Cystic versus non-cystic silent corticotrophic adenomas: clinical and histological analysis of 62 cases after microscopic transsphenoidal surgery-a retrospective, single-center study.

Silent corticotrophic adenomas (SCAs) represent a rare group of non-functioning adenomas with a potentially aggressive clinical course. Cystic component is a very common finding among SCAs, but its clinical relevance has not yet been investigated. The aim of this study was to analyze clinical features of cystic and non-cystic SCAs, perioperative complications after microscopic transsphenoidal surgery, clinical outcome after first and repeat surgery along with risk factors for recurrence. We conducted a retrospective analysis of 62 silent corticotrophic adenomas treated at our university medical center via microscopic transsphenoidal surgery between January 2008 and July 2019. Parameters investigated included histology, invasiveness, intratumoral haemorrhage or cystic component on MRI, perioperative alteration of visual field, tumor size, pre- and postoperative ACTH, FSH, GH, LH, TSH, prolactin, cortisol, free T4, free T3, IGF-1, estrogen and testosterone levels, perioperative complications, neoadjuvant and adjuvant therapy along with clinical outcomes. A total of 62 patients were analyzed. The mean follow up was 28.3 months. Tumors with a cystic component occur statistically significant more often among male than non-cystic (80.6% vs. 44.4%, p = 0.02) and display lower rates of cavernous sinus invasion and sphenoid sinus invasion were significantly lower for cystic lesions comparing to non-cystic tumors (42.3% vs. 69.4%, p = 0.04 and 3.8% vs. 47.2%, p < 0.001). GTR after MTS was not statistically significant higher by cystic SCAs (80% vs. 57.1%, p = 0.09). Cystic lesions were also associated with higher risk of hyperprolactinemia (19.4% vs. 2.8%, p = 0.02) and only densely granulated cystic SCAs presented with preoperative intratumoral hemorrhage (19.2% vs. 0%, p = 0.01). Mean duration of first surgery was significantly shorter for cystic SCAs (71.6(± 18.7) vs. 94.8(± 31.1) minutes, p = 0.01). Preoperative pituitary insufficiency (25% vs. 16.7%, p = 0.49), intraoperative CSF space opening (21.1% vs. 37.5%, p = 0.32), along with postoperative new pituitary insufficiency (15% vs. 10%, p = 0.67) or diabetes insipidus/SIADH (10% vs. 13.3%, p > 0.99) with histological markers such as Ki67 (21.1% vs. 13.8%, p = 0.70) and p53 expression (6.3% vs. 0%, p = 0.39) as well as mitotic rate (5.3% vs. 10.3%, p > 0.99) were comparable between both groups. The presence of cystic component did not affect the tumor recurrence (10% vs. 16%, p = 0.68). Mean duration of surgery was first surgeries was not statistically shorter than repeat surgeries (85.4 ± 29.1 vs. 93.8 ± 28 min, p = 0.15). Patients undergoing first surgery had a higher probability of gross total resection (74.4% vs. 30%, p = 0.01) and lower probability of intraoperative CSF space opening (26% vs. 58.3%, p = 0.04) as well as a lower rate of preoperative anterior pituitary insufficiency (20% vs. 58.3%, p = 0.01). The incidence of new postoperative anterior pituitary insufficiency (10% vs. 0%, p = 0.57) and transient diabetes insipidus/SIADH (12% vs. 8.3%, p > 0.99) between those groups were comparable. No statistical difference was observed between patients with remission and with recurrent tumor regarding cortisol and ACTH levels, incidence of different histological subgroups, invasively growing tumors and lesions with cystic components as well as the percentage of cases with increased Ki67 proliferation index, p53 expression and mitotic indices. Our study presents one of the largest available cohorts of SCAs after microscopic transsphenoidal surgery and first clinical analysis of cystic versus non-cystic SCAs so far. We also performed the first comparison of index and repeat surgeries for this tumor entity. Cystic tumors presented with characteristic clinical aspects like male predominance, higher risk of hyperprolactinemia as well as lower rates of cavernous sinus and sphenoid sinus invasion comparing to non-cystic lesions. Mean duration of first surgery was significantly shorter for cystic SCAs. Moreover preoperative intratumoral hemorrhage had 100% specificity and 60% sensitivity for densely granulated cystic SCAs. All these clinical hallmarks may suggest a novel subgroup of SCAs with distinct clinical and biological features, however further clinical and molecular investigations are required. Second surgeries are associated with a higher incidence of preoperative pituitary insufficiency, and a higher risk of subtotal resection, and a higher probability of CSF space opening intraoperatively compared to first surgeries. On the other hand, the risk of new postoperative pituitary insufficiency was higher after first surgeries. In our cohort of patients, no prognostic factor for recurrence among histological diagnosis, Ki67-proliferation index, p53 expression, number of mitoses, invasive growth or cystic lesions for SCAs could be detected.

