Search

Despite its rarity, pulmonary capillary hemangiomatosis (PCH) presents a significant diagnostic challenge. Due to its similarity to other pulmonary vascular diseases, such as pulmonary veno-occlusive disease, it is characterized by abnormal pulmonary capillary proliferation, which is a rare cause of primary pulmonary hypertension. This case was the first reported instance of PCH in Shahid Rajaee Heart Hospital in Tehran, Iran, in 2023, which was confirmed by genetic testing. It highlighted the importance of considering PCH among the differential diagnoses for pulmonary hypertension, even in adolescent patients. The 13-year-old patient's main complaints were progressive exertional dyspnea and chest pain. He had no previous medical history and had not taken any pharmaceutical or herbal medications. Critical clinical findings included a heart murmur, an electrocardiogram revealing right ventricular hypertrophy, and echocardiogram evidence of pulmonary hypertension. The main diagnosis was PCH, as shown by CT findings of pulmonary artery dilatation and diffuse nodular ground glass opacities. Genetic tests indicated pathogenic EIF2AK4 mutations and suspicion of PCH. Therapeutic intervention included vasodilator therapy, which exacerbated the patient's condition. This case emphasized the importance of maintaining a high index of suspicion for rare causes of pulmonary hypertension, such as PCH. The outcome was to prepare the patient for lung transplantation. To differentiate PCH from other pulmonary vascular diseases, a combination of clinical presentation, radiologic studies, genetic analysis, and response to treatment is required to determine appropriate management, particularly lung transplantation., Competing Interests: None declared., (Copyright: © Iranian Journal of Medical Sciences.)

Background: Pulmonary capillary hemangiomatosis (PCH) is an idiopathic disease with the anomalous proliferation of a small capillary-like vessel in the pulmonary tissue, which can lead to a severe form of PH. There are only several cases of PCH described in veterinary literature: 27 cases in dogs and 2 cases in cats. In veterinary medicine, PH is mostly recognized as a consequence of left heart failure as a progression of the postcapillary PH to the precapillary form. PCH is mostly described as a primary disease, but resistant postcapillary PH with the high possibility of pulmonary edema raises speculation that PCH could be a secondary malformation to the left heart disease., Aim: Discover the features associated with the shift between left- and right-sided heart disease in the context of PH development., Methods: Retrospective analysis of materials from cats and dogs with histological markers of PCH (sPCH) versus those with right heart failure (RHF)., Results: Animals with histological and immunohistochemistry markers of PCH had a previous history of disease with left heart volume overload. There were no differences between the groups in radiography and gross pathology. Histologically, pulmonary fibrosis and arteriopathy could be found in RHF; in sPCH-a duplication of capillaries in alveolar septa and bizarre proliferation in surrounding structures., Conclusion: PCH could be a secondary pattern of vascular remodeling due to volume overload., Competing Interests: The authors declare that there is no conflict of interest.

Intramedullary spinal capillary hemangioma is a rare occurrence in pediatric patients, and only limited cases have been reported. This study presents the first two cases of spinal capillary hemangioma co-present with retained medullary cord and one case of spinal capillary hemangioma with lumbosacral lipomatous malformation. Previous literature on ten patients with this pathology was reviewed. We speculated pathogenesis, imaging features, and histopathologic findings of the disease., (© 2024. The Author(s).)

