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.

Non-dysraphic Subpial Intramedullary Lipoma of Cervicothoracic Spine

Intramedullary spinal cord tumors are rare and comprise less than one percentage of all central nervous system (CNS) neoplasms. When encountered, most are found in the lumbosacral region with coinciding spinal dysraphism. Here, we discuss a case of isolated non-dysraphic intramedullary lipoma of cervicothoracic spine in an adolescent who was surgically managed with posterior decompression and subtotal resection via laminoplasty. A 21-year-old male adolescent, without a history of spinal dysraphism, presented with a progressive sensory ataxia. This manifestation was attributed to magnetic resonance documented intramedullary lipoma, extending from C7 to T2 spinal level. Patient underwent subtotal surgical resection of the lesion; and histopathology report was confirmed as spinal lipoma. Surgical management of this rare pathology has a wide variety of options depending on clinical presentation. In our case, a subtotal resection and laminoplasty was achieved with no further worsening of neurologic symptoms.

Nondysraphic Intradural Spinal Lipoma: A Rare Case in a Child

Lipoma is a rare and benign tumor, with lesions associated with spinal dysraphisms. The reported case is about an 8-month-old child who arrived at the hospital with developmental delay, excessive crying, undergoing MRI and reported spinal cord lipoma. Intradural lipomas without association with dysraphism are difficult to diagnose, being the most accurate form of MRI.

Patterns of spinal cord malformation in cloacal exstrophy

OBJECTIVE The objective of this study was to assess the prevalence and spectrum of spinal dysraphism in a cohort of children with cloacal exstrophy (CEX) using MRI. METHODS Children with CEX presenting between 1999 and 2019 with baseline spinal MRI were included. The images were reviewed in consensus to assess the type of dysraphism. The dysraphisms were initially reviewed and described based on their descriptive anatomy, and then classified according to anomalies of gastrulation, primary neurulation, or secondary neurulation. RESULTS Thirty-four children were included. Thirty-three of these children had closed spinal dysraphism, and 1 had a normal spine. Of the 33 cases of closed spinal dysraphism, the conus and/or filum terminale were involved in all cases. The most common malformations were spinal lipoma (n = 20) and terminal myelocystocele (n = 11). The lipomas were heterogeneous: 4 dorsal, 9 transitional, 4 chaotic, and 3 terminal. A large subgroup (10/20, 50%) within the lipomas had an unusual morphology of noncontiguous double lipomas, the proximal fat related to the conus and the distal fat within the filum. These were difficult to characterize using existing classifications. In 2 cases, only a thickened filum was noted. The majority of these malformations were compatible with a disorder of secondary neurulation. CONCLUSIONS Complex spinal dysraphisms are consistently associated with CEX. The unusual dysraphism patterns found in this group of patients highlight the limitations of current embryological classifications. Given the propensity for neurological deterioration in this group of patients, spinal MRI should be routinely performed. The type and distribution of malformations seen have implications for the wider understanding of the pathogenesis and classification of lumbosacral lipomas.

Rapid deterioration of an asymptomatic lumbosacral lipoma due to formation of an extracanalicular syrinx: case report

The authors present the case of a 1-month-old girl with a lumbosacral lipoma who then developed an extracanalicular syrinx and experienced rapid deterioration. The patient’s initial MRI study, obtained before she became symptomatic, revealed a spinal lipoma with a syrinx in contact with the lipoma-cord interface. She was initially asymptomatic but developed loss of motor function in the left leg 14 days after MRI. Emergency surgery was performed. Intraoperative findings revealed a swollen spinal cord. Lipomatous tissue on the caudal side of the conus was removed subtotally, and the central canal was opened. Expansion of the syrinx was observed intraoperatively. Postoperatively, the patient’s left leg paresis remained. Postoperative MRI revealed rostral and extracanalicular expansion of the syrinx. This is the first report on the rapid deterioration of a conus lipoma due to extracanalicular expansion of a syrinx. Careful follow-up and repeat MRI should be considered for patients with spinal lipomas with syringomyelia, especially when the syrinx is attached to the lipoma-cord interface.