Localization and Likelihood of Chondral and Osteochondral Lesions After Patellar Dislocation in Surgically Treated Children and Adolescents.

Background: Patellar dislocation is a common injury in children and adolescents. There is a lack of information about concomitant osteochondral lesions in these patients. Hypothesis: We hypothesized that the likelihood of chondral or osteochondral lesions would increase with each prior patellar dislocation and that the appearance of lesions in surgically treated children and adolescents would be influenced by age, sex, injury mechanism, and body mass index (BMI). Study Design: Case-control study; Level of evidence, 3. Methods: Inclusion criteria were (1) age <18 years, (2) surgical treatment with diagnostic arthroscopy of the knee including description of chondral and osteochondral lesions, and (3) maximum time period between the last patellar dislocation and presentation in our department of 6 weeks and maximum of 4 weeks between presentation and surgery. A total of 153 children (173 knees) with a mean age of 13.0 ± 2.1 years were included. All surgical reports and intraoperative arthroscopic imaging were analyzed retrospectively for the location and severity of lesions. The likelihood of lesions was calculated with multiple stepwise regression models regarding injury mechanism, number of dislocations, sex, age, physeal closure, and BMI. Results: The regression model to predict femoral lesions was statistically significant (χ2[5] = 26.55; P < .001) and identified male sex, BMI ≥25, traumatic injury mechanism, and physeal closure as independent factors associated with the appearance of femoral lesions. The second regression model predicting the appearance of patellar lesions was also statistically significant (χ2[4] = 26.07; P < .001) and identified the traumatic injury mechanism as a single independent predictor for patellar lesions. Conclusion: BMI ≥25, male sex, and physeal closure were factors significantly associated with femoral chondral and osteochondral lesions in our cohort. In case of traumatic lateral patellar dislocation (LPD), the likelihood of patellar and femoral chondral and osteochondral lesions is significantly higher than in nontraumatic LPD. Early magnetic resonance imaging and surgical treatment should be considered in obese patients, male patients, and patients with physeal closure and after traumatic LPD.

Management of spinal deformities and tibial pseudarthrosis in children with neurofibromatosis type 1 (NF-1).

The skeletal system is affected in up to 60% of patients with neurofibromatosis type 1. The most commonly observed entities are spinal deformities and tibial dysplasia. Early recognition of radiologic osseous dystrophy signs is of utmost importance because worsening of the deformities without treatment is commonly observed and surgical intervention is often necessary. Due to the relative rarity and the heterogenic presentation of the disease, evidence regarding the best surgical strategy is still lacking. PURPOSE: To report our experience with the treatment of skeletal manifestations in pediatric patients with (neurofibromatosis type 1) NF-1 and to present the results with our treatment protocols. MATERIALS AND METHODS: This is a retrospective, single expert center study on children with spinal deformities and tibial dysplasia associated with NF-1 treated between 2006 and 2020 in a tertiary referral institution. RESULTS: Spinal deformity: Thirty-three patients (n = 33) were included. Mean age at index surgery was 9.8 years. In 30 patients (91%), the deformity was localized in the thoracic and/or lumbar spine, and in 3 patients (9%), there was isolated involvement of the cervical spine. Eleven patients (33%) received definitive spinal fusion as an index procedure and 22 (67%) were treated by means of "growth-preserving" spinal surgery. Halo-gravity traction before index surgery was applied in 11 patients (33%). Progression of deformity was stopped in all patients and a mean curve correction of 60% (range 23-98%) was achieved. Mechanical problems with instrumentation requiring revision surgery were observed in 55% of the patients treated by growth-preserving techniques and in none of the patients treated by definitive fusion. One patient (3%) developed a late incomplete paraplegia due to a progressive kyphotic deformity. Tibial dysplasia: The study group comprised of 14 patients. In 5 of them (36%) pathological fractures were present on initial presentation. In the remaining 9 patients (64%), anterior tibial bowing without fracture was observed initially. Four of them (n = 4, 28%) subsequently developed a pathologic fracture despite brace treatment. Surgical treatment was indicated in 89% of the children with pathological fractures. This involved resection of the pseudarthrosis, autologous bone grafting, and intramedullary nailing combined with external fixation in some of the cases. In 50% of the patients, bone morphogenic protein was used "off-label" in order to promote union. Healing of the pseudarthrosis was achieved in all of the cases and occurred between 5 to 13 months after the index surgical intervention. Four of the patients treated surgically needed more than one surgical intervention in order to achieve union; one patient had a re-fracture. All patients had a good functional result at last follow-up. CONCLUSION: Early surgical intervention is recommended for the treatment dystrophic spinal deformity in children with NF-1. Good and sustainable curve correction without relevant thoracic growth inhibition can be achieved with growth-preserving techniques alone or in combination with short spinal fusion at the apex of the curve. Preoperative halo-gravity traction is a safe and very effective tool for the correction of severe and rigid deformity in order to avoid neurologic injury. Fracture union in tibial dysplasia with satisfactory functional results can be obtained in over 80% of the children by means of surgical resection of the pseudarthrosis, intramedullary nailing, and bone grafting. Wearing a brace until skeletal maturity is achieved is mandatory in order to minimize the risk of re-fracture.

