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1.

Context

There has been widespread interest surrounding the use of beta-blockers (i.e. propranolol, timolol, nadolol, acebutolol) in the treatment of infantile hemangiomas (IH).

Objective

To review literature evaluating treatment of IH with propranolol.

Evidence Acquisition

We conducted a literature search on PubMed and investigated for case reports, case series, and controlled trials by using search terms including “hemangioma” and “propranolol.”

Results

Data suggest that beta-blockers are efficacious in cutaneous, orbital, subglottic, and hepatic hemangiomas and assist in the resolution of ulcerated hemangiomas. Improvement has also been documented in children with PHACE syndrome. Propranolol produces favorable results in children who do not respond to steroids and with no long-term adverse effects. Propranolol should be administered with caution due to rare but serious side effects including hypoglycemia, wheezing, hypotension, and bradycardia. Additionally, recurrence of lesions following the cessation of treatment has been documented.

Conclusions

Although large-scale randomized controlled trials must be conducted in order to further evaluate the safety and the possible role of propranolol in the treatment of IH, the reviewed literature suggests that propranolol carries promise as a potential replacement for corticosteroids as first-line therapy or as a part of a multimodal approach.  相似文献   

2.

Objectives

To evaluate laboratory and radiological features of hemangiomas in childhood in addition to efficacy and safety of propranolol as a first-line treatment of complicated hemangiomas retrospectively.

Methods

The files of 60 patients who were diagnosed as capillary hemangioma were evaluated retrospectively. Fourteen children with complicated hemangiomas treated with propranolol were analysed, in terms of side effects, efficacy and duration of treatment.

Results

These fourteen patients (23 %) were treated with propranolol because of ulcerated, infected and/or deep seated localisations. The duration of treatment with propranolol were between 3 and 12 mo (median: 6 mo). Bronchospasm was observed in one patient during treatment. Except for two patients, all of them responded to propranolol treatment with limited side effects.

Conclusions

The present results support that propranolol is safe and effective treatment choice for complicated infantile hemangiomas, because of minimal side effects and encouraging response rates (80 %). In addition, the authors suggest that routine cranial radiological imagings might not be necessary for hemangiomas without any neurological symptoms.  相似文献   

3.

Purpose

Even if lumbar magnetic resonance imaging (MRI) is considered the gold standard in the diagnosis of occult spinal dysraphism (SD) in patients with anorectal malformations (ARMs), spinal ultrasound (US) performed up to 5 months of life have been largely used as a screening test. The aim of the present study was to evaluate the accuracy in terms of sensibility and specificity of neonatal US to detect occult SD in patients with ARMs.

Methods

Retrospective analysis of all patients treated for ARMs between 1999 and 2013 at our institution who underwent both spinal US (up to 5 months of life) and MRI. Sensibility and specificity have been calculated for US based on MRI results.

Results

Of 244 patients treated for ARMs at our institution, 82 (34 females, 48 males) underwent both the imaging studies and have been included in this study. ARMs types were: anal stenosis (7), recto-vestibular fistula (19), recto-perineal fistula (3) and cloaca (5) in female and imperforate anus (7) recto-perineal fistula (14), recto-urethral fistula (22), recto-vesical fistula (5) in males. Forty-seven patients (57, 3 % of total, 18 females, 29 males) had some occult SD (tethered spinal cord, spinal lipoma, syringomyelia) at MRI. Only 7 (14, 8 %) patients of those with spinal anomalies at MRI had pathological US studies. In our population, sensibility and specificity of US for diagnosis of occult SD were, respectively, 14, 8 and 100 %.

Conclusion

Since it is well known that a screening test must have a high sensibility, our data suggest that spinal ultrasound is not suitable as a screening test for occult spinal dysraphism in patients with ARMs. Furthermore, we strongly advise against the use of US as a screening test for spinal dysraphism to prevent a false sense of security in physician and patients’ families.  相似文献   

4.

Background

The efficacy and safety of beta-blockers versus corticosteroids in the treatment of infantile hemangiomas (IHs) is controversial. This study aimed to summarize evidence described in the literature and to assess the quality of studies involving beta-blockers and corticosteroids for the treatment of cutaneous IHs.

