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Biomedical subjects

Steven J Fishman

Publications and source records attributed to Steven J Fishman.

At least 19 recordsLinked to original sources
PubMed2007

Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry.

PURPOSE: Hepatic hemangiomas, though histologically benign, may be associated with significant morbidity and mortality in afflicted infants. The literature presents much confusion regarding the natural history and treatment options for hepatic hemangiomas. Clinical manifestations range from asymptomatic self-limiting lesions to congestive heart failure associated with high-volume vascular shunting to fulminant hepatic failure with hypothyroidism, abdominal compartment syndrome, and death. There has been little rationale to choose among observation, corticosteroid, other pharmacologic agents, arterial embolization, hepatic artery ligation, resection, or liver transplantation for any given patient. METHODS: We analyzed several recent retrospective radiologic analyses and pathologic studies to determine whether hepatic hemangiomas could be categorized, allowing prediction of their natural history and rational choice of therapies based upon their clinical presentation and radiographic appearance. RESULTS: We propose that hepatic hemangiomas do not represent a single entity but, rather, 3 principle categories of lesions: focal, multifocal, and diffuse. Because these 2 categories represent different anatomical and physiologic variants, so, too, may they respond differently to previously anecdotally applied treatment regimens. With input from international multidisciplinary authorities on hemangiomas, we developed and proposed a clinical practice algorithm for the evaluation and management of hepatic hemangiomas. Toward that end, we propose a plan to institute a web-based international hepatic hemangioma registry. Participants in the registry could obtain no-cost centralized review of imaging studies (and histology if available) and guidance regarding the management algorithm from an established multidisciplinary team. In exchange, the registry will facilitate the acquisition of systematic clinical and imaging information. CONCLUSION: Longitudinal observation of response to more directed treatment protocols may contribute greatly to the understanding of these potentially fatal tumors.

Algorithms↗
PubMed2006

Complex pancreatic vascular anomalies in children.

BACKGROUND: Vascular anomalies are vascular tumors and congenital malformations that rarely involve the pancreas. Diagnosis and management of these lesions is complex. METHODS: An institutional review board-approved retrospective database and record review from 1994 through 2004 at a quaternary referral center for vascular anomalies was conducted. RESULTS: Of 5051 patients with a vascular anomaly, 6 had a lesion involving the pancreas. All patients were younger than 3 years. There were three tumors (two infantile hemangiomas and one Kaposiform hemangioendothelioma) and three malformations (two lymphatic and one venous). The referring diagnoses were correct for 4 patients. All anomalies were diagnosed with a combination of cross-sectional imaging and angiography. Five patients received medical therapy including two malformation patients who were initially treated with antiangiogenic drugs. Two patients with lymphatic malformations underwent operative intervention. One infantile hemangioma was treated with percutaneous biliary drainage for obstructive jaundice. CONCLUSION: Complex pancreatic vascular anomalies are rare. Vascular tumors are generally amenable to treatment with antiangiogenic medications. Operative resection is reserved for refractory cases or complications. Symptomatic vascular malformations are treated exclusively by surgery. Evaluation and management of these lesions is complex and may benefit from coordinated interdisciplinary care.

Angiogenesis Inhibitors↗
PubMed2006

Vascular anomalies of the female external genitalia.

BACKGROUND/PURPOSE: Vascular tumors and malformations are rare. This large series describes vascular anomalies of the female genitalia and their management. METHODS: An international review board-approved retrospective database and a record review from 1994 through 2004 was conducted. RESULTS: Of the 3186 female patients with a vascular anomaly, 82 (2.6%) had a lesion in the external genitalia. There were 60 malformations and 22 tumors. The most common malformations were combined capillary-lymphaticovenous, venous, and lymphatic. Tumors included 20 infantile hemangiomas, 1 kaposiform hemangioendothelioma, and 1 kaposiform lymphangioendothelioma. The referring diagnosis was incorrect in 56% of patients. Cutaneous stains, swelling, deformity, bleeding, fluid leakage, or infection were the prominent symptoms. Magnetic resonance imaging, ultrasonography, angiography, and computed tomography were used for diagnostic clarification. Malformation treatment consisted of sclerotherapy, embolization, and operative resection. Tumor management included observation, surgical excision, and antiangiogenic pharmacotherapy. CONCLUSIONS: Vascular anomalies of the female external genitalia are uncommon, and the initial diagnosis is often inaccurate. Correct diagnosis using clinical and radiographic data is feasible and leads to meaningful intervention for these frequently devastating lesions. Whereas tumors may respond to excision or antiangiogenic drugs, malformations require ablation or resection. Evaluation and management of these lesions is complex and benefits from interdisciplinary care.

