Pulmonary Capillary Hemangiomatosis as a Rare Underlying Cause of Primary Pulmonary Hypertension: A Case Report in an Adolescent.

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.

Adult transverse sinus capillary hemangioma: case report and review of the literature.

BACKGROUND: Adult intracranial capillary hemangioma (ICH) is an extremely rare disease with very few cases reported in the literature. Natural history is poorly understood and therapeutic management has not been clearly defined. METHODS: Using PRISMA guidelines, we systematically reviewed all published adult cases of ICH, to which we added our own case. CASE PRESENTATION: A 24-year-old patient with intracranial hypertension underwent stenting for left transverse sinus ICH. Recurrence was managed by subtotal resection, and radiotherapy was undertaken after a second relapse. Radiotherapy achieved complete response at last follow-up. RESULTS: Among cases of ICH found in the literature, 36 concerned adults (55.3%), with clear female predominance, and only 2 cases affecting the transverse sinus (8.1%). Adult ICHs grew over time, and pregnancy seemed to accelerate natural progression. Complete resection was achieved in most cases, and radiotherapy was used in case of relapse, with high rates of tumor control. CONCLUSION: Transverse sinus ICH is uncommon, and venous stenting is a suitable option in case of tumor invasion to treat intracranial hypertension. Surgery is the gold-standard treatment, and radiotherapy is a very effective alternative, with high rates of tumor control. Natural history is not benign, particularly in case of pregnancy.

Malignant rhabdoid tumor of the orbit in an infant.

We present the case of an infant with rapidly progressing orbital tumor that had initial radiological and clinical features of both rhabdomyosarcoma and capillary hemangioma. The patient was eventually diagnosed with malignant rhabdoid tumor of the orbit. We discuss the salient histological and radiological features of our case and review the literature on orbital malignant rhabdoid tumors.

Long-Term Results of Oral Propranolol Treatment Protocol for Periocular Infantile Hemangioma: Should There Be Any Contraindication?

PURPOSE: The authors report long-term results of the oral propranolol treatment protocol for periocular infantile hemangioma regardless of conventional indications. METHODS: This is a retrospective study (2008-2018) on children with propranolol treatment protocol for periocular infantile hemangioma and last follow-up (FU) of ≥4 months after treatment and age of >24 months. After systemic evaluation, it was started with 0.5 and increased by 0.5 every 3 days to reach 2 mg/kg/day. If there was no good response, it would be gradually increased up to 3 mg/kg/day. Tapering (0.5 mg/kg/day every 4 weeks) was started when patients were on treatment for ≥6 months and ≥12 months old and there was no change in the infantile hemangioma size for ≥3 months. Rebound growth was based on the parents' report and would be treated by increasing the dose to at least 2 mg/kg/day for 3 months. FU was either in-person or via social media. Change in periocular infantile hemangioma size on the photos was scored by 3 masked observers (visual analog scale) and presented as excellent (≥50% reduction), good (1%-49%), fair (no change), and failure (enlarged). RESULTS: Forty-three patients were included. Orbital involvement was in 49%, ptosis in 58%, and risk of amblyopia in 63%. Mean treatment, tapering, and FU duration were 37, 13, and 74 months, respectively. Mean age at presentation, start of propranolol treatment protocol, tapering, stop, and last FU were 1.5, 5, 29, 42, and 78 months, respectively. Twenty-seven patients received 2, seven 2.5, and nine 3 mg/kg/day. Mean visual analog scale significantly increased from 43% (FU1) to 92% (last FU) when 97.6% of patients showed an excellent response. The remaining skin lesions were observed in 35%. No side effect was reported. CONCLUSIONS: Long-term results of the propranolol treatment protocol for periocular infantile hemangioma showed an excellent response in 97.6% of patients with no side effects. A residual skin lesion was observed in 1/3 of the patient.

Documented growth of an intracranial capillary hemangioma: A case report.