Methods

Comparative studies were collected from 15 online electronic databases, including OVID Medline, PubMed, ISI Web of Science, CENTRAL, CNKI, ChiCTR, JPCTR, CTRIndia, IranCTR, SLCTR, ISRCTRN, NLCTR, GCTR, ANCTR, ClinicalTrial. gov, and associated references. Studies without a control group were excluded, and the remaining studies were assessed by two reviewers independently using the Downs & Black scale for reported quality. The main areas assessed in the included studies were volume changes, overall improvement in appearance, eye function, and adverse events.

Results

Ten comparative studies were included with a total of 419 children. A meta-analysis was not performed due to the considerable heterogeneity across studies. Some evidence showed that beta-blockers are superior to steroids in reducing volume and improving the overall appearance of IHs, such as lightening of the color and flattening of the surface. Conclusions regarding improved eye function and adverse events were divided, and no consensus has been reached on the superiority of one treatment over another. No episodes of severe-onset asthma, hypotension, or bradycardia occurred in the beta-blocker treatment due to the rigorous exclusion of patients with contraindications.

Conclusions

Available studies indicate that beta-blockers are an alternative option to corticosteroids for IH treatment with respect to volume shrinkage and improvement in appearance. No evidence has shown a significant difference in improved eye function and adverse events between beta-blockers and corticosteroids in the treatment of IH; indeed, there is a lack of well-designed, high-quality randomized control trials.  相似文献   

5.

Introduction

Infantile hepatic hemangioma with consumptive hypothyroidism is a rare condition.

Case report

A 4-month-old girl presented with diffuse hepatic hemangiomas during treatment of congenital hypothyroidism. Serum reverse triiodothyronine was elevated, and her hypothyroidism improved concomitant with involution of the hemangioma following prednisolone and interferon-α administration. At 20 months of age, 7 months after discontinuing prednisolone and interferon-α, a focal hemangioma regrew from one of the previous lesions and was surgically resected. The expression and activity of type 3 iodothyronine deiodinase (D3) were elevated in the resected tumor tissue compared with placenta.

Discussion

Here, we describe a patient with consumptive hypothyroidism and diffuse infantile hepatic hemangiomas, one of which regrew after involution following pharmacotherapy. The etiology of elevated D3 activity is also discussed.

Conclusion

It is important to identify infantile hepatic hemangioma in patients with hypothyroidism refractory to hormone replacement therapy, who have low free triiodothyronine despite high thyrotropin and normal free thyroxine levels, and long-term follow-up will be needed for these patients.  相似文献   

6.

Background

Rickets is a complication of infantile osteopetrosis and pre-treatment recognition of this complication is important.

Objective

To describe four children with infantile osteopetrosis complicated by rickets (osteopetrorickets) and review the relevant literature.

Materials and methods

Retrospective chart analysis of four infants with osteopetrorickets and a systematic review of the relevant literature.

Results

We saw five children with infantile osteopetrosis, of whom four had superimposed rickets, for a period of 12 years. The review of the literature (including the current four children), yielded 20 children with infantile osteopetrorickets. The children ranged in age from 2 months to 12 months. In all children, hepatosplenomegaly was found. Sixteen (80%) children had visual impairments and eight (40%) children had hearing impairments. Serum calcium-phosphorus product was less than 30 in 18 children (90%). Twelve children (60%) were hypocalcemic and 18 (90%) were hypophosphatemic. In all children, the radiological examination demonstrated diffuse bony sclerosis and metaphyseal splaying and fraying of long bones. Five children (25%) had pathological fracture of extremities and 15 (75%) had rachitic rosary.

Conclusion

Rickets as a complication to infantile osteopetrosis is not uncommon. Skeletal roentgenograms are of critical importance in the diagnosis of both osteopetrosis and superimposed rickets.  相似文献   

7.

Purpose

We have evaluated the clinical, radiological and metabolic features of infantile urolithiasis (UL).

Materials and methods

We have reviewed the medical records of 93 children who were diagnosed as having UL before 1 year of age. We recorded patient demographics, the age at diagnosis, presenting symptoms, family history, the localizations and dimensions of stones, urinary metabolic examinations, as well as physical, laboratory, and radiologic findings. Our secondary objective was to compare some features of this group with those of older children with UL followed-up in the same clinic which were previously reported.

