Biomedical subjects

Patricia E Burrows

Publications and source records attributed to Patricia E Burrows.

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

Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity.

OBJECTIVE: To determine the efficacy and safety of radiofrequency (RF) ablation of vesicles and the resulting symptomatic control of microcystic lymphatic malformation (LM) in the oral cavity. DESIGN: An institutional review board-approved retrospective study with follow-up telephone interview. SETTING: Tertiary pediatric medical center. Patients Eleven children (6 girls and 5 boys), aged 4 to 16 years, presenting between August 1, 2002, and December 1, 2004. Intervention Radiofrequency ablation of LM in the oral cavity. MAIN OUTCOME MEASURES: Symptoms related to LM, postoperative oral intake, and postoperative antibiotic requirements. RESULTS: Eleven patients presented with microcystic LM involving the lips, tongue, floor of the mouth, or buccal mucosa. Complaints included bleeding, infection, swelling, vesicle formation, and malocclusion. Patients underwent RF ablation (coblation) of oral cavity lesions. Seven (64%) of the 11 patients were able to tolerate oral intake in the recovery room. The need for antibiotics was reduced after RF ablation. All patients related diminished bleeding, pain, infection, or vesicle formation, with more than half reporting a significant improvement (6 patients) or complete resolution (1 patient). Five (62%) of 8 parents stated that the improvement after RF ablation was superior to that following previous procedures. CONCLUSIONS: Subtotal RF ablation of LM appears to be safe, with early postoperative oral intake and minimal postoperative pain. Further studies are needed to determine long-term control of LM.

Adolescent↗

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

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↗

PubMed2006

Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-evaluated improvement.

PURPOSE: To evaluate the midterm outcomes of percutaneous sclerotherapy of lymphatic malformations (LMs) as judged by patients. MATERIALS AND METHODS: A 13-item survey questionnaire was sent to 74 patients who had undergone at least one sclerotherapy procedure in our hospital from January 1997 through January 2003. Information regarding the anatomic location, specific symptoms reported, history, treatment satisfaction, postprocedural complications, and number of treatment sessions was elicited. Four sclerosing agents (as single agents or in combination with other agents) were used: ethanol, sodium tetradecyl sulfate 3% (STS), OK-432, and doxycycline. RESULTS: Fifty-five patients or their caregivers completed the survey. The patients' ages ranged from 6 months to 48 years at the time of the first procedure (mean, 12 y; median, 4 y). A majority of LMs were located in the cervicofacial region. The size and location of the lesion, recurrent infection, and pain were the most frequent indications for treatment. Fifty-one percent of these patients received sclerotherapy alone or in conjunction with surgery as primary treatment. Ethanol was the most common sclerosing agent used, followed by doxycycline, STS, and OK-432. Response varied with the type of LM, with 100%, 86%, and 43% of the patients reporting good to complete response for macrocystic, microcystic, and combined-type LMs, respectively. Skin blistering and ulcers were the most common complications. Permanent complications were uncommon and were largely related to ethanol use. CONCLUSIONS: Percutaneous sclerotherapy provides effective midterm primary treatment for LMs. Treatment outcomes appear to vary according to the morphology of the malformation.

Adolescent↗

PubMed2006

Value of bispectral index monitor in differentiating between moderate and deep Ramsay Sedation Scores in children.

BACKGROUND: Pediatric patients who undergo diagnostic radiological imaging studies routinely require moderate or deep sedation to a Ramsay Sedation Score (RSS) of 4 or 5, respectively. The correlation between moderate and deep RSS and bispectral index (BIS) in children has never been validated. This study was designed to determine whether the BIS values correlate with RSS of children sedated for diagnostic imaging studies. METHODS: After Institutional Review Board approval, data were prospectively collected on all children receiving pentobarbital alone for computed tomography or magnetic resonance imaging. On arrival in the recovery room, any child assigned a Ramsay score of 4 or 5 was subsequently monitored with the BIS. Receiver-operating characteristic (ROC) analysis was used to evaluate the discriminative ability of BIS in differentiating depth of anesthesia. RESULTS: A total of 86 children over 1 year of age were enrolled. Children with Ramsay 4 (moderate) and Ramsay 5 (deep) sedation scores showed no significant difference in age or pentobarbital dose (mg.kg(-1)). BIS values followed a normal Gaussian-shaped distribution for both Ramsay scores. There was no significant difference in mean BIS values between the Ramsay groups (P = 0.64). There was a wide variation in BIS values (range of 31-90), which demonstrates that the BIS monitor does not correlate with the clinically assigned RSS depth of anesthesia in children. The ROC curve for BIS is equivalent to a line of nondiscrimination. CONCLUSION: BIS has limited ability to distinguish between moderate and deep Ramsay sedation levels in children who receive pentobarbital sedation for diagnostic imaging studies.

