Almost all of the epidural hemangiomas reported are cavernous hemangiomas. Purely extradural spinal capillary hemangiomas are very rare. Capillary hemangiomas are hamartomatous malformations that ...result from proliferations of vascular endothelial cells. Only ten cases have been reported in the English literature, treated with surgical excision.
A case of a dorsal extradural spinal capillary hemangioma is described. A total surgical removal has been performed after spinal angiography and embolization. Complete surgical removal should always be the goal in these lesions. Embolization did not show to reduce bleeding during the surgical procedure in this case.
Infantile hemangioma (IH) is the most common pediatric vascular tumor. Its pathogenesis is poorly understood but thought to represent an aberrant response of pluripotent stem cells to stimuli such as ...hypoxia and the renin-angiotensin system. IH usually appears during the first few weeks of life and follows a characteristic natural trajectory of proliferation and involution. Their clinical appearance depends on their depth and distribution. Classification comprises superficial, mixed, and deep IH as well as IH with minimal or arrested growth. Multifocal IHs are more likely to be associated with infantile hepatic hemangioma and, although the need for screening based on a specific number of IH has been recently debated, 5 remains the most widely acceptable cutoff point. Large facial IHs warrant investigation for posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects or aortic coarctation and eye anomalies (PHACE) syndrome. Lumbar IHs warrant investigation for lower body IH and other cutaneous defects, urogenital anomalies, ulceration, myelopathy, bony deformity, anorectal malformations, arterial anomalies, and renal anomalies (LUMBAR) syndrome. Complications of IH include ulceration, obstruction or functional impairment, hypothyroidism, and cosmetic sequelae. Differential diagnoses mostly consist of other vascular tumors and vascular malformations, although IH may sometimes mimic nonvascular tumors or developmental anomalies. Diagnosis is usually clinical and biopsy is rarely indicated. High-frequency ultrasonography may help with the differential diagnosis, particularly with subcutaneous lesions. Referral to other specialists may be required in specific cases.
Background
Hepatic hemangiomas (HHs) are benign liver lesions often discovered incidentally on imaging for various unrelated pathologies. We herein review the etiology, classification, diagnostic ...imaging, and management of HHs.
Methods
A comprehensive systematic review was performed utilizing MEDLINE/PubMed and Web of Science databases, with the end of search date being March 1, 2022, regarding HH diagnosis, imaging, and management.
Results
HHs can be broadly classified as capillary hemangiomas or cavernous hemangiomas. While the exact pathophysiology related to the development of HHs remains largely unknown, hormone exposure has been postulated to cause HH growth. HHs appear homogenously hyperechoic on US with distinct margins and posterior acoustic enhancement. While cavernous hemangiomas appear as well-defined hypodense lesions on pre-contrast CT images with the same density as the vasculature, one of the most reliable imaging features for diagnosing cavernous hemangiomas is high signal intensity on T2 weighted images. While most HHs are asymptomatic, some patients can present with pain or compressive symptoms with bleeding/rupture being very rare. Kasabach-Merritt syndrome is a rare but life-threatening condition associated with thrombocytopenia and microangiopathic hemolytic anemia. When HHs are symptomatic or in the setting of Kasabach Merritt syndrome, surgery is indicated. Enucleation is an attractive surgical option for HH as it spares normal liver tissue. Most patients experience symptom relief following surgical resection.
Conclusion
HHs are very common benign liver lesions. High-quality imaging is imperative to distinguish HHs from other liver lesions. Surgery is generally reserved for patients who present with symptoms such as pain, obstruction, or rarely Kasabach-Merritt syndrome. Surgery can involve either formal resection or, in most instances, simple enucleation. Patients generally have good outcomes following surgery with resolution of their symptoms.
Vascular malformations are thought to be monogenic disorders that result in dysregulated growth of blood vessels. In the brain, cerebral cavernous malformations (CCMs) arise owing to inactivation of ...the endothelial CCM protein complex, which is required to dampen the activity of the kinase MEKK3
. Environmental factors can explain differences in the natural history of CCMs between individuals
, but why single CCMs often exhibit sudden, rapid growth, culminating in strokes or seizures, is unknown. Here we show that growth of CCMs requires increased signalling through the phosphatidylinositol-3-kinase (PI3K)-mTOR pathway as well as loss of function of the CCM complex. We identify somatic gain-of-function mutations in PIK3CA and loss-of-function mutations in the CCM complex in the same cells in a majority of human CCMs. Using mouse models, we show that growth of CCMs requires both PI3K gain of function and CCM loss of function in endothelial cells, and that both CCM loss of function and increased expression of the transcription factor KLF4 (a downstream effector of MEKK3) augment mTOR signalling in endothelial cells. Consistent with these findings, the mTORC1 inhibitor rapamycin effectively blocks the formation of CCMs in mouse models. We establish a three-hit mechanism analogous to cancer, in which aggressive vascular malformations arise through the loss of vascular 'suppressor genes' that constrain vessel growth and gain of a vascular 'oncogene' that stimulates excess vessel growth. These findings suggest that aggressive CCMs could be treated using clinically approved mTORC1 inhibitors.