Blood Vessels↗
PubMed2006

Hydrostatic stretch-induced growth facilitating primary anastomosis in long-gap esophageal atresia.

We present a novel simple technique for stimulating distal esophageal pouch growth in a patient with isolated, long-gap esophageal atresia. In this case report, distal esophageal growth was achieved through daily intermittent pressurization via a surgically placed indwelling balloon catheter. The patient ultimately underwent successful, uncomplicated, tension-free, primary repair and remains asymptomatic.

Anastomosis, Surgical↗
PubMed2006

Extracellular matrix dynamics associated with tissue-engineered intravascular sclerotherapy.

BACKGROUND: The extracellular dynamics after intravascular sclerotherapy with an injectable, fibroblast-based engineered construct is unknown. METHODS: Rabbits underwent ethanol sclerotherapy of a jugular vein segment. Control animals (n = 40) underwent no further treatment or an acellular collagen hydrogel was injected. Experimental animals (n = 20) received a tissue-engineered construct. After 1, 2, 4, and 20 to 24 weeks, segments were evaluated for collagen, glycosaminoglycan (GAG), matrix metalloproteinase (MMP) 2 and 9, and tissue inhibitors of MMP (TIMPs) 1 and 2 and scored on a scale of 0 to 3. Groups and time points were compared using nonparametric statistical analysis. RESULTS: Collagen content was higher in animals that received fibroblasts (P < .05). Glycosaminoglycan analysis showed a higher grade only at 1 week (P < .05). Collagen and GAG deposition were prominent at weeks 1 through 4, and decreased over time. Both MMP-2 and MMP-9 and TIMP-1 and TIMP-2 grade decreased with time (P < .01) in all groups, with no differences between groups. CONCLUSION: Enhancement of intravascular sclerotherapy by tissue engineering stems, at least in part, from increased local deposition of collagen and GAG. MMP and TIMPs may play a role in recanalization after experimental sclerotherapy. Tissue engineering may be a valuable adjunct for the treatment of vascular malformations.

Animals↗
PubMed2005

Evidence by molecular profiling for a placental origin of infantile hemangioma.

The origin of the pathogenic endothelial cells in common infantile hemangioma is unknown. We show here that the transcriptomes of human placenta and infantile hemangioma are sufficiently similar to suggest a placental origin for this tumor, expanding on recent immunophenotypical studies that have suggested this possibility [North, P. E., et al. (2001) Arch. Dermatol. 137, 559-570]. The transcriptomes of placenta, hemangioma, and eight normal and diseased tissues were compared by hierarchical and nonhierarchical clustering analysis of >7,800 genes. We found that the level of transcriptome similarity between placenta and hemangioma exceeded that of any other tissue compared and paralleled that observed between a given tissue and its derived tumor, such as normal and cancerous lung. The degree of similarity was even greater when a subset of endothelial cell-specific genes was analyzed. Genes preferentially expressed in both placenta and hemangiomas were identified, including 17-beta hydroxysteroid dehydrogenase type 2 and tissue factor pathway inhibitor 2. These data demonstrate the value of global molecular profiling of tissues as a tool for hypothesis-driven research. Furthermore, it suggests that the unique self-limited growth of infantile hemangioma may, in fact, mirror the lifetime of placental endothelium.

17-Hydroxysteroid Dehydrogenases↗
PubMed2005

Cutaneovisceral angiomatosis with thrombocytopenia.