Results

We evaluated 93 children referred to our pediatric nephrology clinics. A family history of UL was 56.2 % in the study group. Resolution of stones was observed in 30.1 % of the cases. Urinary tract infections (UTIs) were detected in 65.9 % of females and 46.2 % of males. At least one urinary metabolic abnormality was found in 79.5 % of all the children. Most commonly seen metabolic abnormality was hypercalciuria. In all patients stones were located in kidneys except one infant who had an ureteral stone together with a kidney stone. Fifteen (16.1 %) children had an accompanying systemic disorder.

Conclusions

Among pediatric urinary stone diseases infantile UL can be regarded as a separate clinical entity. Coexistence of systemic disorders and anatomic anomalies at high frequencies may indicate a role of distinct pathogenetic mechanisms. In addition, high rates of UTIs and metabolic abnormalities in this age group justify screening for these parameters during follow-up of these children.  相似文献   

8.

Objective

To evaluate the efficacy, adverse effects, and recurrence of oral propranolol for treatment of infantile hemangioma.

Methods

Participants were treated with oral propranolol three times daily, with inpatient monitoring of adverse effects. The starting dosage was 2 mg/kg per day, which had been for the remaining duration of treatment. Therapy duration was planned for 4–6 months; if there was significant relapse, the period of treatment was extended. A photograph based severity scoring assessment was performed by three observers to evaluate efficacy by visual analog scale (VAS).

Results

Sixty-one infants [median age 3.3 (1.2–8.1) months] were included in the study. The median follow-up-time was 15 (6–20) months and 53 patients completed treatment at a median age of 10.3 (8.4–18.1) months, after a duration of 8.5 (4.5–14) months. In all patients, there was significant fading of color [with a VAS of ?9 (?6 to ?9) after 6 months] and significant decrease in size of the infantile hemangiomas [with a VAS of ?8 (?3 to ?10) after 6 months]. We did not observe any life-threatening adverse effects. The therapy was interrupted due to temporary aggravation of pre-existing bronchial asthma in one child. Four cases presented partial recurrences.

Conclusions

Oral propranolol 2 mg/kg per day was a well-tolerated and effective treatment, mild adverse effects, and low recurrence for infantile hemangiomas. Propranolol should now be used as a first-line treatment in hemangiomas when intervention is required. Also, prospective studies should be needed in determining the most effective treatment dosage, optimum treatment duration, and exact mechanism of action of propranolol in future.  相似文献   

9.

Background

Fibrosarcoma is a rare tumor in children with limited information on imaging features of these tumors in the literature.

Objective

To retrospectively review the imaging features of histologically proven congenital infantile fibrosarcoma.

Materials and methods

The list of histologically confirmed congenital infantile fibrosarcomas between November 1999 and June 2013 was obtained from the oncology-pathology database. Imaging features and pathology reports of these tumors were reviewed. Patient charts were reviewed and clinical features, management and outcomes were recorded.

Results

During the study period, 13 children (9 girls and 4 boys; age range: 0 day–16 months, median age: 2.5 months) with congenital infantile fibrosarcomas were available for complete radiological review. The translocation (t[12;15]) was present in 11/13 (84.6%) and absent in 2/13. Eight/thirteen (61.5%) tumors were located in extremities (5 in lower and 3 in upper), 3/13 in thoracolumbar paraspinal regions, and one each in abdomen and sternocleidomastoid muscle. Imaging features included iso- to hyperintensity on T1-W, hyperintensity on T2-W as compared to skeletal muscles and heterogeneous enhancement. Six (37.5%) tumors showed hemorrhagic components and 2 (15.4%) showed low intensity foci. None of the patients had evidence of regional or distant metastases at diagnosis. Management included surgical resection only (1/13) and combined surgery and chemotherapy (10/13). Overall survival was 100% with a median follow-up of 49.3 months.

Conclusion

Congenital infantile fibrosarcoma has nonspecific imaging characteristics but should be high on the differential diagnosis in a soft-tissue tumor presenting in infancy, located in an extremity and showing tumoral hemorrhage. Patients have a favorable outcome.  相似文献   

10.

Purpose

This study aimed to define morbidities and costs related to modern-day medical care for children with vascular anomalies.

