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Biomedical subjects

Josée Dubois

Publications and source records attributed to Josée Dubois.

16 recordsLinked to original sources

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↗

Orbit and eyelid hemangiomas: is there a relationship between location and ocular problems?

PURPOSE: Infantile hemangiomas of the orbit and eyelid can cause serious ocular problems, such as astigmatism and amblyopia. Magnetic resonance imaging and ultrasonography are the best ways to establish the diagnosis and to evaluate the extent of the lesion. Our goal was to correlate the lesion location with the visual impairment. MATERIAL AND METHODS: This was a retrospective study (1992-2004) of 63 cases of orbit and eyelid hemangiomas (13 male, 50 female patients; ages: 1 day old to 3 years old). Imaging methods were magnetic resonance imaging or computed tomographic-scanning and color Doppler ultrasonography. The anatomic definition was palpebral, extraconal, and extraconal with intraconal involvement. All patients had ophthalmologic evaluations. RESULTS: The hemangiomas were palpebral (n = 32) (normal vision: 19; amblyopia and/or astigmatism: 13), extraconal, and extraconal with intraconal involvement (n = 31) (normal vision: 4; amblyopia and/or astigmatism: 27). LIMITATIONS: This study was small; a prospective study is needed. CONCLUSION: Orbit and eyelid hemangiomas need to be carefully evaluated by an ophthalmologist. Extraconal and intraconal hemangiomas are more frequently associated with ocular involvement. However, ocular complications can occur in palpebral lesions.

Amblyopia↗

Alcoholic solution of zein (Ethibloc) sclerotherapy for treatment of lymphangiomas in children.

PURPOSE: The aim of this study was to report the experience and efficacy of Ethibloc sclerotherapy as treatment of lymphangiomas. METHODS: Between 1992 and 2004, 63 patients had Ethibloc sclerotherapy for lymphangiomas at our institution. Computed tomographic scan or magnetic resonance imaging and clinical evaluation determined efficacy of the treatment. Results were classified as excellent (> or =95% decrease in lesion volume), satisfactory (> or =50% decrease and asymptomatic), or poor (<50% decrease or symptomatic). RESULTS: Sixty-three patients with 67 lesions underwent sclerotherapy with a median of 2 treatments per patient. Thirty-five involved the neck; 10, the head and face; and 22, the thorax or limb. Thirteen were predominantly microcystic; 28, macrocystic; and 26, mixed. Of the 63 patients, 6 underwent sclerotherapy for postsurgical residual lesions. Results were classified by type: of the 54 macrocystic/mixed cases, 26 (49%) had excellent, 19 (35%) had good, and 9 (16%) had poor results; in the 13 predominantly microcystic lesions, 3 (23%) had excellent, 7 (54%) had good, and 3 (23%) poor results. Five patients (7.7%) required surgery for complications; 2, for scar revision; 2, for persistent drainage; and 1, for a salivary fistula. Infection occurred in 4 patients (6.2%) after sclerotherapy. Follow-up averaged 3.5 years (range, 12 months to 12 years). CONCLUSION: Ethibloc sclerotherapy is a safe and effective alternative to surgical excision of macrocystic lymphangiomas and can be used for postsurgical recurrences.

Child↗

Imaging characteristics of two subtypes of congenital hemangiomas: rapidly involuting congenital hemangiomas and non-involuting congenital hemangiomas.

BACKGROUND: Common infantile hemangiomas (COMMON) occur in approximately 10% of infants by the age of 1 year, with a female predominance. Some hemangiomas can be fully developed at birth and are thus called congenital hemangiomas (CH). Within this population, two courses have been identified: rapidly involuting CH (RICH) and non-involuting CH (NICH). Little has been reported on the clinical prognosis and imaging features of these entities. OBJECTIVE: To describe the imaging characteristics of two subtypes of CH, i.e. RICH and NICH, and to compare them with COMMON. MATERIALS AND METHODS: We retrospectively gathered data on 26 children presenting with CH, i.e. lesions fully developed at birth. These lesions were divided into two groups according to the clinical course: suspected RICH (n=8) and suspected NICH (n=18). We used US, CT or MRI and angiography to identify the gross anatomy and structure and the vascularization. Imaging findings were compared with the clinical course and pathology results, when available. The imaging findings in these patients were compared retrospectively with those in 26 patients with COMMON randomly chosen from the database of our multidisciplinary clinic. RESULTS: When compared with COMMON imaging characteristics, NICH and RICH had distinctive features on US such as being heterogeneous (72% of NICH and 62.5% of RICH vs 42.3% of COMMON), visible vessels (72% of NICH and 62.5% of RICH vs 15.4% of COMMON), calcifications (17% of NICH and 37.5% of RICH vs no case of COMMON). On CT and/or MRI, we compared imaging features such as well-defined limits (67% of NICH and 60% of RICH vs 100% of COMMON), and fat stranding (29.4% of NICH and RICH vs 7.7% of COMMON). CONCLUSION: Distinctive imaging characteristics are observed in cases of CH with US findings of visible vessels and calcifications statistically significant.

