalized because of a mass in the sacrococcygeal region. CT showed a large, complex mass with bony structure resembling sacrococcygeal bone, hip bone and the femur in the sacrococcygeal region of the boy. The blood supply of the mass was from the aorta of the host. MRI revealed the mass was connected with the dilated sacral canal of the host, which resulted in tethered cord. A preoperative diagnosis of FIF was made and surgery was performed to remove the mass. Surgical removal and subsequent pathological examination revealed the anencephalic fetus had limb buds and a sacrum but no axial skeleton, which supported the diagnosis of FIF. Conclusions CT and MRI played important roles in diagnosing FIF based on the location of the lesion.

Estimating the prevalence of neurosurgical interventions in adults with spina bifida using the Health Facts data set: implications for transition planning and the development of adult clinics

OBJECTIVE As the care of patients with spina bifida continues to evolve, life expectancy is increasing, leading to a critical need for transition planning from pediatric-based to adult-based care. The burden of neurosurgical care for adults with spina bifida remains unknown. In this study, the authors sought to use a large national data set to estimate the prevalence of neurosurgical interventions in adults with spina bifida. METHODS This study utilized Health Facts, which is a de-identified proprietary data set abstracted from all Cerner electronic health records. It includes 69 million unique patients with > 500 million encounters in 580 centers. Validation, technical exclusions, and data filters were applied to obtain an appropriate cohort of patients. The ICD-9 and ICD-10 codes for all types of spinal dysraphism, as well as the Current Procedural Terminology (CPT) codes for hydrocephalus procedures, spinal cord untethering, and Chiari decompression, were queried and records were retrieved. Demographic variables along with differences in age groups and temporal trends were analyzed. RESULTS Overall, 24,764 unique patients with ≥ 1 encounter with a spinal dysraphism diagnosis between 2000 and 2017 were identified. The pediatric cohort included 11,123 patients with 60,027 separate encounters, and the adult cohort included 13,641 patients with 41,618 separate encounters. The proportion of females was higher in the adult (62.9%) than in the pediatric (51.4%) cohort. Annual encounters were stable from 2 to 18 years of age, but then decreased by approximately half with a precipitous drop after age 21 years. The sex distribution of adults and children who underwent procedures was similar (54.6% female adults vs 52.4% female children). Surgical interventions in adults were common. Between 2013 and 2017, there were 4913 procedures for hydrocephalus, with 2435 (49.6%) adult patients. Similarly, 273 (33.3%) of the 819 tethered cord procedures were performed in adults, as were 307 (32.9%) of 933 Chiari decompressions. CONCLUSIONS The Health Facts database offered another option for studying care delivery and utilization in patients aging with spina bifida. The median age of this population has now reached early adulthood, and a significant number of neurosurgical procedures were performed in adults. An abrupt drop in the rate of encounters occurred at 21 years of age, possibly reflecting transition issues such as access-to-care problems and lack of coordinated care.

Intradural view of the spinal cord and dura after three-column osteotomy: illustrative case

BACKGROUND A three-column osteotomy results in dural buckling, which may appear concerning upon intraoperative visualization because it may appear that the neural elements may also be buckled. The authors presented an intraoperative view after intentional durotomy of the neural elements and the relaxed state of the dura after three-column osteotomy. OBSERVATIONS A 52-year-old woman with adult tethered cord syndrome and previous untethering presented with worsening leg pain and stiffness, urinary incontinence, and unbalanced gait. Magnetic resonance imaging demonstrated an arachnoid web at T6 and spinal cord tethering. Spinal column shortening via three-column osteotomy was performed with concomitant intradural excision of the arachnoid web. Dural buckling was observed intraoperatively after spinal column shortening. After the durotomy, the spinal cord was visualized without kinking or buckling. LESSONS Dural buckling after spinal column shortening of 15 mm via three-column osteotomy at T6 did not result in concomitant buckling of the underlying neural elements.