Child↗

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

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

Auricular arteriovenous malformation: evaluation, management, and outcome.

BACKGROUND: The external ear is the second most common site for extracranial arteriovenous malformation in the head and neck. METHODS: This retrospective review of 41 patients with auricular arteriovenous malformation was based on medical records, imaging studies, and photographs. Data were collected on natural history, progression, and outcome; patients were questioned about quality of life after treatment. RESULTS: The median age at initial presentation was 26 years (range, 1 to 55 years), and Schobinger stage was I in two patients, II in 19 patients, and III in 20 patients. No patients had a Schobinger stage of IV. Expansion occurred during childhood in seven patients, adolescence in 14 patients, pregnancy in 10 patients, and adulthood in 10 patients. Distribution of auricular and extra-auricular arteriovenous malformation was not limited to "watershed" areas between vascular territories (angiosomes). Twelve patients were untreated (follow-up, 0.5 to 6 years). Mean follow-up time for the 29 treated patients was 5.19 years (range, 1 to 18.75 years). Proximal ligation in nine patients caused progression: eight of them underwent amputation and one had embolization. Fifteen patients had embolization only: the arteriovenous malformation worsened and amputation was necessary in six patients; in the remaining nine patients, two improved, four persisted, and three worsened. Of 20 patients who had auricular amputation, 16 (80 percent) were controlled, three (15 percent) improved, and one had unresectable, residual cervicofacial arteriovenous malformation. Of 22 of 29 treated patients surveyed, 81 percent were satisfied with their management. Hearing was either unaffected (n = 15) or diminished (n = 5); two patients noted decreased sound localization. CONCLUSIONS: The authors recommend periodic evaluation for stage I to II auricular arteriovenous malformation and intervention if there is evolution to stage III. Preoperative embolization and partial or total amputation effectively control auricular and para-auricular arteriovenous malformation. Embolization can be palliative in children or in patients who are not psychologically prepared for amputation. Extensive extra-auricular arteriovenous malformation requires individualized endovascular therapy and resection.

Adolescent↗

PubMed2005

Lymphatic malformation of the lingual base and oral floor.

BACKGROUND: Lymphatic malformation of the tongue and floor of the mouth is associated with chronic airway problems, recurrent infection, and functional issues related to speech, oral hygiene, and malocclusion. There are no accepted anatomic guidelines or treatment protocols. METHODS: This retrospective review focused on anatomic extent, treatment, complications, and airway management in 31 patients with lymphatic malformation of the lingual base and oral floor. RESULTS: Involved adjacent structures included the neck (77 percent), mandible (41 percent), face (42 percent), lips (10 percent), pharynx (45 percent), and larynx (26 percent). Fifty-eight percent of patients required tracheostomy during infancy; decannulation was possible in two-thirds of these patients. Management included resection alone (42 percent), resection and sclerotherapy (26 percent), resection and laser coagulation (16 percent), sclerotherapy and laser coagulation (16 percent), and resection and radiofrequency ablation (3 percent). Resection involved the neck (58 percent), floor of the mouth (52 percent), and tongue (42 percent); there were often multiple procedures. Aspiration was tried with little success in 10 percent of patients. Virtually all patients had residual abnormal lymphatic tissue. Complications and posttherapeutic problems included infection (81 percent), neural damage (27 percent), difficulty in speech (23 percent), feeding problems (10 percent), and seroma or hematoma (6 percent). Associated dental/orthognathic conditions, particularly prognathism and anterior open bite, were documented in one-third of patients. CONCLUSIONS: The initial step in the protocol is control of the neonatal airway. Staged cervical resection is undertaken in late infancy to early childhood; resection should also include abnormal tissue in the oral floor. Sclerotherapy is primarily for macrocystic disease or secondarily for recurrent cysts following partial extirpation. Vesicles of the mucous membranes and dorsal tongue are treated either by sclerotherapy, laser (carbon dioxide, yttrium-aluminum-garnet, or potassium-titanyl-phosphate), or radiofrequency ablation. Reduction for macroglossia is indicated for persistent protrusion or to allow correction of malocclusion. Embolization controls lingual bleeding. Orthognathic procedures are undertaken at the appropriate age, only after lingual size and position are acceptable.