Klippel-Trenaunay syndrome (KTS) is a rare, congenital disorder typically emerging in early infancy or childhood. The classic presentation of KTS is distinguished by a triad of clinical features: a port-wine stain, early-onset varicosities and limb overgrowth. However, a notable variant of KTS has been documented, characterised by limb shortening rather than lengthening, occasionally referred to as 'inverse KTS'. This report details two cases that display this unusual presentation-both patients had classical features of port-wine stain and varicose veins but both experienced shortening of the affected limb. Whether these cases represent a variant of KTS or a new clinical syndrome altogether is uncertain. They however offer valuable insights into the nuances and breadth of clinical manifestations associated with this syndrome., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Hemangiomas, also called infantile hemangiomas (IH) or hemangiomas of infancy are the most frequently seen benign vascular tumors of infancy. Different types of hemangiomas are described in the literature. The current approach is to assess the risk and, if needed, first line treatment is to initiate systemic propranolol., Case Presentation: A 3-month-old Caucasian female patient was brought as an outpatient. The main complaint was an infantile hemangioma in the facial area, which as per the parents' story appeared within a week of birth like a small reddish line and it rapidly grew. Systemic propranolol was proposed as a first-line treatment and the adverse effects were explained. The parents, afraid of the side effects, wanted to explore other possibilities such as topical timolol, however, since it had no effect, propranolol was initiated in the end. Hemangioma was completely reduced in size; however, a skin defect was detected. As per the dermatologist's counsel, topical cream was initiated. The skin defect was reduced but not fully healed. The child is still being monitored periodically., Conclusion: After successful treatment of hemangioma, we identified a skin defect, which was very similar to steroid-induced skin atrophy. However, we cannot attribute this to a single factor. The only thing that can be concluded is that the subject needs a thorough studying, since rate of infantile hemangioma is high, and pediatricians need a clear management strategy of how to approach skin atrophy after successfully treating the hemangioma itself., (© 2024. The Author(s).)

Competing Interests: Competing interests: None declared.

Competing Interests: The authors have no conflicts of interest to disclose.

Background/objectives: Ulceration is a common complication of infantile hemangioma (IH). Severe, persistent ulceration occurs in a minority of patients. This study aims to characterize the clinical features of IH with aggressive ulceration (AU)., Methods: Multicenter retrospective study of clinical features of IH with AU., Results: Thirty-five patients with AU were identified and included in the study. The majority of AU occurred in segmental IH (23/35, 65%). Segmental IH with AU were large (≥10 cm 2 ; 16/23, 69%, p < .001) with a thin (<3 mm) superficial component (16/23, 69%, p < .001). Localized IH with AU had a thick (>3 mm) superficial component (11/12, 92%, p < .001). All diaper area IH with AU (9/35) were segmental with thin superficial component (100%, p = .02). IH with AU in the head/neck (10/35) were more commonly localized (67%) and mixed (62.5%), while segmental, thick superficial morphology was more common on trunk (9/35) and upper extremities (7/35)., Conclusions: IH resulting in AU differ in clinical features by anatomic site. Those in the diaper area are nearly always segmental with thin superficial component, whereas other sites tend to be localized, mixed, with thick superficial component. These distinct phenotypes may prove useful in the clinical setting for physicians to identify patterns of IH ulceration with increased risk of aggressive, persistent ulceration., (© 2023 Wiley Periodicals LLC.)

Infantile hemangiomas (IHs) are the most common pediatric vascular tumors, although their genetic etiology is largely unknown. Congenital capillary malformations (CMs) are associated with known somatic pathogenic variants, including GNAQ, GNA11, PIK3CA, and PIK3R1. Co-occurrence of a facial CM such as port wine stain and IH is not associated with any recognized vascular anomaly syndromes and rarely reported in the literature. We describe a case of a 5-week-old female patient with a large facial CM and extensive IHs of the lower lip, airway, and orbit who presented with airway compromise and responded to propranolol therapy., (© 2023 Wiley Periodicals LLC.)