Adolescent↗

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↗

Wireless capsule endoscopy for obscure small-bowel disorders: final results of the first pediatric controlled trial.

BACKGROUND AND AIMS: Obscure small-bowel disorders are jejunal and ileal lesions undiagnosed by traditional imaging techniques (endoscopic, radiologic). We evaluated the diagnostic usefulness and safety of capsule endoscopy for obscure small-bowel disorders in children and adolescents. METHODS: Comparative, prospective, self-controlled trials in patients (age, 10-18 y) suspected to have either small-bowel Crohn's disease, polyps, or obscure gastrointestinal (GI) bleeding. Capsule results were compared with the diagnostic imaging studies normally used in this age group. RESULTS: Among 20 patients suspected of Crohn's disease, multiple lesions consistent with this diagnosis were observed by capsule endoscopy in 50%. Small-bowel Crohn's disease was ruled out in 8 patients. Eosinophilic enteropathy was found in 2 others. For polyp detection (n = 6), capsule endoscopy yielded 100% concordance with the control studies when analyzed per patient. However, capsule endoscopy revealed a greater number (50%) of polyps. Among patients with obscure bleeding (n = 4), the capsule examination confirmed a diagnosis of vascular malformations in 3. Capsule endoscopy more accurately identified the precise source of bleeding compared with angiography. All 30 capsule studies were well tolerated, although 1 capsule was retained owing to an inflammatory stenosis. The capsule eventually was expelled after corticosteroid therapy. CONCLUSIONS: Capsule endoscopy correctly diagnosed or excluded a bleeding source, small-bowel polyps, or Crohn's disease of the small bowel in 29 of 30 patients. Capsule endoscopy permits an accurate, noninvasive approach for diagnosing obscure small bowel lesions in children over the age of 10.

Adolescent↗

Complications of ENT infections: pseudoaneurysm of the internal carotid artery.

Ear, nose and throat infections are common, especially in children and young adults. Since the advent of antibiotics, complications from tonsillitis and pharyngeal abscess are rare, but potentially lethal. Vascular complications can be imaged with Doppler ultrasound and CT scan. The treatment of infectious vascular complications represents a significant challenge. We describe the case of a young girl presenting with a pseudoaneurysm of the internal carotid and thrombosis of the internal jugular vein. Endovascular therapy was utilized to treat the patient.

Aneurysm, False↗

Infantile hepatic hemangiomas: clinical and imaging findings and their correlation with therapy.

OBJECTIVE: This study was undertaken to determine different imaging patterns in infantile hepatic hemangiomas and to explore the relationship between clinical presentations, imaging findings, and response to therapy. MATERIALS AND METHODS: The imaging studies and clinical records of all patients with infantile hepatic hemangiomas from two tertiary children's hospitals were reviewed. Univariate and multivariate stepwise logistic regression techniques were used to determine whether clinical presentation and imaging variables differentiated the type of treatment required. RESULTS: Typical hemangiomas appeared as focal or multifocal T2-hyperintense spheres with centripetal contrast enhancement and dilated feeding and draining vessels. Three atypical patterns included focal mass lesions with central varix with or without direct shunts, focal mass with central necrosis or thrombosis, and massive hemangiomatous involvement of the liver with abdominal vascular compression. In general, patients with focal lesions without high flow needed no treatment, and those with central varix and direct shunts developed severe high-output cardiac failure that responded quickly to embolization. The pattern of massive replacement of liver was associated with hypothyroidism, abdominal compartment syndrome, and a high mortality rate. Multivariate analysis of 55 patients indicated that congestive heart failure was the only independent predictor of treatment (p = 0.005). The presence of a shunt was the only independent factor associated with embolization or surgery (p = 0.002). CONCLUSION: Although the imaging features of infantile hepatic hemangiomas vary to some extent, MRI features are typical in most patients and certain imaging findings are predictive of the clinical course. MRI is the technique of choice in diagnosing infantile hepatic masses.

Chi-Square Distribution↗

[Anatomic variations of the spermatic vein and endovascular treatment of left varicoceles: a pediatric series].