Intracranial capillary hemangiomas in adults are rare, and diagnosis can be challenging. Hemangiomas, in general (and particularly in the skin), are more often noted in the pediatric population. Due to the lack of imaging undertaken in the presymptomatic phase, the literature provides few clues on the rate of growth of these unusual tumors. Therefore, we report a case of a 64-year-old man with a medical history of Lyme disease who presented with exhaustion and confusion. Imaging demonstrated an intra-axial lesion with vascularity in the posterior right temporal lobe, raising the possibility of a glioma. Imaging two years prior revealed a very small lesion in the same location. The patient underwent a craniectomy, total resection of the lesion was completed, and his symptoms of confusion resolved. Biopsy revealed a capillary hemangioma composed of small vascular channels lined by endothelial cells and pericytes without smooth muscle. Features of glioma, vascular neoplasms or neuroborreliosis (cerebral Lyme disease) were not identified. Our case documents the growth over two years of a rare intracranial capillary hemangioma in an older adult male.

Capillary Haemangioma of Gingiva - A Rare Lesion.

Capillary Haemangioma is a benign vascular tumour characterized by proliferation of blood vessels with a very striking similarity to pyogenic granuloma with a predilection to occur on the gingiva. Though the head and neck are the most common region of occurrence, but is considered to be rare occurring on the gingiva, intra-orally. The lesion clinically mimicked pyogenic granuloma but was histopathologically diagnosed as capillary haemangioma. It was surgically excised and followed up for 6 months without any recurrence.

[Infantile hemangioma : Clinical manifestation, treatment, and differential diagnoses]. / Infantile Hämangiome : Manifestationsformen, Therapie, Differenzialdiagnosen.

With an incidence of approximately 4% infantile hemangiomas are the most common vascular tumors in children and show characteristic growth dynamics. In order to avoid erroneous treatment, they need to be differentiated from other vascular tumors (granuloma pyogenicum and kaposiform hemangioendothelioma) and vascular malformations. Of all infantile hemangiomas 85% are uncomplicated and undergo spontaneous resolution starting towards the end of the first year of life. First-line treatment for complicated infantile hemangiomas (15%), i.e. those with imminent obstruction (eyes and nose), ulceration or permanent disfigurement, is oral propranolol (2 mg/kg BW and day for at least 6 months).

Clinical and Hemodynamic Responses to Imatinib in Pulmonary Veno-Occlusive Disease/Pulmonary Capillary Hemangiomatosis: A Retrospective Pilot Study of Five Cases and Review of the Literature.

BACKGROUND: Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare types of pulmonary arterial hypertension with dismal prognoses; there is no established medical treatment for these conditions. Possible efficacy of imatinib against these conditions has been reported in 15 cases; however, how and in whom imatinib is effective remain unknown. METHODS: We retrospectively evaluated clinical data from consecutive patients with PVOD/PCH treated with imatinib at our institution. The diagnosis of PVOD/PCH was established using the following criteria: pre-capillary pulmonary hypertension; diffusion capacity of the lung for carbon monoxide < 60%; and two or more high-resolution computed tomography findings of interlobular septal thickening, centrilobular opacities, and mediastinal lymphadenopathy. The dose of pulmonary vasodilators remained unchanged during the assessment of imatinib. RESULTS: The medical records of five patients with PVOD/PCH were reviewed. The patients were aged 67 ± 13 years, their diffusion capacity of the lung for carbon monoxide was 29 ± 8%, and their mean pulmonary artery pressure was 40 ± 7 mmHg. Imatinib was administered at 50-100 mg/day; consequently, the World Health Organization functional class improved in one patient. In addition, imatinib improved the arterial oxygen partial pressure in this and another patient (these two also experienced a decreased mean pulmonary artery pressure and pulmonary vascular resistance after imatinib usage). CONCLUSIONS: This study indicated that imatinib improves the clinical condition, including pulmonary hemodynamics, of some patients with PVOD/PCH. In addition, patients with a certain high-resolution computed tomography pattern or PCH-dominant vasculopathy may respond favorably to imatinib.

