Robert S. Shapiro

Cryoablation of unresectable malignant liver tumors

BACKGROUNDnUnresectable malignant liver tumors, particularly metastases of gastrointestinal origin, are rapidly lethal in a vast majority of patients, regardless of treatment.nnnPATIENTS AND METHODSnWe evaluated 58 patients by laparoscopy and/or laparotomy. Thirty-nine were treated with cryoablation of liver tumors using a liquid-nitrogen cryoprobe delivering a tumoricidal temperature of -196 degrees C with intraoperative ultrasound monitoring. Histologic evaluation showed that 25 patients had colorectal metastases, 3 had gastric tumors, 4 hepatocellular carcinomas, 6 carcinoids, and 1 gastrinoma.nnnRESULTSnAll patients who received complete cryoablation are alive with a mean follow-up of 14 months. Five whose treatment could not be completed died between 3 and 9 months postoperatively. Thirteen patients (28%) have recurrent disease and 20 (51%) have no evidence of disease. There were no operative mortalities. Postoperative transient elevation of liver function tests and thrombocytopenia were noted. All patients received postoperative chemotherapy.nnnCONCLUSIONnCryoablation is an active and safe treatment for advanced liver malignancies.

Enzyme Augmentation in Moderate to Life- Threatening Gaucher Disease

ABSTRACT: Gaucher disease type 1 (GD type 1) is the most prevalent lysosomal storage disease and has its highest frequency in the Ashkenazi Jewish population. Deficiency of the enzyme, acid β-glucosidase, results in the deposition of glucocerebroside primarily in macrophages. The accumulation of such “Gaucher cells” leads to viscer-omegaly, hepatic and bone marrow dysfunction, hyper-splenism, and bony disease. Eleven GD type 1 patients, ages 4–52 y, with moderate to life-threatening manifestations, received 6–12 mo of enzyme augmentation with a macrophage-targeted acid β-ghicosidase preparation. Within 6 mo, substantial increases in Hb levels (mean = +30%) and platelet counts (mean = +39%) were observed. Hepatic and splenic volumes decreased by ∼20% (range = 3–35%) and ∼35% (20–52%), respectively. Hematotogic and hepatic volume improvements were similar in the splenectomized (n = 4) and nonsplenectomized (n = 7) patient groups. In this patient population, no major differences were observed in the hematologic and visceral improvements with enzyme doses of 30, 50, or 60 IU/kg administered every 2 wk. Normal levels of acid β-glucosi-dase activity were present in hepatic autopsy samples from one patient 11 d after enzyme infusion. In comparison, exogenous activity was absent from brain and lung specimens of the same patient High levels (∼10-fold normal) were present in bone marrow samples from two patients obtained at 1 and 11 d after infusions. These studies demonstrate biochemical and clinical improvements by targeted enzyme augmentation in GD type 1, even in far advanced, life-threatening involvement. These and previous studies indicate that earlier intervention in patients with more mild signs may be warranted to obviate the need to rescue extraordinarily ill patients with GD type 1.

CT diagnosis of acquired intercostal lung herniation

Intercostal lung herniation is a rare complication of trauma, best demonstrated by computed tomography. Most intercostal lung herniations are the result of direct trauma to the chest wall or occur at sites of prior percutaneously placed chest tubes. We present two cases of acquired intercostal lung herniation.

MRI of the liver a pictorial essay

Magnetic resonance imaging (MRI) is an extremely useful modality for evaluation of the complex pathophysiology of the liver. The high degree of soft tissue contrast afforded by MRI accurately detects and characterizes both focal and diffuse abnormalities of the liver. In this article we present a pictorial review of MRI of the liver.

Underassessment of deep venous thrombosis of the lower extremity on color flow doppler compared to compression sonography

MATERIALS AND METHODS: We prospectively studied 59 hips (30 patients, 18 females and 12 males; age rcegs 1 dayto 18 months) with the following clinical diagasses: posalbly subluxated (n= 18), subluxated (n= 15), dislocated (n=9), normal (n= 17). We performed simultaneously conventional and 3-1) soasgraphy. The 3-]) images were viewed in two forms of presentation: as orthogonal section analysis and as a transparent revolving spatial image. The technical quality of the studies was evaluated jointly by two of the authors with knowledge of the clinical diagnosis. Correlation was made with Convcetiasal soeography and radiographs, when available.

Intrahepatic gas formation without abscess after hepatic artery thrombosis in the setting of liver transplantation

We present a case in which intraparenchymal hepatic gas was observed in the absence of an abscess. This occurred following hepatic artery thrombosis in the setting of liver transplantation. An analogy is drawn to the postinfarction syndrome seen following therapeutic embolization and intrauterine fetal death. The unique features of the liver transplant and how they relate to the mechanisms of gas formation are discussed.

Carcinoid tumor of the lung presenting as a pleural mass : CT diagnosis

Carcinoid tumors of the lung typically arise centrally within the tracheobronchial tree. We report a rare case of a carcinoid tumor presenting as a pleural mass. The classification and X-ray features are described.