OBJECTIVE: To report on the high incidence of anatomical variants of the origin and course of the internal spermatic vein (ISV) discovered at the time of percutaneous embolization of left varicoceles in a pediatric population. METHODS: We reviewed retrospectively the 65 cases of left varicocele treated by percutaneous embolization (grade II and III) in our institution between 1990 and 2000. The course of the left renal vein (LRV), the origin of the ISV, and the number of ISVs and their pathway were recorded in all cases, according to the Bähren classification. RESULTS: In 37/65 (57%), the ISV was single and arose from a normal LRV (type I). The following variants were encountered: type V--circumaortic LRV 9/65 (14%); type IVb--intrarenal origin of ISV 8/65 (12%); type II--multiple ISV 5/65 (8%); and pelvic collaterals 6/65 (9%). CONCLUSION: Venous anatomical variants are frequently encountered (43%) at the time of left varicocele embolization in children. Such variants often impose some adjustments to the technique of embolization and, at times, hamper the procedure.

Adolescent↗

A large unilateral renal artery aneurysm in a young child.

The case of a 13-month-old boy with fibromuscular dysplasia (FMD) presenting with a large saccular aneurysm of the left renal artery and renovascular hypertension is reported. Renal and intrarenal arteries showed numerous small aneurysms alternating with stenoses. All arterial lesions were localized to the left kidney. After left nephrectomy, the patient's blood pressure normalized. Histopathologic examination of the arteries disclosed changes typical of medial fibroplasias, the most frequently described form of FMD in children. This diagnosis is rewarding as it represents a surgically curable cause of severe hypertension.

Aneurysm↗

Sclerotherapy in aneurysmal bone cysts in children: a review of 17 cases.

OBJECTIVE: To determine the efficacy of percutaneous sclerotherapy in the treatment of aneurysmal bone cysts. MATERIALS AND METHODS: Seventeen patients (7 girls, 10 boys) with aneurysmal bone cysts were treated by the percutaneous approach with Ethibloc ( n=14) and histoacryl glue ( n=3) in our institution between January 1994 and June 2000. The cysts were located in the extremities ( n=6), pelvis ( n=2), spine ( n=2), mandible ( n=5), rib ( n=1) and sphenoid bone ( n=1). Percutaneous sclerotherapy was performed with fluoroscopic and/or computed tomographic guidance under general anesthesia. Clinical and imaging follow-up lasted from 24 months to 9 years and 6 months (mean: 57.3 months). The results were quantified as: excellent (residual cyst less than 20% of the initial involvement), satisfactory (residual cyst 30-50%), unsatisfactory (residual cyst more than 50%). RESULTS: The age of the patients ranged from 4 years and 6 months to 15 years and 8 months (mean: 11 years and 2 months). In nine patients, the therapeutic procedure was repeated 2-5 times. Excellent regression was observed in 16 (94%), satisfactory results in 1 (6%). There was no failure (unsatisfactory result or no response to treatment) in this reported series. The complications were minor and included: local inflammatory reaction ( n=2), small blister ( n=1), and leakage ( n=1). Relief of symptoms was achieved in all patients. No recurrence was noted during follow-up. CONCLUSION: Percutaneous sclerotherapy of aneurysmal bone cysts with Ethibloc is safe and effective. It is an important alternative to surgery, especially when surgery is technically impossible or not recommended in high-risk patients.

Adolescent↗

Centrilobular fibrosis in long-term follow-up of pediatric liver transplant recipients.

BACKGROUND: Centrilobular fibrosis after liver transplant in adults is caused mainly by viral hepatitis, chronic rejection, and azathioprine toxicity. The aim of this study was to investigate possible etiologies and the long-term outcome of this lesion in children. METHODS: We identified centrilobular fibrosis in 12 of 117 pediatric liver transplant recipients who were investigated for persistent elevations in aminotransferases. Etiologic factors, histologic features on serial biopsies, and clinical and biochemical changes over time were noted for 8 recipients in whom a readily identifiable cause was not apparent. RESULTS: Centrilobular fibrosis developed a mean of 1.7 years (range: 30 days-3.6 years) posttransplantation in patients receiving cyclosporine, azathioprine, and prednisone. Centrilobular fibrosis was always associated with portal fibrosis and, in six recipients, with persistent, low-grade, cellular rejection. None demonstrated chronic cholestasis, ductopenia, or identifiable vasculopathy. Ischemic, viral, and autoimmune etiologies were excluded. Discontinuing azathioprine did not lead to biochemical or histological improvement. After changing to tacrolimus, aminotransferases normalized in three recipients and repeat biopsies in six were unchanged during a further 2 years of follow-up. CONCLUSIONS: Centrilobular fibrosis may develop in a small number of pediatric liver transplant recipients, resulting in considerable difficulties in biopsy interpretation. It is not associated with viral hepatitis nor with classical features of chronic rejection. The prognostic significance of centrilobular fibrosis is uncertain, although no child has required retransplantation in up to 12 years of follow-up. A role for a low-grade, chronic form of cellular rejection heralded by persistent, variable, and otherwise unexplained elevations in aminotransferases is suggested.