We describe 10 children with multiple vascular lesions of the skin and gastrointestinal tract associated with sustained, minor thrombocytopenia. In some children, there was involvement of the lung (n = 5), bone (n = 2), liver (n = 1), spleen (n = 1), and muscle (n = 1). The cutaneous lesions were congenital, multifocal, discrete, red-brown and variably blue macules and papules; in 3 children, a large dominant plaque was also present. All children developed hematemesis and/or melena and endoscopic evaluation revealed several to numerous small mucosal lesions that involved all levels of the gastrointestinal tract. Three of 5 children with pulmonary nodules had cough and 1 also had hemoptysis. Biopsies of cutaneous, gastrointestinal, and pulmonary lesions showed thin-walled, blood-filled vascular channels and variable endothelial hyperplasia. The endothelial nuclei were elongated, round, crescentic, or hobnailed. Cytoplasmic and extracellular periodic acid-Schiff positive deposits were often present in the zones of endothelial hyperplasia. The platelets were small in some children, suggesting a primary defect, possibly accounting for the thrombocytopenia. Gastrointestinal hemorrhage and hemoptysis required antiangiogenic therapy. The constellation of findings defines a congenital proliferative disorder of blood vessels with a distinctive microscopic appearance. We have termed this relatively indolent or slowly progressive disorder cutaneovisceral angiomatosis with thrombocytopenia because this designation incorporates its major clinical and histopathologic features.

Angiogenesis Inhibitors↗
PubMed2005

Fetal surgery in otolaryngology: a new era in the diagnosis and management of fetal airway obstruction because of advances in prenatal imaging.

OBJECTIVE: To evaluate the efficacy, safety, and outcome of prenatal imaging and fetal surgery in the diagnosis and management of fetal airway obstruction caused by cervical teratoma or lymphatic malformation. SETTING: Tertiary care medical center. Patients A retrospective study of all consecutive fetal patients with cervical teratoma or lymphatic malformation between January 2001 and December 2003. RESULTS: The indication was potential airway obstruction due to a fetal neck mass in 8 patients. Prenatal images were obtained by ultrasonography and magnetic resonance imaging, and were consistent with teratoma in 4 patients. The mean cervical mass was 8.3 x 7.3 x 6.7 cm, with airway obstruction suspected in all 4 patients. All 4 patients were successfully delivered by ex utero intrapartum treatment, during which 3 newborns required tracheotomy and 1 was successfully intubated. Prenatal images were consistent with lymphatic malformation in the remaining 4 patients. The mean cervical mass was 4.6 x 4.4 x 3.4 cm. There was no indication of airway obstruction based on prenatal images. All 4 patients had an uncomplicated vaginal delivery. CONCLUSIONS: Technological advances in prenatal ultrasonography and magnetic resonance imaging have improved the ability to diagnose congenital abnormalities in utero. This allows for proper assessment of the airway to prevent any unexpected problems at delivery. We believe that many airway emergencies can be avoided by prenatal imaging and initiation of airway management in the prenatal period.

Adult↗
PubMed2005

Mesogonadal shunts for extrahepatic portal vein thrombosis and variceal hemorrhage.

Extrahepatic portal vein thrombosis (EHPVT) may occur in children or adults and usually comes to clinical attention due to complications of portal hypertension such as variceal hemorrhage. A variety of standard surgical techniques exist to manage these patients, but when these fail surgical options are limited. We describe two novel portosystemic shunts that utilize the gonadal vein as an autologous conduit. Four patients were evaluated for EHPVT with variceal bleeding. None of the patients were candidates for a standard splenorenal shunt due to prior surgical procedures. The first patient underwent a left mesogonadal shunt and the remaining 3 patients underwent a right mesogonadal shunt. Postoperative ultrasound or computed tomography (CT) scan confirmed early patency of the shunt in each patient. There have been no further episodes of variceal hemorrhage with follow-up of 3.5 years in the child who underwent the left mesogonadal shunt, and 17, 19, and 20 months in the patients who underwent the right mesogonadal shunt. Three of the 4 shunts remain patent. One shunt thrombosis occurred in a patient homozygous for the Factor V Leiden mutation despite anticoagulation with coumadin. This is the first report of the successful use of the gonadal vein as an in situ conduit for constructing a portosystemic shunt. In conclusion, the right and left mesogonadal shunts may be useful as salvage operations for patients with EHPVT who have failed standard surgical shunt procedures.

Adolescent↗
PubMed2005

Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon.