Pituitary adenomas in children and young adults.

PURPOSE: Pituitary adenomas are rare in the pediatric population. We present a recent cohort of children operated transsphenoidally on pituitary adenoma from a single center. METHODS: Inclusion criteria were age < 21 years and histopathological diagnosis of adenoma after transsphenoidal surgery. The electronic file was used for prospective and retrospective data collection on symptoms, pituitary function before/after surgical intervention, and surgical complications. Surgical reports were used for assessment of resection grade. Follow-up data were collected from outpatient clinical visit and/or correspondence concerning pituitary function and MRI. RESULTS: Among 962 consecutive patients who underwent transsphenoidal surgery at the University Medical Center Hamburg-Eppendorf (Germany) between April 2013-December 2016, 50 patients (5.2%) were 21 years old or younger (range 6-21 years, mean 16.5). Twenty-five (50%) patients had pituitary adenomas; M:F = 13:12. Eight were macroadenomas (> 1cm3). Time from onset of symptoms to first operation had a range of 0-48 months (mean 18.5 months). Nine (36%) patients overall complained of headaches preoperatively. One patient had objective (hemianopsia) and four subjective visual disturbances preoperatively. Histopathological diagnosis revealed 13 (52%) ACTH adenomas, 5 (20%) prolactinomas, 3 (12%) mixed GH-prolactinoma, 1 (4%) GH adenoma, 2 (8%) TSH-producing adenomas, and 1 (4%) non-functioning adenoma. There were no surgical complications. On clinical follow-up (range 1-34.5 months, mean 11.7 months), 21/24 (88%) patients with hormonally active tumors were in biochemical remission by surgery alone; 2 patients with Cushing disease and 1 with GH-hypersecretion required further treatment. CONCLUSIONS: Our recent series confirms the effectiveness and safety of transsphenoidal surgery, especially in the pediatric patient population.

Intradural lumbar disc herniation associated with degenerative spine disease and rheumatoid arthritis.

STUDY DESIGN: A case report. OBJECTIVE: To demonstrate a case of intradural lumbar disc herniation including imaging studies, intraoperative imaging, and an intraoperative video. SUMMARY OF BACKGROUND DATA: The first case of lumbar intradural disc herniation was reported as early as 1942; since then more than 150 cases have been reported, mostly in the lumbar spine. Gadolinium-enhanced magnetic resonance image (MRI) is considered the "gold standard" for diagnosing this entity, although it is rarely performed routinely in lumbar disc disease and diagnosis is often made intraoperatively. METHODS: A 70-year-old man presented to the emergency department as a referral complaining of lower back pain, loss of sensation in the right thigh, and difficulty walking. On examination, he showed uneven gait, right-sided foot drop (1/5), hypesthesias in the right inguinal area and ventral thigh, and a positive straight leg raise test on the right. Anal sphincter tone was within normal limits. A magnetic resonance image of the lumbar spine showed a large mediolateral herniated disc at L3-L4, with caudal displacement and unclear signal changes intradurally. RESULTS: Intraoperatively, the herniated disc was found upon opening the dural sac. CONCLUSION: Intradural disc herniations are a rare entity. The opening and inspection of the dural sack should be considered when the correct spinal level can be confirmed and insufficient herniated disc material can be visualized extradurally.