Isolated deep orbital hemangioma treated successfully with oral propranolol in a 2-month-old infant: Case report with literature review.
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ABSTRACT: Purpose
Infantile hemangiomas (IH) are the most common benign vascular tumors of infancy. Ocular complications due to orbital IH include strabismal, deprivational, or anisometropic astigmatism, and visual loss secondary to amblyopia.Observations
We report a case of a two-month-old female infant with a severe deep orbital IH. The diagnosis was established by clinical examination and magnetic resonance imaging (MRI). The patient was treated with oral propranolol for six months. Complete tumor regression was observed clinically and by MRI following the treatment. The patient showed no adverse effects or tumor recurrence at the 14-month follow-up.Conclusions and importance
Despite its benign nature, periorbital IH requires a rapid diagnosis and prompt treatment to prevent vision loss, particularly in infants. Our case confirms that oral propranolol may be regarded as a safe drug for periorbital IH with an excellent outcome.
SUBMITTER: Zvizdic D
PROVIDER: S-EPMC8082548 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:BackgroundPropranolol has become the first-line treatment for complicated Infantile Hemangioma (IH), showing so far a good risk-benefit profile.Case presentationWe report the case of a toddler, on propranolol, who suffered cardiac arrest during an acute viral infection. She had a neurally-mediated syncope that progressed to asystole, probably because of concurrent factors as dehydration, beta-blocking and probably individual susceptibility to vaso-vagal phenomena. In fact a significant history of breath-holding spells was consistent with vagal hyperactivity.ConclusionsThe number of patients treated with propranolol for IHs will increase and sharing experience will help to better define the safety profile of this drug.
| S-EPMC5693590 | biostudies-literature
Project description:The purpose of this study was to compare long-term neurocognitive functioning (working memory, processing speed, and attention) between children who had been treated with either propranolol or atenolol for infantile hemangioma during infancy. All eligible children (n = 158) aged 6 years or older and treated with propranolol or atenolol as infants were invited to participate in this two-center cross-sectional study. The primary outcome was the Wechsler Intelligence Scale for Children-V Cognitive Proficiency Index (CPI), a measure of working memory, processing speed, and attention. Secondary outcomes were general intelligence, auditory, visuospatial, and narrative memory, as well as executive functioning and sleep. A total of 105 children, of whom 36 had been treated with propranolol (age 6.0-11.8 years, follow-up time 1.6-9.7 years, 19% male) and 69 had been treated with atenolol (age 6.9-9.7 years, follow-up time 4.5-8.4 years, 19% male), were analyzed. The CPI and other neurocognitive outcomes did not differ between the propranolol and atenolol groups and were in line with general population test norms. Post hoc analyses revealed lower CPI scores for males, both compared to participating females (10.3 IQ points, medium effect size) and compared to matched test norms (12.4 IQ points, medium effect size).Conclusions Long-term neurocognitive functioning did not differ between children treated with propranolol and those treated with atenolol for IH. Overall, propranolol and atenolol appear to be safe treatments for IH regarding long-term neurocognitive functioning. The substantially lower CPI scores in males warrant further investigation.Trial registration Netherlands Trial Register, NL7703 https://www.trialregister.nl/trial/7703 What is Known: • Infants with infantile hemangioma are effectively treated with propranolol or atenolol. • Parents and professionals are concerned about long-term neurocognitive effects.What is new• No long-term (≥ 6 years) differences in neurocognitive functioning were found between children treated with propranolol or atenolol. • Males treated with beta-blockers had substantially lower IQ scores than treated females and males from the general population, which is a matter of concern and should be considered when evaluating the risk/benefit ratio in less severe forms of infantile hemangioma.
| S-EPMC9899165 | biostudies-literature
Project description:BackgroundPropranolol is used clinically to treat infantile hemangioma (IH), although the exact mechanism that underlies its effectiveness is not fully understood. The Jagged1/Notch signaling pathway is downstream of the β2-adrenergic receptor (β2-AR). Propranolol is a non-selective β2-AR blocker that was shown to inhibit demethylation adrenaline-induced Jagged1 expression. A previous study has shown that propranolol dose-dependently inhibits the growth of IH. However, the effects of propranolol on stemness of IH are not known and are thus addressed in the current study.MethodsWe analyzed the expression of Jagged1 and Notch3 in IH specimens, using genetic tools to alter Notch signaling. The transduced IH cells were treated with different doses of propranolol, and the effects on IH cell proliferation, migration, and potential for tumor sphere formation were investigated. The effects of altered Notch signaling on tumor formation in vivo were also assessed.ResultsNotch3 and Jagged1 were significantly upregulated in IH. Augmented Notch signaling in IH cells increased cell proliferation, migration, the potential for tumor sphere formation and in vivo tumor formation. On the other hand, reduced Notch signaling in IH cells decreased cell proliferation, migration, the potential for tumor sphere formation and in vivo tumor formation.ConclusionsJagged1/Notch signaling regulated the stemness of IH, and propranolol inhibited it through suppression of Notch signaling.
