Amrami, Kimberly K.

BACKGROUND Cystic adventitial disease (CAD) and intraneural ganglion cysts (IGCs) are rare mucinous lesions typically linked to an articular origin via macroscopic conduits. While secondary propagation between vascular and neural systems may occur through shared fascial sheaths, a direct microvascular route has not been previously described. OBSERVATIONS A 60-year-old man presented with progressive left tibial neuropathy. High-resolution 7T MRI and surgery revealed a large popliteal CAD lesion connecting to the knee joint via the middle genicular artery, alongside a secondary tibial IGC lacking an independent articular nerve connection. Findings demonstrated that high-pressure CAD mucin tracked along a small connecting vascular branch, creating a novel microvascular arterio-neural pathway to infiltrate the tibial nerve. Targeted articular branch disconnection and cyst decompression yielded significant clinical improvement, with no recurrence on 7-month follow-up 7T MRI. LESSONS This case expands the unifying articular theory, demonstrating that synovial fluid propagation can exploit a microvascular arterio-neural pathway rather than strictly macroscopic fascial planes. High-resolution 7T MRI is essential for visualizing these subtle connections. Successful management requires meticulous identification and disconnection of the primary joint connection to prevent recurrence. https://thejns.org/doi/10.3171/CASE26411

BACKGROUND Neurolymphomatosis (NL), characterized by lymphoma infiltration of peripheral nerves, can be diagnostically challenging because of nonspecific clinical presentations and imaging findings. Tumefactive NL refers to circumferential subparaneurial tumor deposition beneath the paraneurium, outside the epineurium, producing an “encased” nerve appearance on MRI and at surgery. OBSERVATIONS A 76‑year‑old man with treated large B‑cell lymphoma developed a painful left lower trunk brachial plexopathy with numbness of the ring and little fingers and progressive weakness in finger flexors and intrinsic hand muscles. Serial brachial plexus MRI and 18F-fluorodeoxyglucose positron emission tomography over 4 months demonstrated evolution from a thin, circumferential subparaneurial rim of enhancement with mild nerve enlargement to a homogeneously enhancing, tumefactive lesion of the C8 nerve root and lower trunk. Targeted fascicular biopsy of a nonfunctioning C8 fascicle confirmed recurrent B‑cell lymphoma. LESSONS This case illustrates temporal evolution along the tumefactive NL spectrum, from early, subtle subparaneurial involvement to a frankly tumefactive lesion. Recognizing this early MRI pattern, particularly in patients with a history of lymphoma and focal plexopathy, can guide targeted biopsy and facilitate earlier diagnosis and treatment. The clinicoradiological findings suggest that subtle peripheral/subparaneurial enhancement may precede overt tumefactive enlargement. https://thejns.org/doi/10.3171/CASE26139

BACKGROUND AND OBJECTIVES: Intraneural ganglion cysts (IGCs) can arise in different nerves but are uncommon in the tibial nerve (TN) and its branches near the ankle. According to the unified articular theory, a joint connection is always present in IGCs. This study aimed to identify joint connections of tibial-region IGCs and characterize their clinical, radiological, and propagation patterns. METHODS: A prospectively collected international database of >1100 IGCs was reviewed to identify tibial and medial plantar nerve (MPN) and lateral plantar nerve (LPN) IGCs around the ankle. RESULTS: Sixty-two ankle region IGCs were identified: 29 involving the TN (46.8%), 25 the MPN (40.3%), and 4 the LPN (6.4%); in 4 cases, the parent nerve was indeterminate. Thirty-one (50%) cases demonstrated joint connections. Of these, all TN at/distal to the tarsal tunnel (n = 4/4, 100%), MPN (n = 16/16, 100%) and LPN IGCs (n = 2/2, 100%) had a connection to the subtalar (ST) joint. A tibial IGC just proximal to the tarsal tunnel was connected to the tibiotalar (TT) joint in 75% of cases (n = 3/4) and the ST joint in 25% of cases (n = 1/4). Surgical ligation/disconnection of the articular branch was associated with markedly lower recurrence (0/13, 0%) compared with incision or resection of cyst without ligation (8/29, 27.6%; P = .04). CONCLUSION: Our findings support the unified articular theory. All IGCs in the ankle primarily ascend. Two distinct propagation patterns emerged: (1) those that ascend and enter the parent nerve proximal to the tarsal tunnel continue to ascend in the leg, without affecting the plantar nerves, and (2) those that ascend and enter the parent nerve at/distal to the tarsal tunnel have the potential to undergo crossover at the TN bifurcation and subsequently descend down the plantar nerves seen only in ST-origin cysts. Recognizing these pathways enhances diagnostic accuracy, directs identification of the joint of origin, and guides articular branch disconnection to reduce recurrence.