Kasabach-Merritt phenomenon is a serious coagulopathy associated with kaposiform hemangioendothelioma (KHE), tufted angioma, and possibly other vascular neoplasms. KHE presenting in the absence of Kasabach-Merritt phenomenon is rare, although tufted angioma frequently occurs without thrombocytopenia. We retrospectively reviewed 10 cases of KHE without Kasabach-Merritt phenomenon. The tumors appeared as soft tissue masses with the overlying skin being either normal, erythematous, or violaceous. There were no radiologic or microscopic differences in noncoagulopathic KHE as compared with coagulopathic KHE. Evidence of platelet trapping and hemosiderin deposition was seen histologically, despite normal serum platelet levels. All KHE were less than 8 cm in diameter, suggesting that tumors that grow no larger than this size are less likely to trap platelets in sufficient quantity to cause thrombocytopenia. Our series confirms that KHE appears with a wide spectrum of behavior and response to treatment. The decision as to whether or not to treat a noncoagulopathic KHE should be based on the size and location of the tumor and the possible side effects of therapy.

Child↗
PubMed2005

Enhancement of intravascular sclerotherapy by tissue engineering: short-term results.

BACKGROUND/PURPOSE: Treatment of vascular malformations with sclerotherapy is often complicated by reexpansion secondary to endothelial recanalization. This study examined the use of an autologous fibroblast construct to enhance intraluminal scar formation after sclerotherapy. METHODS: New Zealand rabbits (n = 15) underwent ethanol sclerotherapy of a segment of the facial vein. After intraluminal saline flush, animals were equally divided into 3 groups. In group I, no further manipulations were performed. In groups II and III, collagen hydrogel was injected into the sclerosed vein, respectively, without and seeded with autologous green fluorescent protein-labeled fibroblasts. One week postoperatively, the vein segments were examined for patency and resected for histology. RESULTS: The sclerosed vein segments remained occluded in all animals. Histological examination of luminal thrombi demonstrated numerous viable fibroblasts in group III, whereas there were none in the control specimens from groups I and II. The presence of the injected autologous green fluorescent protein-labeled fibroblasts within thrombi of group III was confirmed by immunohistochemistry. CONCLUSIONS: An injectable tissue-engineered construct enhances sclerotherapy of the jugular vein in a leporine model by reliably delivering fibroblasts that populate the resultant thrombus. Further analysis of this novel therapeutic concept as a means to augment permanent scar formation and reduce luminal recanalization is warranted.

Animals↗
PubMed2005

An injectable tissue-engineered embolus prevents luminal recanalization after vascular sclerotherapy.

BACKGROUND/PURPOSE: Sclerotherapy for vascular malformations is often limited by luminal recanalization. This study examined whether an injectable tissue-engineered construct could prevent this complication in a rabbit model of venous sclerotherapy. METHODS: Ethanol sclerotherapy of a temporarily occluded jugular vein segment was performed in 46 rabbits, which were then divided into 3 groups. Group I (n = 16) had no further manipulations. In groups II (n = 15) and III (n = 15), 0.5 mL collagen hydrogel was injected intraluminally, respectively, devoid of and seeded with autologous fibroblasts. At 1, 4, and 20 to 24 weeks postoperatively, vein segments were examined for patency and resected for histological evaluation. Statistical analysis was by Fisher's Exact test. RESULTS: All vein segments were occluded at 1 and 4 weeks in all groups, despite histological evidence of progressive endothelial ingrowth. However, at 20 to 24 weeks, angiography demonstrated restoration of vessel patency in groups I (3/6) and II (3/5), but not in group III (0/6; P = .043), in which histology confirmed an obliterated lumen for all vessels. CONCLUSION: An injectable, fibroblast-based, engineered construct prevents midterm to long-term recanalization in a leporine model of vascular sclerotherapy. This novel therapeutic approach may prevent recurrence of vascular malformations after sclerotherapy, thus reducing the need for repeated procedures and morbid operative resections.

Animals↗
PubMed2005

Iliac artery stenosis in a child with cutis marmorata telangiectatica congenita.

Cutis marmorata telangiectatica congenita (CMTC) is a rare congenital disorder. We describe an 8-year-old boy with CMTC who presented with symptomatic claudication and diminished distal pulses. Imaging showed severe stenosis of the right common iliac artery, and the child underwent uncomplicated ilio-iliac bypass using prosthetic graft. This is the first report of a patient with CMTC and major vessel stenosis, successfully treated with a prosthetic graft bypass.

Blood Vessel Prosthesis Implantation↗
PubMed2005

Neonatal thoracoscopic repair of congenital diaphragmatic hernia: selection criteria for successful outcome.