Introduction: Spinal epidural capillary hemangioma is a very rare variety of tumors, usually with a predilection for the thoracic spine., Case Presentation: A 16-year-old female complained of hyperacute neurologic deficit progressed within hour, which presented by acute paraplegia, and loss of all sensations from her lower limbs up to her breasts. Neurologic exam revealed paralysis of lower limbs (0/5 on both legs) with a flaccid tone, absence of reflexes, weakness of the trunk with sensory level T4, bilateral flexion of plantar reflexes, and loss of sphincters' controls. Emergent magnetic resonance imaging showed a dumbbell-shaped epidural mass in the posterior aspect of the spinal canal at the T1-T2 level, measuring approximately 1.1 × 4.5 × 1.5 cm in size. The lesion was isointense on T1-weighted, hyperintense on T2-weighted, and a little enhancement after gadolinium administration. The surgery was obtained nearly 16 hours after paralysis, which eradicated the lesion with good hemostasis. Histological examination showed a well-organized vascular tissue that haphazardly arranged and confirmed the diagnosis of capillary hemangioma. Neurological improvement was quickly observed within days after surgery and further complete recovery was achieved 2 months after discharge., Conclusion: We report an extremely rare case of spinal epidural capillary hemangioma, where acute spontaneous hemorrhage in the lesion resulted in the hyperacute neurologic deficit within an hour. Since these are benign lesions, the immediate surgical intervention results in a very favorable prognosis and is considered the treatment of choice. Also, this case highlighted and rose the question of a better neurologic improvement in younger age patients with spinal cord injury., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Ulceration is an important complication in infantile hemangiomas (IHs). Prior to the use of β-blockers, the estimated incidence of this complication in a referral population was between 15% and 30%. The incidence and factors associated with ulceration have not been systematically studied since the emergence of β-blocker therapy., Objective: Examine the incidence and clinical predictors for ulceration in IHs., Methods: Retrospective study at tertiary referral centers., Results: Compared with a previous large pre-propranolol cohort study, ulceration occurred at a significantly lower incidence of 11.4%. Clinical factors associated with ulceration included partial segmental morphology, location in the diaper area, and size greater than 5 cm. Higher risk of ulceration in Black patients was observed, suggesting barriers to care including delayed diagnosis and referral to specialty care., Limitations: Retrospective design at tertiary referral centers., Conclusion: Compared with reports before the use of β-blockers became widespread, the incidence of ulceration in IHs has decreased. However, it continues to be a relatively frequent complication of IH., Competing Interests: Conflicts of interest Dr Fernández Faith is a consultant for Verrica Pharmaceuticals, Novartis, and Canfield Scientific. Dr Irene Lara-Corrales is a consultant for AbbVie, Janssen, Sanofi Genzyme, Johnson & Johnson, Pfizer, Pierre Fabre, Valeant, Loreal, Avicanna, Ipsen, Novartis, Eli Lilly, and Timber Pharma. Dr Pope is an advisory board member for AbbVie, Sanofi Genzyme, Johnson & Johnson, Pfizer, Pierre Fabre, Boehringer-Ingelheim, Novartis, and Timber Pharma. Dr Frieden is a consultant for Novartis and chairs the Data Safety monitoring board for Pfizer. Drs Shah, Witman, Harfmann, Bradley, and Hallagan and authors Braun, Keesari, and Jackson have no conflicts of interest to declare., (Copyright © 2022 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Spinal intramedullary capillary hemangioma is a rare condition. Although most spinal/spinal cord hemangiomas are inborn in origin, perinatal presentation is uncommon. We present a neonate with an intramedullary hemangioma, born with communicating hydrocephalus and complete paraplegia. Spinal imagining showed an intradural mass with hemorrhagic foci, a low-lying conus medullaris, and scalloping of dorsal elements. Ventriculoperitoneal shunting was performed in an emergent setting, with cerebrospinal fluid showing a brownish appearance with high viscosity which implied a possible old hemorrhage. Tissue sample was taken from the spinal lesion in an elective setting. Histopathological examination revealed capillary lobules and extralobular large vessels resembling abnormal veins. The mentioned clues suggested that this congenital hemangioma had become symptomatic from the prenatal period. Though it is a are event, it should be kept in mind while evaluating a neonate with communicating hydrocephalus and weakness of extremities with or without cutaneous hemangiomatous stigmata.