Adolescent↗

Angiographic classification of hepatic hemangiomas in infants.

PURPOSE: To review the angiograms in patients with hepatic hemangiomas referred to two North American children's hospitals to determine the variability in angiographic findings and to propose a classification system that is based on these findings. MATERIALS AND METHODS: Angiograms obtained in 15 infants with a diagnosis of hepatic hemangioma who were examined at or referred to two tertiary pediatric hospitals in North America from 1981 through 2000 were reviewed. The angiographic findings were then used to classify hemangiomas into types on the basis of a number of features, including high-flow nodules, early filling of veins, and the type of direct shunt present. Clinical data, including age at presentation, presence of cardiac insufficiency, and treatment, were also recorded. RESULTS: Lesions were classified into five types on the basis of angiographic findings. In three of 15 patients, angiograms demonstrated the classic appearance of hepatic hemangiomas, with early filling of abnormal vascular channels, stagnation of contrast material, and no evidence of a direct shunt (type 1). In four patients, images showed high-flow nodules without direct shunts (type 2). In eight patients, direct shunts were demonstrated: arteriovenous shunts (type 3) in one, portovenous shunts (type 4) in three, and both arteriovenous and portovenous shunts (type 5) in four. CONCLUSION: Hepatic hemangioma in infants is a heterogeneous lesion with variable angioarchitecture and a spectrum of angiographic findings.

Angiography↗

Vascular soft-tissue tumors in infancy: distinguishing features on Doppler sonography.

OBJECTIVE: We describe the sonographic appearance and vascularization of three types of vascular tumors, including hemangioendothelioma, tufted angioma, and infantile myofibromatosis, and we determine whether vessel density and peak systolic shift can distinguish these tumors from angiomas and differentiate between the subtypes of these three entities. SUBJECTS AND METHODS: Our study included 16 infants with vascular tumors, other than hemangiomas, who were to undergo biopsy. We used gray-scale sonography to identify calcifications, to evaluate the borders of the lesions to determine whether they were poorly defined or well defined, and to determine the echogenicity relative to the surrounding soft tissue. Doppler sonography served to determine the number of vessels per square centimeter and the peak arterial Doppler shift. Sonographic findings were compared with the final diagnoses established by biopsy. RESULTS: The final diagnoses included five hemangioendotheliomas, six tufted angiomas, and five infantile myofibromatoses. Hemangioendotheliomas and tufted angiomas were ill defined compared with infantile myofibromatoses that were well defined. Only one vascular tumor, a hemangioendothelioma, fulfilled the diagnostic criteria of hemangioma. Tufted angiomas and infantile myofibromatoses were the least vascularized, with the lowest vessel density (zero to two vessels per square centimeter) and a relatively low systolic Doppler shift (0.7-1.0 kHz). CONCLUSION: The vascular tumors-hemangioendotheliomas, tufted angiomas, and infantile myofibromatoses-were distinguishable from hemangiomas on Doppler sonography in all cases except one hemangioendothelioma. Unlike hemangiomas, these lesions should be investigated by biopsy or excision.

Child↗

Obstetric US: watch the fetal hands.

Hand anomalies are difficult to diagnose and are often overlooked during prenatal ultrasonography (US). The spectrum of malformations varies from subtle finger deformities to the complete amputation of limbs. Malformations of the hand can be classified, according to the predominant anomaly, among the following categories: alignment abnormalities (clenched hand, camptodactyly, clinodactyly, hypokinesia, clubhand, phocomelia), thumb anomalies, abnormal size (macrodactyly, trident hand), abnormal echogenicity (abnormal calcifications), abnormal number (polydactyly, syndactyly, ectrodactyly), and constriction band sequence. A fetal hand anomaly has important diagnostic and prognostic implications as well as functional consequences. Malformation may be isolated but often is associated with a syndrome or karyotype anomaly. Classification and characterization of the anomaly help to narrow the differential diagnosis: Some malformations (clenched hand, hitchhiker thumb) are highly suggestive of a specific diagnosis. The detection of a fetal hand malformation warrants a complete work-up, including complete fetal and cardiac US examinations, as well as genetic counseling to determine whether familial inquiry and karyotype analysis are necessary.

Hand Deformities, Congenital↗