| S-EPMC8667105 | biostudies-literature
Project description:Infant mental development occurs in interplay with a caregiver. The infant establishes an inner world, a psyche, by using his or her caregiver as transitional mental space for the development of a sense of self. This mental progress occurs simultaneously with motor elaboration, pre-conditioned by neurophysiological maturation. The bodily holding function of the caregiver, through initial skin-to-skin contact, enables the infant to develop a sense of bodily self. The pivotal role of the body as a first place of ego development is illustrated by the vignette of Nino, a 3-month-old infant whose caregiver is unable to provide the necessary physical contact, and therefore insufficiently containing the young infant. This lack of physical holding limits the infant from developing a sense of bodily self, a primary sense of self. Without the caregiver's holding function, it is impossible for the infant to establish a relationship to a whole person.
| S-EPMC5518255 | biostudies-other
Project description:BackgroundInfants with infantile hemangioma (IH) have been effectively treated with propranolol or atenolol. Concerns were raised about the mental health of these children at school age, due to central nervous system effects of propranolol and visible nature of IH.ObjectiveThis study aimed to compare the mental health at school age of children treated with propranolol to children treated with atenolol for IHs and their parents.MethodsThis two-centered cross-sectional study included children aged ≥6 years and treated with either propranolol or atenolol for IH during infancy. Children's outcomes were performance-based affect recognition (Dutch version of the Developmental Neuropsychological Assessment-II [NEPSY-II-NL]), parent-reported emotional and behavioral functioning (Child Behavioral Checklist [CBCL]), and health-related quality of life (KIDSCREEN-27). Parents' outcome was parenting stress (Parenting Stress Questionnaire [OBVL]).ResultsData of 105 children (36 propranolol, 69 atenolol; 6.0-11.8 years) were analyzed. Mental health outcomes did not differ between both β-blocker groups. Although overall functioning was in line with norms, children presented specific problems concerning affect recognition, parent-reported attention, and social quality of life. Parents showed increased physical symptoms, depressive symptoms, and parent-child relationship problems.ConclusionNo difference in mental health at school age was found between children treated with propranolol or atenolol for IH. Although few overall mental health problems were found, specific problems require follow-up. Follow-up of children should be directed toward affect recognition, attention, and social functioning in daily life. Problems reported by parents could be ameliorated by mental health support during and after their infant's β-blocker treatment.
| S-EPMC10997238 | biostudies-literature
Project description:Infantile hemangiomas can cause significant morbidity during proliferation, yet there is no U.S. Food and Drug Administration-approved treatment. They are believed to form from hemangioma stem cells, which differentiate toward a hemangioma endothelial cell phenotype. Recently, propranolol has demonstrated effectiveness in treating complicated infantile hemangiomas. The authors hypothesize that propranolol facilitates their involution by altering cellular behavior in both hemangioma endothelial and stem cells.Hemangioma endothelial and stem cells were isolated from resected infantile hemangioma specimens. Cells were treated with 100 ?M propranolol for 48 hours, and apoptosis was determined by the presence of annexin V antibody. Proliferation of stem and endothelial cells was assessed after treatment with 50 or 100 ?M propranolol or vehicle, for 72 and 96 hours, respectively. Adipogenesis was induced in stem cells with and without propranolol. Pro-adipogenic genes PPAR?, PPAR?, C/EBP?, C/EBP?, C/EBP?, RXR?, and RXR? were analyzed by quantitative polymerase chain reaction.Annexin V levels were increased in propranolol-treated endothelial cells but not in stem cells. Proliferation of stem and endothelial cells was inhibited by propranolol in a dose-dependent manner. Propranolol-treated stem cells demonstrated accelerated adipogenesis when compared with untreated controls. Transcript levels of C/EBP? (p < 0.05), RXR? (p < 0.05), and PPAR? (p < 0.02) were significantly increased when treated with 50 or 100 ?M propranolol; and C/EBP? (p < 0.05), RXR? (p < 0.05), and PPAR? (p < 0.01) transcripts were increased when treated with 100 ?M propranolol. C/EBP? transcript levels remained unchanged at either dose.Propranolol increased apoptosis of hemangioma endothelial cells, but not stem cells, and accelerated adipogenesis of hemangioma stem cells. Thus, propranolol likely accelerates involution to fibrofatty residuum.