BACKGROUND Bertolotti syndrome is an underrecognized cause of chronic low back pain. Back pain generated from Bertolotti syndrome is believed to result from altered weight dissipation mechanics and/or instability of the pseudoarticulation, while local nerve root compression may result in radiculopathy. Here, the authors describe the findings of a patient with treatment-refractory low back pain found to have a cystic lesion arising from a Bertolotti joint causing L5 nerve root compression. To their knowledge, this has not been previously described in the literature. OBSERVATIONS Despite early recognition of Bertolotti syndrome, the patient’s back pain remained resistant to many years of both conservative and surgical management. The novel finding of a cyst arising from the pseudoarticulation complicated the management. The patient is scheduled to undergo further surgery this year. LESSONS This case highlights a pseudocyst associated with a Bertolotti joint. The cyst introduced additional clinical uncertainty to what is already an uncommon and difficult to treat condition. Resection of the Bertolotti joint failed to improve the patient’s symptoms. Postoperatively, a complex hemorrhagic pseudocyst was seen on imaging, possibly due to the pseudoarticulation with persistent motion. Ongoing efforts to characterize Bertolotti syndrome and its associated imaging findings are necessary to better optimize management strategies in these patients. https://thejns.org/doi/10.3171/CASE25948

BACKGROUND Intraneural ganglion cysts (IGCs) are mucinous lesions that originate from adjacent synovial joints. According to the articular theory, synovial fluid tracks from a joint into the parent nerve through an articular branch. In uncommon sites such as the sural nerve, this connection can be difficult to identify. This report illustrates the use of a preoperative intra-articular fluorescein injection as a tool to identify the articular branch during surgery. OBSERVATIONS A 60-year-old woman presented with lateral foot pain, numbness, and a palpable mass along the left sural distribution. MRI showed a large cyst extending along the course of the sural nerve with connection to the subtalar joint. Fluorescein was injected preoperatively into this joint to detect the articular branch intraoperatively with ultraviolet illumination. The connection was transected at the joint origin, and the cyst was decompressed, preserving the parent nerve. Postoperatively, the patient had complete pain relief and sensory recovery, and MRI at 3 and 8 months confirmed resolution of the cyst. LESSONS Sural IGCs conform to the articular theory and can be managed effectively by transection of the articular branch. Preoperative intra-articular fluorescein injection is a useful tool to detect the articular branch that can be difficult to find in sural IGCs. https://thejns.org/doi/10.3171/CASE251018

ABSTRACT The paraneurium is a distinct connective tissue sheath that loosely envelopes the epineurium. The space between the para‐ and epineurium (the subparaneurial compartment) holds significant clinical relevance, serving as a target for deposition of regional anesthesia and a potential pathway for the pathological spread of cysts and tumors. However, visualization of the paraneurial layer, as a separate structure from the epineurium, remains challenging with conventional MRI, especially in the absence of pathology. This study evaluated the visualization of the paraneurium using ultra‐high‐field 7‐Tesla (7 T) MRI compared to conventional field strengths. We assessed normal nerve anatomy and a spectrum of pathological cases, including intraneural ganglion cysts, schwannomas, intraneural perineuriomas, and metastatic melanoma. The results demonstrated that 7 T MRI provides superior resolution and delineation of the paraneurium compared to 1.5 and 3 T systems. In normal anatomy, T1‐weighted and proton density (PD) sequences provided optimal contrast between the paraneurium and surrounding adipose tissue. In contrast, for pathological cases, T2‐weighted sequences with fat suppression were superior for depicting the paraneurium and identifying subparaneurial involvement. In conclusion, 7 T MRI offers a significant advantage in identifying the thin paraneurial layer. This capability provides valuable insights into the pathogenesis of tumor spread and holds promise for improving pre‐operative planning and clinical decision‐making in peripheral nerve pathology.

BACKGROUND Recently, the distribution of subparaneurial ganglion cysts involving the common peroneal and tibial nerves was described based on MRI analysis. Crossover in extreme cases was observed at the sciatic bifurcation through a mechanism comparable to a geyser, enabled by fenestrations in the epi- and paraneurium at the level of the bifurcation. The goal of this study was to investigate whether the geyser theory for crossover holds for other locations. OBSERVATIONS The authors’ prospectively maintained database of > 1100 cases of intraneural ganglion cysts in the world’s literature was queried for the existence of subparaneurial ganglion cysts at locations other than the knee. A previously unrecognized case involving the ulnar nerve originating from the elbow joint was found. Reinterpretation of the patient’s MRI studies showed circumferential subparaneurial distribution of a cyst over a long distance. Based on their previously reported geyser theory, the authors wondered if crossover had occurred at the level of the medial cord. A fenestration in the epi- and paraneurium at this level was confirmed on histological analysis of a normal cadaveric specimen. LESSONS This illustrative case shows that the geyser theory also holds in other locations. Surgical treatment of these subparaneurial cysts still follows the principles of the unified articular theory. https://thejns.org/doi/10.3171/CASE25671