Catheter Ablation↗

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↗

PubMed2005

Adverse cardiovascular and respiratory events during sedation of pediatric patients for imaging examinations.

PURPOSE: To retrospectively identify factors associated with an increased risk of adverse cardiovascular or respiratory events during sedation of pediatric patients for imaging examinations. MATERIALS AND METHODS: This HIPAA-compliant study was institutional review board approved; the requirement for informed consent was waived. All sedation information--including patient demographics, medications (doses and routes of administration), time required to sedate and before discharge, American Society of Anesthesiologists physical status classification, adverse events, and failed sedations--was maintained in a computerized database. A review of the data on all patients sedated between 1997 and 2003 for magnetic resonance imaging, computed tomography, and interventional radiology revealed associated adverse respiratory events in 70 patients. Adverse respiratory event was defined as oxygen desaturation of at least 5%, pulmonary aspiration, and need for airway resuscitation. Adverse cardiovascular events were defined as cardiac arrest and hemodynamic changes requiring medical therapy. Adverse events were compared between sedation regimens--which included fentanyl, chloral hydrate, pentobarbital, and midazolam hydrochloride--by using the Fisher exact test. Multiple logistic regression analysis was applied to identify potential predictors of adverse events. RESULTS: Among 16,467 sedations performed, 70 (0.4%) were associated with adverse respiratory events: 58 cases of oxygen desaturation, two pulmonary aspirations, 10 cases of airway resuscitation, and no cardiovascular events. Nearly 30% (n = 20) of the 70 patients who had an adverse event had a history of serious respiratory illness. Logistic regression analysis revealed that neither patient age, weight, or sex nor type of imaging procedure was associated with an increased risk of an adverse event. Use of a single sedation agent was associated with lower adverse event risk than was use of multiple agents (P < .001). CONCLUSION: Consideration should be given to using single agents, avoiding the use of multidrug sedation regimens, and recognizing that a history of pulmonary disease could be associated with an increased risk of adverse respiratory events despite a currently stable respiratory state.

Cardiovascular System↗

PubMed2005

Increased expression of urinary matrix metalloproteinases parallels the extent and activity of vascular anomalies.

OBJECTIVE: Matrix metalloproteinases (MMPs) and the angiogenic proteins basic fibroblast growth factor (bFGF) and vascular endothelial growth factor (VEGF) have been implicated in mechanisms of human cancer and metastasis. Assays were conducted on the urine of patients with vascular anomalies (tumors and malformations), relatively common and occasionally life-threatening disorders for which few therapies exist. We sought to determine whether these angiogenesis modulators are present in the urine and whether their expression is associated with the extent and clinical course of the vascular lesion. METHODS: A total of 217 patients with vascular anomalies and 74 age-matched control subjects participated. Urinary MMP expression was determined by substrate gel electrophoresis. Urinary bFGF and VEGF levels were measured by enzyme-linked immunosorbent assay. Each patient was assigned to 1 of 2 categories (tumor or malformation) and 1 of 9 specific groups. Extent of the vascular lesion and activity were scored by a blinded clinician. RESULTS: Urinary high molecular weight (hMW) MMPs and bFGF were significantly increased in patients with vascular tumors (53%) and vascular malformations (41%), compared with control subjects (22%). These percentages increased as a function of extent of the lesion and disease activity. hMW MMPs were increased in 4 groups: infantile hemangioma, other vascular neoplasms, lymphatic malformation and capillary-lymphaticovenous malformations, and extensive and unremitting capillary malformation and arteriovenous malformation. No significant differences among the groups were detected for low molecular weight MMPs or VEGF. CONCLUSIONS: Expression patterns of hMW MMPs and bFGF in the urine of patients with tumors and malformations are consistent with their different clinical behavior. These data represent the first evidence that MMPs are elevated in the urine of children with vascular anomalies. These data also suggest that the increased expression of urinary MMPs parallels the extent and activity of vascular anomalies in children. In addition to tumors, vascular malformations are angiogenesis dependent, suggesting that progression of a vascular malformation might be suppressed by angiogenic inhibitors, which would target bFGF and MMPs.