Cerebral cavernous malformations (CCMs) are a cause of stroke and seizure for which no effective medical therapies yet exist. CCMs arise from the loss of an adaptor complex that negatively regulates ...MEKK3-KLF2/4 signalling in brain endothelial cells, but upstream activators of this disease pathway have yet to be identified. Here we identify endothelial Toll-like receptor 4 (TLR4) and the gut microbiome as critical stimulants of CCM formation. Activation of TLR4 by Gram-negative bacteria or lipopolysaccharide accelerates CCM formation, and genetic or pharmacologic blockade of TLR4 signalling prevents CCM formation in mice. Polymorphisms that increase expression of the TLR4 gene or the gene encoding its co-receptor CD14 are associated with higher CCM lesion burden in humans. Germ-free mice are protected from CCM formation, and a single course of antibiotics permanently alters CCM susceptibility in mice. These studies identify unexpected roles for the microbiome and innate immune signalling in the pathogenesis of a cerebrovascular disease, as well as strategies for its treatment.
Infantile hemangiomas (IHs) are common neoplasms composed of proliferating endothelial-like cells. Despite the relative frequency of IH and the potential severity of complications, there are ...currently no uniform guidelines for treatment. Although propranolol has rapidly been adopted, there is significant uncertainty and divergence of opinion regarding safety monitoring, dose escalation, and its use in PHACE syndrome (PHACE = posterior fossa, hemangioma, arterial lesions, cardiac abnormalities, eye abnormalities; a cutaneous neurovascular syndrome characterized by large, segmental hemangiomas of the head and neck along with congenital anomalies of the brain, heart, eyes and/or chest wall). A consensus conference was held on December 9, 2011. The multidisciplinary team reviewed existing data on the pharmacologic properties of propranolol and all published reports pertaining to the use of propranolol in pediatric patients. Workgroups were assigned specific topics to propose protocols on the following subjects: contraindications, special populations, pretreatment evaluation, dose escalation, and monitoring. Consensus protocols were recorded during the meeting and refined after the meeting. When appropriate, protocol clarifications and revision were made and agreed upon by the group via teleconference. Because of the absence of high-quality clinical research data, evidence-based recommendations are not possible at present. However, the team agreed on a number of recommendations that arose from a review of existing evidence, including when to treat complicated IH; contraindications and pretreatment evaluation protocols; propranolol use in PHACE syndrome; formulation, target dose, and frequency of propranolol; initiation of propranolol in infants; cardiovascular monitoring; ongoing monitoring; and prevention of hypoglycemia. Where there was considerable controversy, the more conservative approach was selected. We acknowledge that the recommendations are conservative in nature and anticipate that they will be revised as more data are made available.
Infantile hemangiomas often are inapparent at birth and have a period of rapid growth during early infancy followed by gradual involution. More precise information on growth could help predict ...short-term outcomes and make decisions about when referral or intervention, if needed, should be initiated. The objective of this study was to describe growth characteristics of infantile hemangioma and compare growth with infantile hemangioma referral patterns.
A prospective cohort study involving 7 tertiary care pediatric dermatology practices was conducted. Growth data were available for a subset of 526 infantile hemangiomas in 433 patients from a cohort study of 1096 children. Inclusion criteria were age younger than 18 months at time of enrollment and presence of at least 1 infantile hemangioma. Growth stage and rate were compared with clinical characteristics and timing of referrals.
Eighty percent of hemangioma size was reached during the early proliferative stage at a mean age of 3 months. Differences in growth between hemangioma subtypes included that deep hemangiomas tend to grow later and longer than superficial hemangiomas and that segmental hemangiomas tended to exhibit more continued growth after 3 months of age. The mean age of first visit was 5 months. Factors that predicted need for follow-up included ongoing proliferation, larger size, deep component, and segmental and indeterminate morphologic subtypes.