Background: Infantile hemangiomas (IHs) are vascular tumors that commonly affect infants and usually regress spontaneously or can be easily treated as an outpatient with topical beta-blockers. However, IHs that present in the airway may cause life-threatening symptoms due to airway obstruction or risk of bleeding. Here we present the first documented case of an infant with rapid deterioration and acute respiratory failure secondary to a lower airway hemangioma., Case Presentation: This 3-month-old male initially presented in respiratory distress with symptoms consistent with a viral respiratory infection, however showed no clinical improvement with standard therapies. An urgent CT scan revealed a mass occluding the right mainstem bronchus. Upon transfer to a tertiary care facility, he developed acute respiratory failure requiring emergent intubation and single lung ventilation. The availability of multiple subspecialists allowed for stabilization of a critically ill child, expedited diagnosis, and ultimately initiation of life-saving treatment with beta blockers. After 17 total hospital days, he was extubated successfully and discharged home in good condition., Conclusions: While IH is a rare cause of infantile respiratory distress, we present multiple pearls for the general pediatrician for management of IHs of the airway., (© 2022. The Author(s).)

Background/objectives: Infantile haemangiomas (IH) are common benign tumours of childhood. The current guideline recommended treatment is oral propranolol, the use of which for IH is relatively recent and there are no safety audits in Australian children published. As a result, it is a primarily inpatient initiated treatment. The aim of this study was to examine the short-term complication rates in infants treated with oral propranolol for IH., Methods: Retrospective case note review of IH patients initiated on oral propranolol admitted to the day-unit of tertiary metropolitan hospital in an Australia capital city, from January 2016 to December 2019., Results: Overall, 72 children were included in the study. Mean age at time of admission was 3.8 ± 2 months. Eight patients (11.1%) experienced complications during their initiation admission. Pulmonary complications (oxygen desaturation, wheeze, increased respiratory rate) was the most common type. No children required high-dependency or intensive care unit admission. The children who experienced complications had a mean age of 2.4 ± 1.2 months; t-test of equality of means found a relationship between propranolol-associated complication rate and age (p = 0.007). All other patient or admission characteristics were not associated with complication events (all p > 0.05)., Conclusions: Propranolol is a safe, effective and well-tolerated treatment in Australian children with IH. This study demonstrates younger infants will most likely benefit from inpatient initiation. More research needs to be done to characterise the risk profile of propranolol initiation for IH., (© 2022 The Australasian College of Dermatologists.)

Infantile hemangioma is the most common soft tissue tumor of infancy. Extensive organ involvement is rare. This report describes an infant with biopsy confirmed infantile hemangioma with diffuse organ involvement causing anemia and failure to thrive. Treatment was initiated with propranolol and led to initial improvement; however, course was complicated by several episodes of respiratory failure secondary to pulmonary edema. Propranolol therapy was interrupted for several months while patient was maintained on a diuretic regimen and treated with vincristine and high-dose corticosteroids. Patient was transitioned back to propranolol and is clinically thriving with objective improvement on radiographic imaging., (© 2022 Wiley Periodicals LLC.)

Purpose: To report a case of von Hippel-Lindau syndrome in a monocular patient with progressive vision loss due to a juxtapapillary capillary retinal angioma and an epiretinal membrane (ERM) formation., Patient: We describe a 37-year-old white male patient with von Hippel-Lindau syndrome who presented for retinal evaluation. The right eye was blind with no light perception vision. The left eye had a best-corrected visual acuity of 20/30 and exhibited a peripheral capillary hemangioblastoma at 12 o'clock and a juxtapapillary capillary hemangioma with an ERM covering the fovea. The patient underwent two sessions of fluorescein-potentiated argon laser treatment to the peripheral capillary hemangioblastoma with initial stabilization of vision. After 18 months of follow-up, the ERM contracted causing decline in vision to 20/50. Intravitreal injection of bevacizumab was given without improvement in vision or distortion. Twenty-five-gauge pars plana vitrectomy with ERM peeling and internal limiting membrane removal was performed with immediate improvement in vision and distortion., Conclusion: This case suggests that pars plana vitrectomy is a reasonable treatment option for vision loss due to a juxtapapillary capillary retinal angioma and ERM formation.