Adolescent↗

PubMed2005

Periorbital lymphatic malformation: clinical course and management in 42 patients.

Lymphatic malformation in the orbital cavity and surrounding region often causes disfigurement and visual problems. To better clarify the evolution and treatment of this condition, the authors studied a retrospective cohort of 42 consecutive patients seen between 1971 and 2003 and analyzed anatomic features, complications, and management. The ratio of female to male patients was 1:1. Most periorbital lymphatic malformations were noted at birth (59 percent), presenting as either unilateral swelling (60 percent) or a periorbital mass (24 percent). Sixty-two percent of lesions were on the left side. The ipsilateral cheek, temple, and forehead also were involved in 57 percent of patients. Twenty-two percent of lesions were intraconal, 30 percent were extraconal, and 48 percent were in both spaces. Forty-five percent of children had an associated cerebral developmental venous anomaly. Periorbital lymphatic malformation caused major morbidity; 52 percent of patients had intralesional bleeding and 26 percent of patients had a history of infection. Other common complications included intermittent swelling (76 percent), blepharoptosis (52 percent), proptosis (45 percent), pain (21 percent), amblyopia (33 percent), chemosis (19 percent), astigmatism (17 percent), and strabismus (7 percent). Ultimately, 40 percent of children had diminished vision and 7 percent became blind in the affected eye. Management of periorbital lymphatic malformation involved an interdisciplinary team that included an interventional radiologist, a craniofacial surgeon, and an ophthalmologist. The two therapeutic strategies were sclerotherapy (40 percent) and resection (57 percent); most patients required several interventions. A coronal approach was used for subtotal excision of fronto-temporal-orbital lymphatic malformation in 13 patients, whereas a tarsal incision was used for lesions isolated to the eyelid (n = 14). Ocular proptosis was temporarily managed by tarsorrhaphy (n = 9), but expansion of the bony orbit was needed to correct persistent proptosis (n = 8). Orbital exenteration was necessary in two patients.

Abnormalities, Multiple↗

PubMed2004

Infant sedation for MR imaging and CT: oral versus intravenous pentobarbital.

PURPOSE: To compare the effectiveness and safety of oral (PO) versus intravenous (IV) pentobarbital sedation for magnetic resonance (MR) imaging and computed tomography (CT) in infants younger than 12 months. MATERIALS AND METHODS: The institutional review board approved the review of medical records and determined informed consent to be unnecessary. All parents gave informed consent for patient sedation. Prior to MR imaging or CT, infants younger than 12 months were sedated with PO pentobarbital (4-8 mg per kilogram body weight) or IV pentobarbital (2-6 mg/kg), depending on the presence of an IV catheter or need for IV contrast medium. A computer database used to record sedation data was reviewed for data from January 1997 to September 2003. PO and IV sedation groups were compared for mean age, weight, dose, time to sedation, time to discharge, and duration of sedation with a two-sample Student t test. Multivariate analysis of covariance was used to determine whether differences in sedation time, time to discharge, and duration of sedation between groups were independent of age, weight, sex, American Society of Anesthesiologists physical status classification, dose, and type of procedure. Sedation effectiveness (outcome) was determined as the percentage of sedation failures in each group. Safety was determined separately for other adverse events as a total and for respiratory adverse events. RESULTS: A total of 2164 infants received 2419 (1264 PO, 1155 IV) doses of pentobarbital for sedation. Weight and sex were comparable between groups. Time to sedation was significantly longer with PO than with IV pentobarbital (18 minutes +/- 11 vs 7 minutes +/- 7; P < .01), but time to discharge was similar, at approximately 108 minutes +/- 35. Total adverse events rate during sedation was not significantly different (0.8% [PO] vs 1.3% [IV]), but incidence of abnormal oxygen saturation (5% decrease from baseline, >1 minute duration) differed significantly (0.2% [PO] vs 0.9% [IV]; P = .02). Sedation effectiveness was comparable (failure rate, 0.5% [PO] vs 0.3% [IV]; P = .76). CONCLUSION: PO pentobarbital has comparable effectiveness and a lower rate of respiratory complications compared with IV pentobarbital in infants younger than 12 months; its use should be considered, regardless of presence of an IV catheter.

Administration, Oral↗