Most infantile hemangioma growth occurs before 5 months, yet 5 months was also the mean age at first visit to a specialist. Recognition of growth characteristics and factors that predict the need for follow-up could help aid in clinical decision-making. The first few weeks to months of life are a critical time in hemangioma growth. Infants with hemangiomas need close observation during this period, and those who need specialty care should be referred and seen as early as possible within this critical growth period.
Objective: To evaluate the efficacy and safety of combined topical timolol and oral propranolol in treatment of infantile hemangioma. Patients and Methods: This prospective study was conducted in ...Basra teaching hospital in Basra, Iraq, where a total of 23 infants with 34 lesions of different types of hemangioma were included. All lesions were in proliferation phase. The infants’ ages were less than one year. They were treated with topical timolol solution 0.5% combined with oral propranolol in a period of 12 weeks (8-16 weeks). The response to treatment was evaluated clinically by using hemangioma score and Visual analogue scale. All infants followed 4-12 months after cessation of drugs. Results: By using hemangioma score system; the combined therapy showed that, 28 (82.4%) lesions showed excellent response, two (5.9%) showed good response, four (11.8%) showed fair response and none of the lesions showed poor response at end of 16 weeks. At the end of 4 months superficial hemangioma showed 100% reduction in their score, while the ulcerative hemangioma showed 94.7% reduction in their score while mixed hemangioma showed only 76.7% reduction in their scores. Complete resolution occurred in 50% of lesions after 4 months of treatment. Neither included infants developed serious side effects nor, any of the regressed hemangioma recur during the follow up. Conclusion: Combined topical Timolol 0.5% solution and oral propranolol seems to be a well-tolerated, safe treatment option with a good to excellent responses. The most responsive type of hemangioma to this combination are the superficial and ulcerative types.
Background/objectives
We sought to describe the experience among members of the Hemangioma Investigator Group with pulsed dye laser (PDL) in the treatment of nonulcerated infantile hemangioma (IH) in ...pediatric patients in the pre‐ and post‐beta‐blocker era.
Methods
A multicenter retrospective cohort study was conducted in patients with nonulcerated IH treated with laser therapy. Patient demographics, IH characteristics, indications for/timing of laser therapy, as well as laser parameters were collected. Responses to laser therapy were evaluated using a visual analog scale (VAS).
Results
One hundred and seventeen patients with IH were treated with PDL. 18/117 (15.4%) had early intervention (defined as <12 months of life), and 99/117 (84.6%) had late intervention (≥12 months of life). In the late intervention group, 73.7% (73/99) had additional medical management of their IH. The mean age at PDL initiation for the late intervention group was 46.7 ± 35.3 months of life (range 12–172 months) with total number of treatments to maximal clearing of 4.2 ± 2.8 (range 1–17). Those who received propranolol prior to PDL received fewer sessions (1.1 fewer sessions, approaching significance p = .056). On the VAS, there was a mean 85% overall improvement compared to baseline (range 18%–100%), with most improvement noted in erythema and/or telangiectasias. The incidence of adverse effects was 6/99 (6.1%).
Conclusions
PDL is a useful tool in the treatment of IH, with notable improvement of telangiectasia and erythema and low risk of complications. PDL is often introduced after the maximal proliferative phase.
OBJECTIVES:The aim of the present study was to document the clinical presentation, diagnostic studies, and therapy of gastrointestinal infantile hemangiomas.
METHODS:This is a retrospective analysis ...of children with gastrointestinal hemangiomas culled from our Vascular Anomalies Center database. We detailed the location of visceral and cutaneous tumors, as well as radiologic and procedural methods used for diagnosis and treatment.
RESULTS:A total of 9 of the 16 children (14 girls and 2 boys) with hollow visceral hemangiomas also had cutaneous lesions. The most common extravisceral sites were regional facial lesions (n = 6), multifocal lesions (n = 2), and a solitary chest lesion (n = 1). Presenting symptoms were melena and hematochezia in the first 4 months of life (n = 14); several infants required multiple blood transfusions. The most frequent locations were small bowel and mesentery. One-half of the patients (n = 8) were diagnosed by laparotomy; the majority (n = 12) had suspicious radiologic findings. Corticosteroid and/or propranolol were the most common therapies.
CONCLUSIONS:Melena and hematochezia, sometimes with profound anemia, in the first 4 months of life, suggest the possibility of intestinal infantile hemangioma even in the absence of cutaneous tumor. Intestinal bleeding, particularly in association with a regional facial lesion, should initiate workupultrasonography, computed tomography, and magnetic resonance imaging display diagnostic features. First-line treatment is medical management; bowel resection may be necessary, particularly for perforation.
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