Methods

We reviewed the 2003–2009 Kids’ Inpatient Database for pediatric patients (age < 21 years) hospitalized with hemangioma, arteriovenous malformation (AVM), or lymphatic malformation (LM). Patient characteristics, hospital complications, and hospital charges were compared by vascular anomaly type. Multivariable linear regression modeling was used to determine predictors of increasing hospital costs for patients with AVMs.

Results

In total, 7485 pediatric inpatients with vascular anomalies were identified. Frequently associated complications included chronic anemia (4.0%), sepsis (4.6%), and hypertension (2.4%). Children with AVM had the highest rate of in-hospital mortality, compared to those with hemangiomas or LM (1.0% vs. 0.1% vs. 0.3%, p < 0.001). AVMs were also associated with the highest median hospital charge, more than twice the cost for hemangiomas or LM ($45,875 vs. $18,909 vs. $18,919; p < 0.001).

Conclusions

There is a significant rate of morbidity in children with vascular anomalies, most often from blood loss and infection. The greater cost of AVM care may be related to the higher mortality rate, associated complications, and complexity of procedures required treating them. Cost-effective management of vascular anomalies should target prevention and the early recognition of both chronic comorbidities and acute complications.
  相似文献   

11.

Objective

Hemangiomas are the most common benign soft tissue tumors occurring in 5–10 % of children at around the age of 1 year and of them 10 % are associated with significant morbidity and entail medical attention. In this study, efficacy and adverse effects of the drug propranolol were observed on hemangiomas.

Methods

Oral propranolol was given to 36 children at a dose of 3 mg/kg/day in three divided doses. Blood pressure and heart rate were recorded during the first 3 h of treatment. Treatment was continued at home and the children were re-evaluated at monthly interval. Photographs were taken in pre- and post-treatment phages and changes of hemangiomas were measured with visual analog scale.

Results

Immediate effects on color and growth were noted in all cases that were especially dramatic in cases of extensive lesions. Clinical evidence of regression was seen within 30 days in all cases and completely regressed within 7 months. Mean duration of treatment was 4.1 months.

Conclusion

Propranolol had a rapid stabilizing effect leading to early regression of hemangiomas when administered orally at a dose of 3 mg/kg/day with good quality safety profile.  相似文献   

12.

Purpose

Primary resection is typically performed for children with localised suspected Wilms tumours. Resource limitation may necessitate performing these operations nights and weekends. We hypothesise that outcomes will be worse in patients having nephrectomies out-of-hours (OOH) compared to those in-hours (IH).

Methods

With IRB ethics approval, primary renal tumour resections performed on oncology patients from 1989–2011 were reviewed retrospectively. IH operations were defined as Monday–Friday 0745–1530 hours. Outcomes included major intraoperative complications, capsule rupture, and blood loss. Data were analysed using Fischer Exact and Mann–Whitney U tests.

Results

There were 64 patients with renal tumours who underwent primary resection. Forty-five procedures were performed IH, and 19 OOH. Groups were similar in age, ASA status, tumour size and grade. In a comparison of major intraoperative complications, capsule rupture, and mean blood loss, differences were 2 vs. 26 % (p = 0.007), 27 vs. 42 % (p = 0.12), 178 vs. 244 ml (p = 0.15) for IH and OOH respectively. There was one perioperative mortality (OOH).

Conclusions

Primary renal tumour resections performed OOH were associated with an increase in major complications compared to those performed in standard hours. Avoidance of OOH operating where possible may reduce morbidity for children undergoing primary renal tumour resections.  相似文献   

13.

Background

The indication and extent of cardiac screening before oral propranolol therapy (OPT) in patients with infantile hemangioma (IH) has been challenged. In this study, we evaluated pre-OPT cardiac diagnostics in a pediatric IH cohort in our department.

Methods

Retrospective chart review of infants ≤ 12 months old with IH undergoing OPT. The diagnostics prior to OPT, occurrence of complications, and outcome were recorded.

