A 62-Year-Old Immunocompromised Man With Halo Sign on Chest Imaging.

CASE PRESENTATION: A 62-year-old White man with a history of orthotopic liver transplantation 16 years ago for alcoholic liver cirrhosis on chronic immunosuppression and recurrent decompensated cirrhosis of his graft liver complicated by ascites, hepatic encephalopathy, and esophageal varices presented to the hospital with altered mental status. Over the last few weeks, he had reduced frequency of bowel movements and subsequently developed altered sensorium 3 days before presentation. On arrival to the hospital, he was disoriented and had asterixis consistent with hepatic encephalopathy. He was not in respiratory distress, he was saturating well on room air, and his lungs were clear to auscultation bilaterally. Plain chest radiograph showed multiple ill-defined bilateral airspace opacities. A CT scan of the abdomen and pelvis done on admission incidentally showed bilateral pulmonary nodules with surrounding ground-glass halo in the lower lung zones. Given these findings, a dedicated CT scan of his chest was performed that showed numerous bilateral randomly distributed nodular airspace opacities, many with a central solid component and surrounding ground-glass halo. Antifungal therapy was initiated empirically. Serum aspergillus antigen and 1,3 beta D-glucan were negative. He subsequently underwent a bronchoscopy with BAL and transbronchial biopsy. BAL fluid was negative for bacterial, fungal, and acid-fast bacilli cultures. Pathology from the transbronchial biopsy showed atypical epithelioid cells in intravascular spaces.

A Comparison of Long-Term Treatment Outcomes Between Giant and Nongiant Craniopharyngiomas.

BACKGROUND: There is limited literature on outcomes after surgical treatment of giant craniopharyngiomas in adult and pediatric patients. METHODS: A retrospective review of 159 patients undergoing surgery for craniopharyngiomas at a single institution was performed. Patients with giant craniopharyngiomas (maximum dimension ≥4.5 cm) were compared with nongiant tumors in terms of various clinical and radiological parameters and long-term surgical outcomes. Extent of resection was determined by postoperative magnetic resonance imaging. Factors associated with post-treatment obesity were also analyzed. RESULTS: Giant craniopharyngiomas (n = 66) were characterized by higher rates of childhood presentation, visual impairment, neurological deficits, multicompartmental involvement, and hydrocephalus as compared with nongiant tumors (n = 139). Giant tumors also were less likely to undergo transsphenoidal resection and were associated with a higher rate of postoperative neurological morbidity. There were no significant differences between the 2 groups in terms of extent of resection, use of postoperative radiation therapy, and long-term endocrinological outcomes. Overall recurrence rates over a mean follow-up period of 4.1 years were similar between giant and nongiant tumors; however, recurrences after presumed gross total resection/near total resection were significantly higher in the former subgroup versus the latter (39.4% vs. 18.4%; P = 0.044). Risk factors for post-treatment obesity in giant craniopharyngiomas included adult age (P = 0.001), preoperative obesity (P = 0.003), and hypothalamic involvement (P = 0.012). CONCLUSION: Gross total resection/near total resection of giant craniopharyngiomas can be achieved at rates comparable to nongiant tumors. However, there remains a greater risk of postoperative neurological morbidity. Radiation therapy mitigates the risk of recurrence on long-term follow-up.

Long-term outcomes following maximal safe resection in a contemporary series of childhood craniopharyngiomas.

BACKGROUND: The optimal management of pediatric craniopharyngiomas remains controversial. This study aimed to characterize long-term outcomes in a contemporary cohort of children undergoing surgery for craniopharyngiomas. METHODS: This was a retrospective review of 37 consecutive children who underwent surgery for craniopharyngioma with a median follow-up duration of 79 months (range 5-127 months). Patients were stratified by extent of resection (EOR) and need for adjuvant radiation therapy (RT). Imaging studies were reviewed to grade extent of hypothalamic involvement. Data on functional outcomes, pituitary function, and obesity were analyzed. RESULTS: Gross total resection was achieved in 16 patients (43.2%), near total resection in six patients (16.2%), and subtotal resection (STR) in 15 patients (40.5%). The recurrence-free survival rate was 81.1% and 70.3% at 5- and 10-year follow-up, respectively. Survival analysis showed superior disease control in patients undergoing STR + RT (p = 0.008). Functional outcomes were independent of EOR, postoperative RT or recurrence. Diabetes insipidus was present in 75% and 44.4% of patients required >2 hormone replacements at last follow-up. Obesity was present in 36.1% patients after treatment, and was associated with preoperative obesity (p = 0.019), preoperative hypothalamic involvement (p = 0.047) and STR + RT (p = 0.011). CONCLUSIONS: Gross or near total resection may be achieved safely in almost 60% of cases; however, radical surgery does not eliminate the risk of recurrence. Over long-term follow-up, STR + RT offers the best disease control rates. Patients with preoperative hypothalamic involvement, obesity, and those with tumors not amenable to radical resection are at risk for developing obesity on long-term follow-up.

Selective perioperative steroid supplementation protocol in patients undergoing endoscopic transsphenoidal surgery for pituitary adenomas.

BACKGROUND: There is no consensus regarding the use of perioperative steroids for transsphenoidal pituitary surgery. We audited the effectiveness and safety of our selective perioperative steroid supplementation protocol in patients with pituitary adenomas. METHODS: Two hundred ninety-seven patients underwent 306 endoscopic transsphenoidal surgeries for removal of their pituitary tumors. Steroids were given to those with an impaired hypothalamic-pituitary-adrenal (HPA) axis, age ≥ 60 years, clinical apoplexy, hyponatremia, or if the pituitary gland was not preserved at surgery. We excluded 111 patients in whom the integrity of the HPA axis could not be determined. We compared the incidence of early postoperative adrenal insufficiency and complications in 135 patients with intact HPA axes who underwent surgery without steroids (group A) with 60 patients who had compromised preoperative HPA axes and received perioperative steroids (group B). In addition, we audited the total number of protocol violations during this period. RESULTS: Five patients (3.7%) in group A developed postoperative hypocortisolemia. There was no significant difference in the incidence of cerebrospinal fluid leak, diabetes insipidus, or hyponatremia between both groups. There were protocol deviations in 47 (15.4%) patients. Twenty one of these patients did not receive perioperative steroids in violation of the protocol, of whom 4 (19%) developed postoperative hypocortisolemia. CONCLUSIONS: Our steroid sparing protocol was both safe and effective. The 15% incidence of protocol deviations is a reminder that the rigorous usage of checklists is mandatory for successful clinical practice.