Noninvoluting congenital hemangiomas with hypovolemic shock, anemia and prolonged jaundice in a neonate: a case report.

Noninvoluting congenital hemangioma is an extremely rare congenital anomaly in newborn babies and may complicate life-threatening events, including hemorrhage. We present a very rare case of noninvoluting congenital hemangiomas in an Asian and Afghan six-day-old girl that was detected at birth. The noninvoluting congenital hemangiomas were accompanied by hypovolemic shock, anemia, and prolonged jaundice during the first two weeks of life. These diseases were diagnosed by medical history, physical examination, blood analysis, and doppler ultrasonography of the skin lesions. Her hypovolemic shock and hemorrhage were improved after supportive treatment, and she was discharged from the hospital in a good condition. After four weeks of life, the hemangiomas did not regress or progress, suggesting noninvoluting congenital hemangiomas. Hemangioma may complicate severe hemorrhage and shock, as well as there may be an association between congenital hemangioma and prolonged neonatal jaundice.

Pediatric Vascular Anomalies: A Clinical and Radiological Perspective.

According to the International Society for the Study of Vascular Anomalies (ISSVA) classification, vascular anomalies include a diverse range of pathologies, classified as either vascular tumors or vascular malformations. This classification, last revised in 2018, aims to explain the biological basis of vascular lesions and help clinicians to manage the anomalies. In vascular tumors, there are proliferative changes of endothelial cells, while vascular malformations primarily consist of structural vascular abnormalities. Infantile hemangioma is the most common soft-tissue vascular tumor. Vascular malformations are an extensive group of malformations of the arterial, venous, and lymphatic systems, either in isolation or in combination. Radiological evaluation plays a key part in the management of pediatric patients with these entities. The understanding of sonography and magnetic resonance imaging findings entails its correlation with clinical findings at the time of scanning.

Hemangioma infantil. Reporte de un caso de presentacion clinica anular

Reportamos el caso clínico de un lactante menor con una presentación anular de hemangioma, que nos plantea dudas en su clasificación. Se precisan los diagnósticos diferenciales y la necesidad de la determinación del marcador GLUT 1 en hospitales públicos.

We report the clinical case of a young infant with an annular presentation of hemangioma, which raises doubts regarding its classification. Differential diagnoses and the need to determine the GLUT 1 marker in public hospitals are specified.

Hemangioma infantil. Reporte de un caso de presentacion clinica anular / Infantile hemangioma. Report of a case of annular clinical presentation

Reportamos el caso clínico de un lactante menor con una presentación anular de hemangioma, que nos plantea dudas en su clasificación. Se precisan los diagnósticos diferenciales y la necesidad de la determinación del marcador GLUT 1 en hospitales públicos.

We report the clinical case of a young infant with an annular presentation of hemangioma, which raises doubts regarding its classification. Differential diagnoses and the need to determine the GLUT 1 marker in public hospitals are specified.

Late third trimester diagnosis of congenital giant hemangioma complicated by the Kasabach-Merritt phenomen: a case report and literature review.

Objective. To describe the case of a large cervical mass diagnosed in the late third trimester with development of Kasabach-Merritt phenomenon (KMP) in the immediate postnatal period, along with a literature review.Methods. Description of case-report and literature search through Medline/Pubmed, performed from inception to December 2022 for articles relating to the pre and postnatal diagnosis of KMP.Results. A 36-year-old multiparous woman was admitted to hospital for contractions at 40 weeks of gestation, in an otherwise uneventful pregnancy. Admission's ultrasound showed the presence of a voluminous mass of 14x15 cm of the posterior side of the neck, highly vascularized, and no signs of hemodynamic imbalance. Postnatally, blood tests showed the presence of severe anemia and thrombocytopenia requiring several transfusions of blood, plasma, platelets and clotting factors. Due to the association of congenital hemangioma and thrombocytopenia a diagnosis of KMP was made. After attempts of conservative treatment, surgical removal was needed to stop the hematological cascade with regression of symptoms. The review of the literature identified 14 articles including 9 cases of prenatally suspected KMP and 6 diagnosed in the immediate postnatal period and without signs of fetal hydrops. Adverse perinatal outcome, in terms of postnatal death/termination of pregnancy, was observed in 67% of cases (6/9) in the prenatally suspected group and 33% of cases in those with a postnatal diagnosis of KMP. Fetal hydrops was present in 83% of cases with adverse perinatal outcome.Conclusions. The Kasabach-Merrit syndrome is a rare condition, which can have a dangerous evolution when it develops in utero or in the immediate postnatal period carrying a risk of perinatal mortality of approximately 50%. Even if the fetus shows no signs of anemia or heart failure, the risk of developing it in the immediate postnatal period is high and should be mentioned to the couple.