Results

A total of 234 patients were evaluated. The mean age at the onset of OPT was 4.2 ± 0.3 months, the average duration of OPT was 6.1 ± 0.1 months, and the average follow-up was 12.3 ± 0.7 months. Echocardiograms and electrocardiograms were performed prior to OPT in all patients. One hundred and three (44.0%) echocardiograms revealed pathological findings, 19 (8.1%) of which were minor (including atrial septal defects, pulmonary stenosis, and patent ductus arteriosus). Pathological findings were observed in 17 (7.3%) of electrocardiograms, only one (0.4%) of which was minor (suspected cardiac arrhythmia, subsequently excluded by long-term electrocardiogram analysis). These findings did not contraindicate OPT and no severe adverse events associated with OPT occurred during the follow-up period.

Conclusions

Routine cardiac screening by electrocardiogram and echocardiogram before OPT is debatable and not routinely indicated in children with IH. Further studies are necessary to draw definite conclusions on the reasonable indication and extent of this diagnostic approach.
  相似文献   

14.

Objective

To evaluate the efficacy and safety of 2% topical propranolol cream in the treatment of proliferating infantile strawberry hemangiomas.

Methods

A total of 40 infants were enrolled; 2% propranolol cream was applied three times daily. In the subsequent monthly visit, dynamic changes in tumor size, texture, and color were recorded. The adverse events (AEs) were observed. Treatment outcomes were scored on a four-point scale. All patients were followed up for 12 mo after treatment.

Results

The overall response was graded Scale 1 (poor response) in 2 patients, Scale 2 (moderate response) in 15 patients, Scale 3 (good response) in 17 patients, and Scale 4 (excellent response) in 6 patients. No significant differences were seen in treatment outcomes between female and male patients, among lesion locations/size, or in the age at the start of the treatment. No obvious AEs were reported.

Conclusions

2% topical propranolol cream is safe and effective for the treatment of proliferating infantile strawberry hemangiomas.
  相似文献   

15.

Purpose

This study analyzes how isolated hypoganglionosis (IH) is diagnosed and treated in Japanese pediatric surgical departments.

Methods

A second questionnaire was sent to 90 pediatric surgical departments that took part in an initial survey on variant Hirschsprung’s disease.

Results

Of 109 cases registered as having a certain diagnosis of IH, we targeted 90 patients and excluded the remaining 19. Symptom onset was neonatal ileus with a male:female ratio of 34:56. In most cases, results from radio-contrast enemas and suction rectal biopsies were normal. Anorectal manometry demonstrated that there was no relaxation in 73 % of the 37 patients examined. Furthermore, out of 55 patients who were examined intraoperatively, IH was diagnosed or suspected in 29 cases. Another 12 cases were eventually diagnosed with IH by other institutions using paraffin-embedded specimens. Stomas were initially created in 89 out of 90 cases and the mortality rate of the group that initially received jejunostomies was significantly lower than that of the ileostomy group (p < 0.05).

Conclusion

Preoperative diagnosis of IH is often difficult and intraoperative biopsies were only able to provide a correct diagnosis in 52 % of cases. More accurate diagnosis and the initial creation of jejunostomies rather than ileostomies may assist in improving patient management and outcomes.  相似文献   

16.
Chorioangioma is the most frequent nontrophoblastic tumor of the placenta with a incidence ranging from 0.01 to 1.3%. Vascular anomalies of the placenta coincidental with infantile hemangioendothelioma (IH) of the liver are rarely described. Here we report a case of a large chorioangioma of the placenta associated with cutaneous hemangiomatosis and IH of the liver. The relationship between hemangiomas and placental chorioangioma is discussed.  相似文献   

17.

Objective

To study the risk factors for hemangioma-related complications, treatment indications and analyze the outcome of patients with infantile hemangioma.

Design

Retrospective.

Setting

University hospital.

Patients

Fifty-five patients (1–69 months; median: 12 months) with infantile hemangioma with mean follow-up 19 months. The eligibility was based on the criteria of the International Society for the Study of Vascular Anomalies (ISSVA).

Intervention

The surgical treatment included total excision whereas medical treatment was carried out by interferon and /or corticosteroids.

Main outcome measures

Data was collected including sex, age, prematurity, age at onset, number, anatomic location and size of hemangioma, age at treatment, cause of treatment decision, family history, presence of extra malformations, involvement of internal organs, presence of life altering or life threatening complications, response to treatment, dose and duration of medications, complications associated with treatment, follow-up period, and final outcome.