OBJECTIVE The formation and propagation of intraneural ganglion cysts (INGCs) is being elucidated by the unified articular theory. Its principles include a connection for joint fluid to egress from a synovial joint via an articular branch to a parent nerve and cyst following the path of least resistance, dependent on pressures and pressure fluxes. The occurrence of so-called extreme peroneal or tibial INGCs in the popliteal fossa extending to the sciatic nerve has been reported. One rarely described variant with a circumferential cyst within the subparaneurial compartment outside the epineurium of nerve(s) has been previously illustrated, but its mechanism and morphology have not been clarified. In this study, the authors aimed to investigate this type of cyst to challenge the principles of the unified articular theory. METHODS Four novel cases of patients with peroneal INGCs and "extreme subparaneurial cyst(s)" of the sciatic nerve and its distal branches were investigated: 3 arose from the superior tibiofibular joint (STFJ) and 1 from the knee joint. Three other cases of recognized extreme subparaneurial cyst (2 peroneal and 1 tibial from the STFJ) and 1 case of a peroneal subparaneurial cyst in the literature were reinterpreted. Data on clinical presentation, MR images, and surgical results were analyzed. RESULTS In all 8 cases, subparaneurial extension was observed to different degrees along the sciatic, tibial, common peroneal, sural, and deep and superficial peroneal nerves, as was subparaneurial-to-subparaneurial communication at the sciatic nerve bifurcation (i.e., crossover). Sequential MRI performed in 7 patients showed variable dynamic changes, including extreme ascent and descent. Extraneural rupture of the subparaneurial cyst, with spread into the surrounding tissue, was present at the sciatic nerve bifurcation in 6 cases. CONCLUSIONS The authors provide pathoanatomical and pathophysiological evidence supporting that extreme subparaneurial cysts follow the principles of the articular theory. They propose a distribution pattern that explains the occurrence and evolution of extreme subparaneurial cysts along the sciatic nerve and its distal branches in patients with peroneal or tibial INGCs and subepineurial cysts. Crossover in the subparaneurial compartment allows potentially extensive circumferential distribution within connected nerves. Also, dynamic factors can lead to dramatic changes in cyst size and appearance from reabsorption or extraneural rupture. In Part 2 of this study, the authors provide evidence showing that a fenestration in the epineurium allows cysts to pass from the subepineurial-to-subparaneurial, subparaneurial-to-subparaneurial, and subparaneurial-to-subepimyseal compartments.

OBJECTIVE Extreme subparaneurial ganglion cysts are poorly understood. In Part 1, the authors demonstrated that patterns of distribution in the subparaneurial space are consistent with the principles of the articular theory for intraneural ganglion cysts (INGCs). How a cyst transfers from the subepineurial to the subparaneurial compartment remains to be determined. In this part, the authors provide evidence supporting that cyst transfer occurs at the sciatic nerve bifurcation based on MRI and histological analysis, and introduce the geyser theory as an explanation for the redistribution of cysts. METHODS The authors analyze MR images obtained in patients with extreme subparaneurial cysts as well as newly identified cases of nearly extreme subparaneurial cysts (i.e., cysts visible in the subparaneurial compartment but not to the extreme degree as defined in Part 1) for potential crossover sites. They also analyze histological sections from 10 cadavers around the sciatic nerve bifurcation for the presence of openings in the epineurium and paraneurium. RESULTS MRI studies performed in 8 patients in Part 1 and 8 newly identified examples with nearly extreme subparaneurial INGCs showed evidence to support communication between different compartments at the sciatic nerve bifurcation (subepineurial-to-subparaneurial and subparaneurial-to-neighboring soft tissues). Openings in the epineurium of the common peroneal and tibial nerves at the sciatic nerve bifurcation were consistently found in all 10 cadaver specimens, as were openings in the paraneurium. CONCLUSIONS The authors expand on the evidence to support cyst redistribution at the sciatic nerve bifurcation for INGCs in the knee region. They put forth a logical explanation for the development of a spectrum of extreme INGCs (ranging from a faint amount of cyst around the epineurium [wedding ring sign] to extreme subparaneurial INGCs [owl eyes]) based on principles seen in a geyser. The authors believe that radiological occurrence of crossover can be explained by the presence of fenestrations in both the epineurium and paraneurium at the sciatic nerve bifurcation, allowing cyst crossover in two steps: from the subepineurial to the subparaneurial space ("cross") and between a shared subparaneurial space of different nerves ("over"). Finally, the frequent occurrence of extraneural rupture ("cross-out") is due to cyst transfer into the soft tissue compartment via an opening in the outermost circumneurial layer.