A multi-step approach to the treatment of giant scalp congenital hemangiomas: a report of two cases.

This is a review of two cases of neonatal giant scalp congenital hemangioma. Both patients were treated with propranolol using a similar multi-step approach that included transarterial embolization of the supplying arteries followed by surgical resection of the lesion. In this report, we discuss the treatments, complications, and clinical outcomes of interventions and surgical procedures.

Pediatric hepatic vascular tumors: clinicopathologic characteristics of 33 cases and proposed updates to current classification schemes.

Pediatric hepatic vascular tumors (HVTs) are rare neoplasms with features distinct from their cutaneous counterparts. Their behavior ranges from benign to malignant, with each subtype having therapeutic differences. Histopathologic descriptions of large cohorts are scarce in the literature. Thirty-three putative HVTs diagnosed from 1970 to 2021 were retrieved. All available clinical and pathologic materials were reviewed. Lesions were reclassified according to the World Health Organization (WHO) classification of pediatric tumors [1] as hepatic congenital hemangioma (HCH; n = 13), hepatic infantile hemangioma (HIH; n = 10), hepatic angiosarcoma (HA; n = 3), and hepatic epithelioid hemangioendothelioma (HEH; n = 1). Vascular malformations (n = 5) or vascular-dominant mesenchymal hamartoma (n = 1) were excluded. HCH frequently showed involutional changes, whereas HIH often had anastomosing channels and pseudopapillae formation. HA had solid areas with epithelioid and/or spindled endothelial morphology, significant atypia, increased mitoses, high proliferation index, and occasionally necrosis. On morphology analysis, a subset of HIH showed features worrisome for progression to HA including solid glomeruloid proliferation, increased mitoses, and epithelioid morphology. The widely metastatic and fatal HEH was observed in a 5-year-old male with multiple liver lesions. Immunohistochemically, HIHs and HA were Glucose transporter isoform 1 (GLUT-1) positive. One HIH patient died from postoperative complications, whereas 3 are alive without disease. Five HCH patients are alive and well. Two of three HA patients died of disease, and 1 is alive without recurrence. To our knowledge, this is the largest series of pediatric HVTs reviewing clinicopathologic features based on current Pediatric WHO nomenclature [1]. We highlight diagnostic challenges and propose inclusion of an intermediate category between HIH and HA which warrants closer follow-up.

Case report: Transcatheter arterial embolization in a newborn with cervical rapidly involuting congenital hemangioma and Kasabach-Merritt phenomenon.

Congenital hemangiomas (CHs) are rare vascular tumors and do not exhibit progressive postnatal growth. The incidence is less than 3% of all hemangiomas. Most CHs have a favorable prognosis; however, the Kasabach-Merritt phenomenon (KMP) is a rare but life-threatening complication in CHs that requires aggressive treatment. Medical treatments with corticosteroids and interferon have been suggested. Surgical resection can be considered for the treatment of complicated CHs in medically resistant lesions. Vascular embolization could be an alternative method if surgery is not considered feasible. Herein, we report a case of a 9-day-old newborn who underwent arterial embolization for a CH with KMP, combined with sirolimus treatment, and the outcome was favorable. The hemangioma completely regressed by 3 months and rapidly involuting congenital hemangioma (RICH) was diagnosed. Our successful experience with treating RICH associated with KMP revealed that RICH can have potentially serious complications although they usually resolve rapidly after birth without treatment. Surgical resection is considered to be the standard method for the treatment of medically resistant vascular tumors, but it is difficult to perform during the active phase of KMP due to acute bleeding and severe coagulopathy. Arterial embolization is feasible and can be used as an alternative to surgical resection, even in small babies.

Treatment of Symptomatic Focal Hepatic Hemangioma with Propranolol in Neonates: Is It Efficient?