Vitrectomy-Assisted Excision in the Treatment of Juxtapapillary Retinal Capillary Hemangioma.

Juxtapapillary retinal capillary hemangiomas are sight-threatening hamartomas located on or adjacent to the optic nerve. Nonsurgical approaches including laser photocoagulation and cryotherapy have been shown to be effective to reduce exudation in peripheral hemangiomas. However, in juxtapapillary hemangiomas, the functional outcomes are limited due to associated potential damage of the retinal nerve fiber layer. We present an 18-year-old female patient with von Hippel-Lindau (VHL) disease who presented with a juxtapapillary retinal capillary hemangioma associated with a tractional epiretinal membrane (ERM) and secondary macular hole. After vitrectomy-assisted excision of the lesion and inner limiting membrane (ILM) peeling around the macular hole, visual acuity and macular anatomy were recovered at 10 months of follow-up. [Ophthalmic Surg Lasers Imaging Retina 2022;53:570-573.].

Monitoring oral propranolol for infantile hemangiomata.

Treating infantile hemangiomas with oral propranolol may be initiated in accordance with various protocols some require hospitalization. However, different adverse events have been reported during treatment, thus it is of special importance to find a protocol which is both safe and feasible. We performed a retrospective cohort study of all cases of infantile hemangiomas treated with oral propranolol at our institute between January 2010 and February 2020. Pretreatment evaluation consisted of pediatric cardiologist evaluation including electrocardiography and echocardiography. The propranolol starting dosage was 0.5 mg/kg bid; 2 weeks later the dosage was escalated to 1 mg/kg bid. During the initiation and escalation visits, heart rate and blood pressure were measured before and every hour for a total of 3 h, and blood glucose level was measured within the first hour of treatment. A total of 131 children were treated during the study period. Scalp, facial and genital region infantile hemangiomas were more prevalent in regard to their relative body surface area. No symptomatic bradycardia, hypotension, hypoglycemia, or any other adverse events were documented; few patients had asymptomatic bradycardia and hypotension, which were more common in infants below 6-months of age. Only one patient had asymptomatic hypoglycemia, not requiring any intervention. Initiation and escalation of propranolol treatment for infantile hemangiomas proved to be safe, and without symptomatic adverse effects. However, considering the young age of the patients and the possible asymptomatic adverse reactions, we recommend the following simple protocol as presented, for pretreatment evaluation and short monitoring during treatment initiation and dose escalation.

Development of an artificial intelligence algorithm for the diagnosis of infantile hemangiomas.

Prompt and accurate diagnosis of infantile hemangiomas is essential to prevent potential complications. This can be difficult due to high rates of misdiagnosis and poor access to pediatric dermatologists. In this study, we trained an artificial intelligence algorithm to diagnose infantile hemangiomas based on clinical images. Our algorithm achieved a 91.7% overall accuracy in the diagnosis of facial infantile hemangiomas.

Lumbar Spinal Epidural Capillary Hemangioma: A Case Report and Literature Review.

BACKGROUND Capillary hemangiomas are often seen on the skin of young individuals and are rarely found in the spine. These vascular lesions can arise from any spinal compartment, although they are more commonly found in the intradural extramedullary (IDEM) than the epidural location. We present a unique case of a woman with a histologically proven spinal epidural capillary hemangioma (SECH). The imaging and histopathological characteristics, as well as the treatment strategy of this vascular lesion, are highlighted along with a comprehensive review of the literature. CASE REPORT A 38-year-old woman presented with progressively worsening low back pain that radiated to both legs. Neurological examination revealed a weakness of the left leg without sensory loss. Magnetic resonance imaging (MRI) demonstrated an epidural tumor at L1-L2 level, making an obtuse angle with the cerebrospinal fluid (CSF) on sagittal T2-weighted images. The patient underwent a complete tumor resection without complications or recurrence. The histology revealed a capillary hemangioma. CONCLUSIONS SECH is exceedingly rare, with only 22 cases in the reported literature. Females are more commonly affected than males, and the thoracic spine is more commonly involved than the lumbar spine. SECH often mimics other epidural and IDEM lesions, leading to misdiagnosis. MRI is useful to differentiate SECH from lesions in the various spinal compartments; additionally, MRI is essential for preoperative planning and patient surveillance. Preoperative embolization is an option given the high vascularity of SECH. Surgery is the mainstay treatment, with a good prognosis, in most cases without recurrence.