Background: In the stratification of potential causes of PH, current guidelines recommend performing V/Q lung scintigraphy to screen for CTEPH. The recognition of CTEPH is based on the identification of lung segments or sub-segments without perfusion but preserved ventilation. The presence of mismatched perfusion defects has also been described in a small proportion of idiopathic pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH). Dual-energy CT lung perfusion changes have not been specifically investigated in these two entities., Purpose: To compare dual-energy CT (DECT) perfusion characteristics in PAH and PVOD/PCH, with specific interest in PE-type perfusion defects., Materials and Methods: Sixty-three patients with idiopathic or heritable PAH (group A; n = 51) and PVOD/PCH (group B; n = 12) were investigated with DECT angiography with reconstruction of morphologic and perfusion images., Results: The number of patients with abnormal perfusion did not differ between group A (35/51; 68.6%) and group B (6/12; 50%) (p = 0.31) nor did the mean number of segments with abnormal perfusion per patient (group A: 17.9 ± 4.9; group B: 18.3 ± 4.1; p = 0.91). The most frequent finding was the presence of patchy defects in group A (15/35; 42.9%) and a variable association of perfusion abnormalities in group B (4/6; 66.7%). The median percentage of segments with PE-type defects per patient was significantly higher in group B than in group A (p = 0.041). Two types of PE-type defects were depicted in 8 patients (group A: 5/51; 9.8%; group B: 3/12; 25%), superimposed on PH-related lung abnormalities (7/8) or normal lung (1/8). The iodine concentration was significantly lower in patients with abnormal perfusion (p < 0.001) but did not differ between groups., Conclusion: Perfusion abnormalities did not differ between the two groups at the exception of a higher median percentage of segments with PE-type defects in patients with PVOD/PCH., Key Points: • Patchy perfusion defect was the most frequent pattern in PAH. • A variable association of perfusion abnormalities was seen in PVOD/PCH. • Lobular and PE-type perfusion defects larger than a sub-segment were depicted in both PAH and PVOD/PCH patients., (© 2022. The Author(s), under exclusive licence to European Society of Radiology.)

Objective: To present a rare case of a middle ear capillary hemangioma in an adult., Patient: A 31-year-old woman with a 6-month history of left ear fullness, pressure, tinnitus, and progressive hearing loss., Intervention: Endoscopic laser-assisted resection., Main Outcome Measures: Clinical, radiographic, and histopathological findings of a capillary hemangioma., Results: Otoscopy revealed an erythematous and slightly pulsating multilobulated middle ear retrotympanic mass. Her audiogram demonstrated a left-sided mixed hearing loss with air-conduction thresholds in the severe-to-profound range. Computed tomography (CT) imaging was significant for total opacification of the left middle ear and mastoid air cells. She underwent a combined endoscopic transcanal and transmastoid excision of the mass with ossicular chain reconstruction. A KTP laser was used to ablate and shrink down the periphery of the lesion. Pathology of the specimen was consistent with a capillary hemangioma. The patient's pulsatile tinnitus and spontaneous vertigo resolved postoperatively., Conclusions: Capillary hemangiomas are an uncommon cause of vascular middle ear lesions in adults and typically present with symptoms of aural fullness, pulsatile tinnitus, conductive hearing loss, otalgia, and vertigo. Surgery resection provides definitive treatment and the use of laser ablation techniques can allow for hemostasis and excellent visualization., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2021, Otology & Neurotology, Inc.)

