Richard Chang

Hepatic abscess in patients with chronic granulomatous disease.

ObjectiveTo evaluate the clinical presentation, diagnostic procedures, and surgical management of hepatic abscesses in patients with chronic granulomatous disease (CGD). Summary Background DataChronic granulomatous disease is a rare inherited primary immunodeficiency in which phagocytes cannot destroy catalase-positive bacteria and fungi. Defects in the phagocytic cells’ respiratory burst lead to life-threatening infections, including hepatic abscess. These abscesses are recurrent and often multiple and are treated differently from bacterial abscesses in patients without CGD. MethodsBetween 1980 and 2000, 61 cases of hepatic abscess in 22 patients with CGD were treated at the National Institutes of Health. Clinicopathologic features were investigated by retrospective review of the medical records, radiographs, and histopathology. ResultsTwelve of the 61 cases were primary hepatic abscesses. Twenty-nine of the cases were recurrent hepatic abscesses, and 20 cases were persistent hepatic abscesses. The median age at the time of initial hepatic abscess presentation was 14 years. Subjective fever was the most frequent presenting symptom, and the erythrocyte sedimentation rate was elevated in 98% of cases. Fifty-two cases were managed surgically and eight cases were managed with percutaneous drainage. One patient refused surgery. The surgical complication rate was 56%; however, there were no deaths directly related to the hepatic abscesses. Staphylococcus aureus was the most frequent organism identified in culture (88% of positive cultures). Aggressive surgery and antibiotics ultimately resulted in successful treatment of all patients. ConclusionsHepatic abscesses occurring in patients with CGD represent a difficult diagnostic and treatment challenge. Early excision and treatment with antibiotics directed against S. aureus is necessary. General surgeons should be aware of this rare immunodeficiency and should aggressively manage hepatic abscesses in these patients.

A prospective analysis of imatinib-induced c-KIT modulation in ovarian cancer: a phase II clinical study with proteomic profiling.

c‐Kit and platelet‐derived growth factor receptor (PDGFR) are potential molecular targets in epithelial ovarian cancer (EOC). Imatinib inhibits the kinase domain and subsequent downstream signaling of these receptor tyrosine kinases. The objective of this study was to investigate biochemical and biologic effects of imatinib on EOC.

Radiologic screening for von Hippel-Lindau disease: the role of Gd-DTPA enhanced MR imaging of the CNS.

Thirty-seven members of a family with von Hippel-Lindau disease (VHL) were prospectively screen for CNS hemangioblastomas; 10 family members were previously known to have VHL. Radiographic studies included noncontrast magnetic resonance (MR) imaging of the head (all patients) and spine (34 patients) and contrast-enhanced CT (CCT) of the head (all adult patients). Eleven patients had Gd-DTPA enhanced MR (CMR) of the head and 10 patients had CMR of the spine. Sixteen patients had radiographic evidence of CNS hemangioblastomas and all but six patients were symptomatic. Using comparable studies, CMR of the head demonstrated more lesions than the other modalities (31, 22, and 19 for CMR, MR, and CCT, respectively). Furthermore, CMR better separated tumor from edema, as well as cystic from solid components. Contrast enhanced MR was superior to noncontrast MR of the spine in lesion detection (31 vs. 4; p less than 0.001). Noncontrast MR was particularly limited in four patients with syringomyelia. We conclude that postcontrast MR of the head and spine is the best currently available means of detecting hemangioblastomas associated with VHL.

Pulse-Spray Treatment of Subclavian and Jugular Venous Thrombi with Recombinant Tissue Plasminogen Activator☆

PURPOSE To evaluate the efficacy of recombinant tissue plasminogen activator (rtPA) injected by pulse-spray in lysing subclavian and jugular venous thrombi. MATERIALS AND METHODS Twelve patients with symptomatic, venogram-confirmed, occlusive thrombi of the subclavian-axillary or jugular veins were treated with one or two daily 15-minute injections of rtPA delivered directly into the clots with pulse-spray catheters. Twenty-four hours after each treatment, repeated venograms were obtained to assess venous patency. Successful thrombolysis was defined as antegrade flow through the previously occluded segments with minimal collateral venous flow. Continued patency was assessed with repeated venograms obtained after 1 and 2 months of oral anticoagulation. RESULTS The 15-minute rtPA injections successfully lysed thrombi in eight of the 12 patients. Hypofibrinogenemia developed in only one patient. The technique had a high success rate with thrombi less than 2 weeks old (seven of eight) regardless of the length of the clot, but had limited success with thrombi more than 2 weeks old (one of four). Continued patency over a 2-month interval was documented in four of the eight patients whose thrombi were successfully lysed. However, patency could be maintained in only one of the four patients who retained a venous access device in the treated vein. CONCLUSION Pulse spray rtPA is an effective, safe, and practical alternative to continuous infusions of thrombolytic agents to treat upper extremity venous thrombi.