Results

Thirty-four (62%) patients were followed-up without treatment, whereas 21 others underwent treatment including steroids, interferon, and surgery. The size of hemangioma was a major factor that predicted hemangioma-related complications (P=0.002). Patients with hemangioma related complications had bigger lesions (size ≥40cm2 or the longest size on a single plane ≥5 cm). Nineteen patients (34%) had complications, but only 8 (14.5%) out of them had life or function-threatening complications.

Conclusion

Although dosing and treatment protocol is still debatable, steroids and interferon are good options for hemangioma treatment. The management strategy should be individualized for each case.  相似文献   

18.

Background

Agenesis of the internal carotid artery (ICA) is a rare congenital anomaly occurring in less than 0.01% of the population, often incidentally discovered in pediatric populations. We recognized a high incidence of additional congenital malformations in children with ICA agenesis.

Objective

Our study reports nine cases of ICA agenesis and co-existent malformations and discusses implications of the association.

Materials and methods

We conducted a retrospective chart review of nine children evaluated at our institution with imaging findings of ICA agenesis.

Results

Seven children (78%) had congenital aortic or cardiac anomalies including coarctation (4), hypoplastic left heart (1), tetralogy of Fallot (1), and muscular ventricular septal defect (VSD) (1). Four children were diagnosed with an inherited disorder: Alagille syndrome (1), PHACE syndrome (1), VACTERL association (1), and methylenetetrahydrofolate reductase (MTHFR) gene variant (1). Additional congenital anomalies are also described.

Conclusion

In the setting of ICA agenesis, we report a robust association with congenital aortic and cardiac anomalies, as well as a broad spectrum of additional anatomical abnormalities that can occur in the setting of known genetic syndromes or as isolated findings. Knowledge of the natural history of ICA agenesis and associated anomalies will guide optimal care for these children.  相似文献   

19.

Purpose

It is common practice for premature infants undergoing elective inguinal hernia (IH) repair to be hospitalized for postoperative apnea monitoring. This study evaluated the risk of apnea after IH repair with regard to gestational age (GA) and postconceptional age (PCA) in formerly premature infants.

Methods

Formerly premature infants who had undergone elective IH repair between 01/2000 and 12/2012 were reviewed retrospectively in terms of GA, PCA, body weight, and comorbidities. All postoperative apneas were evaluated.

Results

A total of 428 formerly premature infant charts were reviewed. Eleven babies had postoperative apnea. Infants younger than 45 weeks PCA were found more prone to develop postoperative apnea after IH repair. In older infants (PCA between 46 and 60 weeks), comorbidities create predisposition to apnea postoperatively. These comorbidities are bronchopulmonary dysplasia, necrotizing enterocolitis and former apnea episodes. Anemia and lower birth weight are also risk factors.

Conclusion

This study suggests that low GA and PCA, low birth weight, anemia, and complicated past medical history affect respiratory complication rates, particularly apnea in formerly premature infants undergoing elective IH repair. Severe apneas occurred earlier than mild ones. Overnight monitoring is mandatory in small infants with low GA and PCA. Otherwise healthy, older infants may be operated on outpatient basis.  相似文献   

20.

Background

Infantile Blount disease is a developmental condition characterized by disorganized endochondral ossification in the medial aspect of the proximal tibial physis.

Objective

To describe the MR imaging abnormalities in the proximal tibia, distal femur, menisci and ligaments of children with infantile Blount disease.

Materials and methods

We retrospectively evaluated 11 children (18 total knee MR examinations) with infantile Blount disease and compared them with an age-matched control group with normal MR examinations. Morphological and morphometric measurements were performed.

Results

The medial menisci were enlarged with increased T2 signal intensity in all MR examinations. The medial femoral epiphyseal cartilage showed abnormal foci of increased signal intensity in nine (50%). The mid-coronal thickness of the medial tibial epiphyseal cartilage was decreased with concomitant increase in the mid-coronal joint space distance. Angular measurements of the proximal tibia demonstrated posteromedial down-sloping configuration.

Conclusions

Most severe abnormalities of infantile Blount disease occur in the medial compartment of the knee, especially at the medial tibial physis and epiphysis. However, other important structures of the knee and the lateral compartment are often affected. MR imaging helps to delineate the extent of multiple tibial and extra-tibial abnormalities, including meniscal abnormalities, perichondrial membrane changes and premature physeal closure.  相似文献   

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