Introduction: We report about a large retinal capillary hemangioma (RCH) with exudative retinal detachment and a macular fold, treated with Ruthenium-106 brachytherapy (Ru-106 BT) and scleral buckling surgery, followed by pars plana vitrectomy (PPV), for the removal of macular tractions., Case Description: A 17-year-old boy was referred to our Ocular Oncology Unit for a large RCH in the left eye. BCVA was hand motion. The RCH measured 4.9 × 6.85 mm in basal diameters and 4.0 mm in thickness and was located in the mid-peripheral temporal retina. It was surrounded by extensive subretinal exudation, forming an exudative retinal detachment, with a retinal fold that extended from the lesion to the optic disc. We performed Ru-106 BT and at the moment of the plaque removal we placed a radial buckle with the aim to unbend the retinal fold. At 3-months follow-up the exudation decreased, we achieved the opening of the peripheral side of the retinal fold, but the macula was still detached. We decided to perform a lens sparing PPV, macular peeling and air tamponade, to remove the vitreoretinal tractions ab interno and to try to complete the opening of the macular fold. After 1-month BCVA was counting fingers, the retina appeared attached, also in the macular area, but the retinal fold remained partially close in the macular side. After 6 months the tumor was inactivated, the macula remained attached, unfortunately, the macular fold remained partially close., Conclusion: Ru-106 BT and scleral buckling concurrent approach can be an effective treatment modality in selected cases of large RCHs, followed by PPV to remove eventual vitreo-retinal tractions.

Background: Oral propranolol is the first-line therapy for infantile hemangioma. Combining it with pulse dye laser (PDL) (595nm-long PDL) could reduce treatment duration and sequelae incidence and severity., Objective: To determine the effect of PDL-propranolol treatment on duration to cure and sequelae., Methods: All consecutive patients with infantile hemangioma who were cured by PDL-propranolol treatment were identified., Results: In the 27 cases, average age at treatment start was 4.3 ± 3.8 months, mean tumor diameter was 11.1 ± 14.0 cm2, and tumor-type was most common (72.4% of lesions). The patients received 9.8 ± 10.5 PDL sessions. After ensuring patients had no physical contraindications, including heart disease, oral propranolol was started at 1 mg/kg/d, increased up to 3 mg/kg/d as a maintenance dose. Mean propranolol treatment duration was 11.1 ± 4.9 months. Total treatment duration was 15.3 ± 10.8 months., Conclusion: Our data in the context of recent literature suggest combining propranolol with PDL may reduce propranolol duration without increasing harms., (Copyright © 2021 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Background: Capillary hemangioma can be found in many organs, but rarely in pleura. Previously, only localized pleural capillary hemangioma cases have been reported. Corticosteroids are the most commonly recommended drugs in capillary hemangioma., Case Presentation: Here, we present a case of a young woman with recurrent hemorrhagic pleural effusion. Despite repeatedly thoracentesis, the routine examinations, including chest computed tomography (CT) scan, pleural effusion biochemical test, and cytology all failed to make a definite diagnosis. Thus, single port video-assisted thoracoscopy (VATS) was then performed. Numerous nodules arising from the parietal pleura were found, and biopsies showed multifocal pleural capillary. However, recurrent pleural effusion was successfully managed by oral azathioprine, after failure of dexamethasone treatment., Conclusions: To our knowledge, this is the first case of a patient with recurrent hemorrhagic pleural effusion masquerading as malignant pleurisy, but in fact caused by multifocal pleural capillary hemangioma.