Consequences of Adrenal Venous Sampling in Primary Hyperaldosteronism and Predictors of Unilateral Adrenal Disease

BACKGROUND In patients with primary hyperaldosteronism, distinguishing between unilateral and bilateral adrenal hypersecretion is critical in assessing treatment options. Adrenal venous sampling (AVS) has been advocated by some to be the gold standard for localization of the responsible lesion, but there remains a lack of consensus for the criteria and the standardization of technique. STUDY DESIGN We performed a retrospective study of 114 patients with a biochemical diagnosis of primary hyperaldosteronism who all underwent CT scan and AVS before and after corticotropin (ACTH) stimulation. Univariate and multivariate analyses were performed to determine what factors were associated with AVS lateralization, and which AVS values were the most accurate criteria for lateralization. RESULTS Eighty-five patients underwent surgery at our institution for unilateral hyperaldosteronism. Of the 57 patients who demonstrated unilateral abnormalities on CT, AVS localized to the contralateral side in 5 patients and revealed bilateral hyperplasia in 6 patients. Of the 52 patients who showed bilateral disease on CT scan, 43 lateralized with AVS. The most accurate criterion on AVS for lateralization was the post-ACTH stimulation value. Factors associated with AVS lateralization included a low renin value, high plasma aldosterone-to plasma-renin ratio, and adrenal mass > or = 3 cm on CT scan. CONCLUSIONS Because 50% of patients would have been inappropriately managed based on CT scan findings, patients with biochemical evidence of primary hyperaldosteronism and considering adrenalectomy should have AVS. The most accurate measurement for AVS lateralization was the post-ACTH stimulation value. Although several factors predict successful AVS lateralization, none are accurate enough to perform AVS selectively.

Reoperation for parathyroid adenoma: a contemporary experience.

BACKGROUND We reviewed reoperations for persistent or recurrent sporadic parathyroid adenoma to evaluate and compare our current results and outcomes to our previous experience. METHODS From 1996 to 2008, 237 patients with persistent or recurrent hyperparathyroidism after failed operation underwent reoperation. Patients were re-explored with the assistance of non-invasive and sometimes invasive imaging. RESULTS A missed adenoma was suspected pre-operatively in 163 patients. Reoperation resulted in long-term resolution of hypercalcemia in 92%. Adenomas were in entopic locations in 32%; the most frequent ectopic location was the thymus (20%). Sestamibi scanning and ultrasonography were the most successful non-invasive imaging studies (96% positive predictive value (PPV) and 84% PPV respectively). Forty-four percent of patients had a reoperation based solely on non-invasive imaging. Of the invasive procedures performed, arteriography resulted in the best localization (92% PPV). Permanent recurrent laryngeal nerve injury occurred in 1.8%. CONCLUSION Compared to our prior experience (1982-1995), outcomes remained similar (92% resolution of hypercalcemia and 1.8% recurrent nerve injury currently versus 96% and 1.3% previously). Fewer patients received invasive studies for pre-operative localization (56% vs 73%, respectively). The decreased use of invasive imaging is due to technical improvements and greater confidence in the combination of ultrasonography and sestamibi scanning.

Detection of insulinoma using 68 Gallium-DOTATATE PET/CT: a case report

Insulinomas are the most common cause of endogenous hyperinsulinemic hypoglycemia in non-diabetic adults. They are most often benign, small and difficult to localize with current imaging techniques. This is of high importance, as complete surgical resection is the only curative treatment. Anatomic imaging, (111)In-pentetreotide scan and (68)Gallium-DOTATATE positron emission tomography/computed tomography (PET/CT) were compared in a patient with insulinoma. (68)Gallium-DOTATATE PET/CT and selective arterial calcium stimulation localized the insulinoma. At surgery, a tumor in the anterior aspect of the pancreatic body was found which confirmed the preoperative localization, and a 2.1 cm tumor was enucleated, World Health Organization (WHO) grade I insulinoma. The patient remains euglycemic and free of symptoms at last follow up. In conclusion, (68)Gallium-DOTATATE PET/CT imaging may be a useful adjunct localizing study for insulinomas. It is a non-invasive preoperative localization study that could guide surgical exploration for successful therapy.

Management of primary aldosteronism in patients with adrenal hemorrhage following adrenal vein sampling: A brief review with illustrative cases

The authors describe the clinical investigation of two cases of primary aldosteronism with adrenal hemorrhage (AH) following adrenal vein sampling. A literature review was conducted regarding the medical management of primary aldosteronism in patients with AH following adrenal vein sampling. Guidelines on the management of primary aldosteronism with AH following adrenal vein sampling are lacking. The two patients were followed with serial imaging to document resolution of AH and treated medically with excellent blood pressure response. Resolution of AH was achieved, but a repeat adrenal vein sampling was deferred given the increased morbidity risk associated with a repeat procedure.