Hepatic hemangiomas (HH) - classified into congenital hepatic hemangiomas (CHH) or infantile hepatic hemangiomas (IHH) - are benign vascular tumors that are mainly asymptomatic, but may cause clinical problems that require treatment. While focal, multifocal, and diffuse IHH are responsive to propranolol treatment, CHH is mainly focal and thought to be resistant to treatment with propranolol. The clinical and imaging distinctions between CHH and IHH in cases of focal lesions can be challenging, while histopathological distinction is mostly lacking in the clinical setting. We report 4 neonatal symptomatic cases of focal HH treated with propranolol, with partial or complete resolution of the tumor, and the positive hemodynamic effect of propranolol in one case. We believe that although clear differentiation cannot be achieved between CHH and IHH without histopathological examination in cases of focal HH in neonates, propranolol treatment should be attempted in symptomatic cases since its benefits outweigh the possible small risk of side effects of propranolol.

Vascular Anomalies: Other Vascular Tumors.

Vascular tumors are classified into three categories by the International Society for the Study of Vascular Anomalies (ISSVA): benign, locally aggressive/borderline, and malignant. Many of these tumors are rare, cutaneous in nature, and present in childhood. The characterization and delineation of these distinct vascular tumors is an evolving area of clinical research. The diagnosis of these lesions relies on history and clinical presentation, location, histologic appearance, immunohistochemistry, and more recently, associated genetic mutations. This article provides a brief, yet comprehensive overview of all cutaneous vascular tumors currently recognized by the ISSVA, including presentation, diagnosis, and treatment.

Vascular anomalies in childhood: When to treat and when to refer.

Vascular anomalies are heterogeneous conditions that affect blood and/or lymphatic vessels. Affected children may experience pain, functional loss, infection, coagulopathies, and psychological challenges. Diagnosis and management often warrant an interdisciplinary approach. There are seven vascular anomalies clinics in Canada that offer interdisciplinary care. This practice point outlines a treatment approach for the most common paediatric vascular anomaly (hemangioma). It reviews indications for referral to a specialized clinic, with focus on complex vascular anomalies, specifically infantile hemangioma, which can pose complications.

Preterm neonate with a large congenital hemangioma on maxillofacial site causing thrombocytopenia and heart failure: A case report.

BACKGROUND: We report a rare case of a large congenital hemangioma (CH) in the maxillofacial region in a female neonate that caused thrombocytopenia and heart failure. With close multidisciplinary collaboration, the congenital hemangioma was successfully resected with good results. CASE SUMMARY: The patient was delivered at gestational age of 36 wk by cesarean section due to cephalopelvic disproportion and lack of onset of labor (birth weight: 2630 g). A right-sided facial tumor was detected in the fetus during routine antenatal ultrasound examination of the mother at 32 wk of gestation. Physical examination revealed a 7 cm × 7 cm × 3 cm hard, dull purple-colored mass on the right maxillofacial region. The mass was tense and had prominent surface telangiectasias. Laboratory investigations revealed reduced hemoglobin and platelet count, and increased activated partial thromboplastin time, prothrombin time, and thrombin time. International normalized ratio, fibrin degradation products, and D-Dimer levels were significantly increased. Thromboelastography showed increased alpha angle, mean amplitude, and the clot formation speed. Thyroid-stimulating hormone level was significantly elevated. The patient was administered prednisone, propranolol, euthyrox, vitamin K1, milrinone, and digoxin. After operation, cefepime was administered for anti-infection and propranolol was prescribed at discharge. CONCLUSION: We report a rare case of CH in the right maxillofacial region causing thrombocytopenia and heart failure.

Conservative management of congenital hepatic hemangioma complicated by ascites.

Involution of a rapidly involuting congenital hemangioma is an unknown cause of neonatal ascites. As involution phase is completed by 14 months after birth, conservative management with diuretics and drainage is possible and may avoid surgical resection.

Symptomatic congenital hemangiomatosis in a neonate: Imaging of a life-threatening presentation with multifocal liver involvement.

Hemangiomas are the most common benign vascular neoplasms of infancy. Congenital hemangiomas proliferate in utero, and are fully formed at birth. They are usually solitary. Generalized forms are exceptional. The liver is the second most common site of hemangiomas after the skin. When >5 cutaneous hemangiomas are present, screening abdominal ultrasound is recommended. Based on the degree of liver parenchyma involvement, 3 hepatic hemangiomas' subtypes are defined: focal, multifocal, and diffuse. Hepatic hemangiomas' clinical presentation varies from asymptomatic to life-threatening. High output cardiac failure, consumptive coagulopathy, abdominal compartment syndrome, and liver dysfunction are possible complications. We report an unusual case of symptomatic congenital hemangiomatosis in a male infant born with innumerable generalized cutaneous hemangiomas whose screening abdominal ultrasound revealed multifocal hepatic hemangiomas with extensive mixed shunts. We aim to highlight this unique entity with severe associated complications and stress the role of imaging at initial presentation, for follow-up, and to guide therapeutic choices.