Importance: Ulceration is a common complication of infantile hemangioma (IH), which leads to substantial morbidity. Ulceration in IH has not been systematically studied since the advent of β-blocker therapy for IH., Objectives: To examine treatment interventions used for ulceration in IH and identify clinical prognostic indicators of healing time., Design, Setting, and Participants: A retrospective, multicenter cohort study was conducted on 436 consecutive patients with a clinical diagnosis of ulcerated IH and available clinical photographs. Patients receiving care at tertiary referral centers evaluated between 2012 and 2016 were included; statistical and data analysis were performed from February 7 to April 27, 2020., Exposures: Clinical characteristics, treatment interventions, course, complications, and resource use were analyzed. Treatment interventions for ulceration in IH included local (wound care, topical), systemic (β-blocker, corticosteroids), and procedural (pulsed-dye laser)., Main Outcomes and Measures: The primary end point was time to complete or nearly complete ulceration healing. Clinical characteristics were analyzed to determine the responses to most common interventions and prognostic factors for healing of ulceration., Results: Of the 436 patients included in the study, 327 were girls (75.0%); median age at ulceration was 13.7 weeks (interquartile range, 8.86-21.30 weeks). The median heal time was 4.79 weeks (95% CI, 3.71-5.86 weeks) with wound care alone, 5.14 weeks (95% CI, 4.57-6.00 weeks) with timolol, 6.36 weeks (95% CI, 5.57-8.00 weeks) with a systemic β-blocker, and 7.71 weeks (95% CI, 6.71-10.14 weeks) with multimodal therapy. After adjusting for IH size, a dose of propranolol less than or equal to 1 mg/kg/d was associated with shorter healing time compared with higher propranolol doses (hazard ratio, 2.04; 95% CI, 1.11 to 3.73; P = .02). Size of the IH was identified as a significant prognostic factor for healing time in multivariable analysis. Increasing size of IH portends a proportionately longer time to heal of the ulceration., Conclusions and Relevance: Despite the use of β-blockers, this cohort study found that a subset of patients with IH ulceration continued to experience prolonged IH healing times. Larger IH size appears to be a poor prognostic factor for time to heal. For patients requiring systemic therapy, initiation of propranolol at lower doses (≤1 mg/kg/d) should be considered.

In a 38-year-old female patient, who had been treated for acute visual impairment and suspected optic neuritis with no organic evidence in the right eye, an exophytically growing juxtapapillary retinal capillary hemangioma was found. The benign tumor is a rare but important differential diagnosis in cases of papilledema and macular exudation. The treatment is difficult; various therapeutic concepts are discussed.

Competing Interests: Competing interests: None declared.

Competing Interests: The authors have no conflicts of interest to declare.

Objective: Infantile hemangiomas (IHs) are the most common benign tumor in infancy. Periorbital IHs may lead to visual dysplasia so prompt and effective treatment should be adopted. In this study, we retrospectively analyzed the diagnosis and treatment of periorbital IHs treated in our clinic and give the strategy for clinical practice., Methods: From Jan. 2006 to Dec. 2018, 35 patients with periorbital IHs were enrolled into this study, including 13 males. The initial age of treatment ranged from 0.6 to 7 months. The clinical manifestations, imaging examination results, treatment measures and follow-up results were analyzed. Then diagnosis and treatment strategy were summarized., Results: All patients were examined by ultrasound to confirm the depth of tumor, evaluate the effect of treatment and provide the evidence for drug withdrawal. For patients with deep hemangiomas, enhanced CT were added. Intralesional injection of glucocorticoids and oral propranolol were applied. All patients achieved good results. Ocular symptoms were avoided in most of the patients., Conclusion: Periorbital IHs have special growth features and can be diagnosed by the appearance, ultrasound, CT scan, and propranolol experimental therapy if necessary. In consideration of potential complications, oral propranolol is the first treatment option for periorbital IHs., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) is one of the major complications in patients with CHD. A timely closure of the left-to-right shunt will generally result in the normalization of the pulmonary hemodynamics, but a few patients have severe prognosis in their early childhood. We hypothesized that wide-ranging pathological mechanism in PAH could elucidate the clinical state of severe CHD-PAH. Using electronic medical records, we retrospectively analyzed six infants with severe CHD-PAH who had treatment-resistant PH. All patients were born with congenital malformation syndrome. After starting on a pulmonary vasodilator, five of the six patients developed complications including pulmonary edema and interstitial lung disease (ILD), and four patients had alveolar hemorrhage. After steroid therapy, the clinical condition improved in four patients, but two patients died. The autopsy findings in one of the deceased patients indicated the presence of recurrent alveolar hemorrhage, pulmonary venous hypertension, ILD, and PAH. Based on the clinical course of these CHD-PAH in patients and the literature, CHD-PAH can occur with pulmonary vascular obstructive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH), ILD, and/or alveolar hemorrhage. The severity of CHD-PAH may depend on a genetic disorder, respiratory infection, and upper airway stenosis. Additionally, pulmonary vasodilators may be involved in the development of PVOD/PCH and ILD. When patients with CHD-PAH show unexpected deterioration, clinicians should consider complications associated with PVOD/PCH and/or pulmonary disease. In addition, the choice of upfront combination therapy for pediatric patients with CHD-PAH should be selected carefully.

