External jugular venous sampling for Cushing’s disease in a patient with hypoplastic inferior petrosal sinuses

Abstract

<jats:p>The authors report the case of a 30-year-old female patient with suspected Cushing’s disease with an anatomical variation of hypoplastic inferior petrosal sinuses and nearly exclusive anterior drainage from the cavernous sinus, who underwent external jugular venous blood sampling with successful disease confirmation and microadenoma localization. The patient presented with signs and symptoms consistent with Cushing’s syndrome, but with discordant preliminary diagnostic testing. She underwent attempted bilateral inferior petrosal sinus sampling; however, she had hypoplastic inferior petrosal sinuses bilaterally and predominantly anterior drainage from the cavernous sinus into the external jugular circulation. Given this finding, the decision was made to proceed with external jugular venous access and sampling in addition to internal jugular venous sampling. A positive adrenocorticotropic hormone (ACTH) response to corticotropin-releasing factor was obtained in the right external jugular vein alone, suggesting a right-sided pituitary microadenoma as the cause of her Cushing’s disease. The patient subsequently underwent a transsphenoidal hypophysectomy that confirmed the presence of a right-sided ACTH-secreting microadenoma, which was successfully resected. She was hypocortisolemic on discharge and has had no signs of recurrence or relapse at 6 months postoperation.</jats:p>

Revalidation of inferior petrosal sinus sampling: the latest results from a single-center experience

“…If both sides are type 4, IPSS is virtually impossible to perform. Interestingly, Peterson et al recently reported that sampling from both external jugular veins could substitute for IPSS in rare cases [15]. Our group previously reported that, with negative MRI, postoperative remission was achieved in very rarely of their patients with negative IPSS and 50% of those with positive IPSS [11,16].…”

Effect of intercavernous sinus drainage pattern on exactness of bilateral inferior petrosal sinus sampling results in foretell the area of Cushing’s disease tumors

“…BIPSS is considered the gold standard for differentiating between pituitary and ectopic sources of ACTH hypersecretion, but it cannot be used for diagnosing the location [20,21] . We believe this may be in uenced by factors such as the intercavernous sinus drainage pattern and the signi cant variation in intraoperative catheter placement.…”

An algorithm to improve lateralization accuracy of inferior petrosal sinus sampling: procedural nuances for complex patterns of venous drainage. Patient series

“…Venous drainage of the cavernous sinus can be altered by the presence of a plexiform IPS, hypoplastic IPS, or significant anterior drainage of the cavernous sinus through the pterygoid plexus. 15 , 17 Although it has been known that cavernous sinus drainage patterns can affect or confound the results of IPS or cavernous sinus sampling, the algorithm we use here to identify drainage patterns to determine optimal sampling locations has not been described previously. 18 The identification of the site of primary drainage allows targeted microcatheter positioning to increase the probability of sampling with the highest possible fidelity, thus allowing more accurate lateralization.…”

“… 6 Other authors have reported success with the concept of identifying the dominant drainage pattern in a single patient, leading to successful lateralization based on external jugular vein sampling. 15 …”

“…Although there has been one report of successful external jugular vein ACTH sampling in a case of a hypoplastic IPS, there is no systematic approach available to address these cases. 15 Here we report the systematic approach and algorithm to IPSS used at the University of Cincinnati Medical Center, which combines careful identification of optimal sampling sites with previously described ACTH normalization methods. 11 , 13 , 14 Our addition to commonly used techniques is the careful identification of the site of primary drainage to determine optimal sampling sites in a systematic manner, which has not been previously described.…”