| S-EPMC3668332 | biostudies-literature
Project description:BackgroundCavernous hemangiomas, more accurately defined as cavernous venous malformations, constitute the most common primary intraorbital tumors of adults comprising 4-9% of all tumors,[4] and the second most frequent cause of unilateral proptosis after thyroid-related orbitopathy.[3] Over 80% are located within the intraconal compartment, most commonly in the lateral aspect.[1] Surgical treatment for orbital cavernous hemangioma is generally required in symptomatic cases, optic nerve compression, and cosmetically disfiguring proptosis.[2] Transcranial approaches, the most familiar approaches for neurosurgeons, provide wide access to the entire superior and lateral orbit. They usually offer direct visualization, allowing for a safer dissection, while minimizing significant injury to the native neural and vascular anatomy of the orbit.[5] Although transcranial approaches continue to evolve, in many cases, they have been supplanted by endoscopic skull base approaches and modifications to deep lateral orbitotomy approaches.[5].Case descriptionA 62-year-old male patient presented with slowly expanding left proptosis, which he had first noticed 3 years before presentation. He was already blind in his right eye due to a history of traumatic amaurosis in childhood. The left eye examination revealed severe proptosis with restricted eye movement in all directions and significant visual impairment (visual acuity of 20/300, expressed by Snellen test, with no improvement on correction). MRI of the orbit showed a large left superolateral intraconal cavernous hemangioma compressing and displacing the optic nerve, with the typical feature of slow gradual irregular enhancement with delayed washout on contrast-enhanced image. A one-piece modified orbitozygomatic approach was performed and a total en block resection was achieved. The bone flap was fixed with titanium miniplates and screws, the temporal muscle and the skin were closed in a standard fashion. The patient did not present any new deficit in the postoperative period. The patient had good functional and cosmetic outcomes with resolution of proptosis, restoration of eye movements, and improvement of visual acuity in the 3-month follow-up. Postoperative MRI showed total resection.ConclusionThe orbitozygomatic approach for large orbital cavernous hemangioma provides satisfactory orbital decompression and large working space, reduces traction, and increases visualization and freedom to dissect small vessels and nerves that may be tightly attached to the tumor pseudocapsule.
| S-EPMC8326111 | biostudies-literature
Project description: Not available
| S-EPMC10601783 | biostudies-literature
Project description:Infantile hemangioma is a common tumor of infancy. Although most hemangiomas spontaneously regress, treatment is indicated based on complications, risk to organ development and function, and disfigurement. The serendipitous discovery of propranolol, a non-selective β-adrenergic receptor blocker, as an effective means to regress hemangiomas has made this a first-line therapy for hemangioma patients. Propranolol has shown remarkable response rates. There are, however, some adverse effects, which include changes in sleep, acrocyanosis, hypotension, and hypoglycemia. Over the last few years, researchers have focused on understanding the mechanisms by which propranolol causes hemangioma regression. This has entailed study of cultured vascular endothelial cells including endothelial cells isolated from hemangioma patients. In this article, we review recent studies offering potential mechanisms of how various cell types found in hemangioma may respond to propranolol.
| S-EPMC4580045 | biostudies-other
Project description:Purulent pericarditis is uncommon among paediatric patients and cases caused by group A Streptococcus (GAS) are even rarer. We report a four-month-old female infant who was referred to the Royal Hospital, Muscat, Oman, in 2015 with pericardial effusion and cardiac tamponade. She had initially presented to a secondary hospital with a two-week history of fever, a runny nose and shortness of breath. Blood and pericardial fluid cultures confirmed GAS isolates. The infant was treated with a two-week course of antibiotics and made a complete recovery with no echocardiographical evidence of pericardial effusion at a two-month follow-up. To the best of the authors' knowledge, this case constitutes the youngest infant to present with GAS pericarditis. As invasive GAS infections can present in infancy, early recognition and treatment is required.
| S-EPMC5488831 | biostudies-other