Background: Splenic cord capillary hemangioma is a rare benign vascular lesion classified as a splenic hamartoma. On the other hand, non-islet cell tumor hypoglycemia (NICTH) is one of the rare causes of spontaneous hypoglycemia and is considered to be one of the paraneoplastic syndromes. To the best of our knowledge, this is the first reported case of a splenic cord capillary hemangioma with NICTH., Case Presentation: A 25-year-old male was referred to our hospital with hypoglycemia. Except for his low blood sugar, there were no abnormal findings from laboratory tests, which included an endocrinological examination. Enhanced computed tomography confirmed the presence of a solid mass measuring about 6 cm in the retroperitoneum, and a tumorectomy was performed. During this operation, it became clear that the tumor turned out to be a splenic parenchyma, and as a result, a total splenectomy was performed. Microscopically, we diagnosed this as a cord capillary hemangioma, and through immunohistochemistry, we found that some tumor cells were positive for insulin-like growth factor -II. Fortunately, the hypoglycemia-related symptoms disappeared after surgical resection was performed. The patient is still alive and well without evidence of local tumor recurrence 15 years after the operation., Conclusions: Splenic cord capillary hemangioma, one of the types of splenic hamartomas, is a very rare benign vascular lesion and might be associated with hypoglycemia thought to be NICTH.

A 71-year-old woman presented with spontaneous microhyphema in her left eye, causing blurry vision. Bleeding stopped spontaneously shortly after several cycles of digital compression on the upper eyelid, (which were documented in video), and therefore, did not require laser photocoagulation, a possible approach previously explained to the patient. A microhemangioma at the edge of the iris was identified to be the cause of the condition. The hemorrhage did not recur during the follow-up period (9 months)., Competing Interests: None

Case Presentation: A 37-year-old woman presented with a 2-month history of recurrent hemoptysis and coughing. Her symptoms started 2 months after the delivery of her third child. In total, she endured four episodes of hemoptysis. All pregnancies were induced by intracytoplasmic sperm injections. She lacked a pulmonary or smoking history and had no history of foreign body aspiration or intubation. There was no dyspnea, dysphagia, fever, or chest pain, and the patient did not complain of purulent sputum. She currently did not use medication and was generally in good health., (Copyright © 2019 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Purpose: To report a case showing a very rare association of chorioretinal coloboma and retinal capillary hemangioma in a previously healthy patient., Methods: Observational case report., Results: A 21-year-old woman presented at our clinic for vision screening. She had a history of bilateral chorioretinal coloboma with amblyopia in her left eye. Her best-corrected visual acuity was 20/25 in her right eye and 20/200 in her left eye. On slit-lamp examination, iris coloboma was observed in the left eye. Fundus evaluation showed inferior chorioretinal coloboma in both eyes, and an elevated, round, and orange-red retinal lesion with feeder vessels localized in the midperipheral region of the right eye. The patient was diagnosed as having solitary retinal capillary hemangioma and underwent argon laser therapy for treating the tumoral lesion. Diagnostic studies were negative for von Hippel-Lindau disease. This is the first reported case of solitary retinal capillary hemangioma associated with bilateral chorioretinal coloboma., Conclusion: Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Retinal capillary hemangioma is a vascular retinal tumor that may occur sporadically or as part of the von Hippel-Lindau syndrome. We report a rare association of chorioretinal coloboma and retinal capillary hemangioma in a previously healthy patient.