Non-involuting congenital hemangioma with delayed hypertrophy: A case series.

BACKGROUND: Noninvoluting congenital hemangiomas (NICH) are rare and poorly understood vascular tumors that are present at birth, characterized by lack of growth after birth and lack of involution. We report uncharacteristic cases of NICH hypertrophy occurring later in life. METHODS: This is a case series describing the clinical presentation, management, and histologic characteristics of two cases of NICH hypertrophy. RESULTS: Two patients with a NICH of the scalp experienced lesion hypertrophy in teenage or early adult life. Case 1 is a 14-year-old female who presented with a flat left parietal scalp lesion that at first grew slowly with the patient; however, over the span of months grew substantially resulting in an exophytic lesion. The patient had the lesion surgically excised. Case 2 is a 26-year-old female with NICH of left occipital scalp and posterior neck who noted new nodules on the inferior border of the lesion. MRA/MRI showed extension into the occipital calvarium, level V of the neck, and paraspinal musculature. The patient elected to observe given the extent of the lesion and her minimal symptoms. CONCLUSION: Although postnatal growth of NICH have been described, cases usually occur during the pre-adolescent period where growth is usually proportional to overall growth of the patient. This study describes two cases of rapid onset NICH hypertrophy occurring later in life. Knowledge of the potential for delayed hypertrophy may lead families to seek earlier intervention or opt for more definitive interventions. Additionally, recognition of these variable distinctions will contribute to a better understanding of CH and its various subtypes.

[The most common benign cutaneous neoplasms of vascular, muscular, and adipose tissue and their treatment]. / Häufigste benigne Gefäß­, Muskel- und Fettgewebstumoren der Haut und ihre Therapie.

Benign cutaneous vascular and cutaneous neoplasms arising from muscle or adipose tissue are rare. Their definition and classification are still controversially discussed. Histopathological and immunohistochemical studies, together with the new genetic tests, contribute significantly to the correct description of the various benign neoplasms of cutaneous origin. Clarifying whether a lesion is benign is fundamental for prognosis and therapeutic management. In most cases, surgical excision is the treatment of choice. Dermatologists should be familiar with the classifications of the most common benign cutaneous neoplasms of vascular, muscular, and adipose tissue and be able to recommend the procedures currently used for an accurate diagnosis. Ensuring that a lesion is benign and a good surgical outcome are essential for the patient.

Early postnatal proliferation of rapidly involuting congenital hemangioma.

Congenital hemangiomas (CH) are benign vascular tumors characterized by being fully developed at birth and following a variable postnatal course. We present the case of a newborn with a CH who experienced proliferation before developing an ulceration followed by rapid involution. This case expands the clinical spectrum of CH, highlighting the variable course of this vascular tumor.

Treatment of superficial benign vascular tumors by high intensity focused ultrasound: Observations in two illustrative cases.

BACKGROUND: Existing therapeutic methods for reduction or removal of superficial vascular malformations and tumors have high risks of scarring and other complications that result in aesthetic appearance less favorable than the baseline. Patients are often cautioned against intervention, which can lead to psychosocial problems and low self-esteem. Improved treatment modalities are therefore relevant from both medical and aesthetic perspectives. METHODS: Two volunteer subjects were treated with a medical 20 MHz high intensity focused ultrasound device developed for dermatological conditions. One patient was given three treatments to remove a superficial congenital hemangioma on the left middle cheek. The other patient was given a single treatment to remove seven cherry angiomas on the thighs. Handpieces with nominal focal depths of 0.8 - 1.8 mm below the skin surface were used to administer acoustic energy of 1.1 - 1.2 J/dose. An integrated dermoscope in the handpiece was used to monitor the treatment in real-time. RESULTS: During treatment, blood in the capillary network of the lesions was coagulated immediately, and capillary walls were collapsed due to the thermal and mechanical effects of the high intensity focused ultrasound. During the healing phase, the areas regenerated a normal skin structure with very limited scar or dyspigmentation. At follow-up, a clear aesthetic improvement was observed over the baseline for all treated targets with the exception of two cherry angiomas, where focal depth and/or dose coverage had not been optimal. CONCLUSION: High intensity focused ultrasound is concluded to be a safe and efficient skin treatment for benign superficial vascular malformations and tumors.