Intradural extramedullary capillary hemangioma with intramedullary component: A case series.

RATIONALE: Capillary hemangioma in the spinal cord is an exceedingly rare entity that is liable to be misdiagnosed. To summarize the clinical presentation, radiological characteristics, and pathological features of capillary hemangioma in the spinal cord and to share our experience for the diagnosis and treatment of intradural extramedullary capillary hemangioma. PATIENT CONCERNS: Three patients underwent surgical treatment at our hospital between January 2020 and August 2020. All patients were male[median age at presentation: 57 years (range: 56-60)]. Two patients were experiencing pain and numbness in the lower back, and 1 patient was experiencing numbness and weakness in the left lower limb. The duration of symptoms ranged from 1 to 5 months. DIAGNOSIS: All patients were diagnosed with spinal cord capillary hemangioma after treatment. All lesions were in an intradural extramedullary location and involved spinal cord components. Two patients had lesions in thoracic segments (T8, Th9-10), and 1 patient had a lesion in lumbar segment (L1). INTERVENTIONS: All patients underwent microscopic laminectomy and complete resection of the extramedullary and intramedullary components of the spinal cord capillary hemangiomas. OUTCOMES: Postoperatively, all patients experienced transient numbness and pain in the lower limbs, which gradually decreased over time. None of the patients experienced bleeding, severe numbness or pain, or recurrence of symptoms at 3-month follow-up. CONCLUSION: Intradural extramedullary capillary hemangioma has unique morphological characteristics. Gross-total resection of the extramedullary and intramedullary components of spinal cord capillary hemangioma is recommended for patients with symptoms of spinal cord compression. Careful preoperative imaging and intraoperative decision-making may help avoid residual lesions or reoperation.

Spinal epidural capillary hemangioma: A systematic literature review and an illustrative case.

BACKGROUND: Spinal hemangiomas are rare vascular malformations resulting from proliferation of vascular endothelial cells. The cavernous form is the most common and represents 5-12% of spinal vascular malformations, while the capillary form is rare. CASE DESCRIPTION: A 56-year-old patient with no past medical history presented with progressive spinal cord compression symptoms localizing to the T10 level with MRC grade 4 proximal paraparesis. Preoperative MRI demonstrated a well-delineated, dumbbell-shaped, epidural lesion, without bony involvement, resulting in spinal cord compression at the T7 and T8 levels. The patient underwent gross total surgical resection of the lesion. At the one month follow up, the patient's strength improved to MRC grade 5, and sensation had fully returned. The histopathological diagnosis was a capillary hemangioma. Exclusively epidural capillary hemangiomas are extremely rare with only 26 cases reported in the literature. They are mainly located at the thoracic level (T4-T6). The MRI features include a well-circumscribed mass, hyperintense on T2-weighted sequence in 92% of cases, isointense on T1-weighted sequence in 88% of cases, and homogeneous contrast enhancement in 100% of cases. No tumor recurrence has been observed after gross total surgical removal. CONCLUSIONS: When evaluating progressive spinal cord compression by a purely epidural spinal lesion, the differential diagnosis should include capillary hemangioma, in addition to schwannoma, meningioma, and lymphoma. Early and complete surgical removal is the first line treatment.