BACKGROUND/PURPOSE: Complications of open conversion, hypercarbia, and intestinal injury have plagued minimally invasive approaches to congenital diaphragmatic hernia (CDH) repair in neonates. To safely begin using minimally invasive techniques for neonatal CDH repair, we formulated preoperative selection criteria and operative techniques that would enhance chances for successful thoracoscopic primary diaphragm repair and uncomplicated outcome. METHODS: During the period from January 2003 to October 2004, neonates were selected for thoracoscopic CDH repair using anatomic and physiologic criteria. Anatomically, all patients were required to have stomach in the abdomen by radiography. Physiologically, all patients were required to be on minimal ventilator support with preoperative ventilator peak inspiratory pressures in the low 20s mm Hg. No patient could have clinical evidence of pulmonary hypertension at the time of surgery. Thoracoscopic CDH repair was performed using 3 trocars (3 and 5 mm). The hernia contents were reduced into the abdomen using 5-mm Hg insufflation, and the diaphragms were repaired primarily using interrupted 3-0 Ethibond simple sutures (Ethicon, Inc, Piscataway, NJ). Posterolateral diaphragm stitches were passed around the posterolateral ribs and tied extracorporeally. RESULTS: Thirty neonates with CDH were admitted to Children's Hospital Boston and Vanderbilt Children's Hospital during the study period. Eight patients (27%) met selection criteria and 7 underwent thoracoscopic CDH repair. Primary diaphragmatic repair was successfully accomplished thoracoscopically in all neonates without perioperative complication. Preoperative anatomic criteria correlated accurately with intact esophageal hiatus and primary diaphragm repair. Physiologically, each patient tolerated intrathoracic insufflation and CDH repair without clinical pulmonary hypertension or blood pressure lability. Three patients had intraoperative respiratory acidosis that was reversed with ventilator changes. Operative times averaged 152 minutes and ranged from 212 to 106 minutes. Postoperative mechanical ventilation ranged from 0 to 7 days, and the length of hospitalization ranged from 5 to 32 days. Longest follow-up has been 17 months. One patient required reoperation for recurrent CDH at 10 months after repair, but there have been no other long-term complications. CONCLUSIONS: Neonatal thoracoscopic CDH repair is safe in selected patients who have good preoperative pulmonary function and anatomy amenable to primary diaphragmatic repair. A wider range of neonates may be acceptable for thoracoscopic CDH repair with increasing surgical experience.

Female↗
PubMed2005

Congenital absence of the right upper lobe bronchus with double segmental tracheal bronchi.

A multidisciplinary approach revealed an absent right upper lobe bronchus and atretic bronchioles in a child with recurrent pulmonary infections. Use of a multidimensional computed tomographic scan and bronchoscopy clearly delineated the anatomical aberration. The child underwent an uncomplicated right upper anterior segmentectomy with an expedient recovery.

Abnormalities, Multiple↗
PubMed2005

Blue rubber bleb nevus syndrome: surgical eradication of gastrointestinal bleeding.

OBJECTIVE: We report the largest clinical experience to date of surgically treated patients with blue rubber bleb nevus syndrome (BRBNS). SUMMARY BACKGROUND DATA: BRBNS is a rare congenital disorder presenting with multifocal venous malformations of the skin, soft tissues, and gastrointestinal (GI) tract. Patients with BRBNS develop anemia from chronic GI bleeding, and require lifelong treatment with iron and blood transfusions. An aggressive surgical approach to treat the GI venous malformations of BRBNS has been considered unlikely to be successful because of the large number of lesions, their position throughout the GI tract, and the likelihood of recurrence. Based on our belief that eradicated lesions would not recur, we undertook the removal of all GI tract lesions in an effort to eliminate bleeding. METHODS: Ten patients with BRBNS were treated from 1993 to 2002. Lesions were identified using complete GI endoscopy. The multiple venous malformations were removed by a combination of wedge resection, polypectomy, suture-ligation, segmental bowel resection, and band ligation. RESULTS: Patient ages ranged from 2 to 36 years, and patients received an average of 53 prior blood transfusions. A mean of 137 focal GI venous malformations per patient were resected at operation (range 4-557), with a mean operative duration of 14 hours (range 7-23 hours). Only 1 patient who had a less extensive procedure developed recurrent GI bleeding. The mean follow-up period was 5.0 years (range 2.9-10.3 years). CONCLUSIONS: We believe that an aggressive excisional approach is indicated for the venous anomalies that cause GI bleeding in BRBNS.

Adolescent↗