Diffuse Neonatal Hemangiomatosis Research Articles

Diagnóstico clínico de hemangiomatose neonatal disseminada: um relato de caso

Hemangioma is the most common vascular tumor of childhood. It may present as multiple cutaneous hemangiomas on rare occasions, with or without visceral involvement. The condition of lesions restricted to the skin is called benign neonatal hemangiomatosis, and in the case of visceral lesions associated with cutaneous lesions, the denomination is disseminated or diffuse neonatal hemangiomatosis (DNH). For the diagnosis of DNH, three criteria must be met: onset in the neonatal period, involvement of three or more organs, and absence of malignancy in hemangiomas. ASAS, a full-term, vaginally delivered newborn, weight: 3560g, length: 52cm, head circumference: 35cm, APGAR: 9/9. Maternal history of syphilis during pregnancy - mother adequately treated in pregnancy. At birth, she presented with purpuric lesions of variable size on the chest, limbs, feet, and hands, which discolored under digital pressure; larger lesions with relief, not scaly. He developed severe heart failure and was admitted to the Neonatal Intensive Care Unit (NICU) in a secondary hospital in the Federal District. Imaging tests were performed: Computed tomography of the skull, thorax, and total abdomen, which revealed the presence of a possible hemangioma in the left frontal lobe, telangiectasias in the lungs, and multiple hepatic hemangiomas. He had a clinical diagnosis of DNH. The case described corroborates the fact that knowledge about the disease is relevant, as early diagnosis can lead to more accurate approaches that may reduce morbidity and mortality from the disease.

A case report of hemorrhagic presentation of diffuse neonatal hemangiomatosis (DNH) associated to obstructive hydrocephalus: Medical and neurosurgical considerations

BackgroundDiffuse neonatal hemangiomatosis (DNH) is a rare disorder typically recognized at birth or during the neonatal period. DNH involves three or more organ systems, including the central nervous system (CNS). In these cases, serious complications such as hemorrhages and obstructive hydrocephalus can develop.Case reportWe present a case of DNH with intracranial hypertension and CNS hemorrhagic lesions on the mesencephalic aqueduct, resulting in triventricular hydrocephalus, treated with endoscopic ventriculocisternostomy (ETV) and medical therapy.DiscussionDNH is a rare condition that can involve the CNS with serious complications. From a review of the literature, we highlighted only two cases of DNH with brain involvement treated surgically. We report the successful outcome of ETV, along with surgical considerations, imaging findings, and the complete resolution of cerebral and skin lesions following medical therapy.ConclusionsMedical therapy is not standardized and must be individualized. In patients who develop severe neurological symptoms such as obstructive hydrocephalus, surgery may be considered to avoid neurological sequelae.

Efficacy of intravenous propranolol for life‐threatening diffuse neonatal hemangiomatosis

Oral propranolol is recommended as the first-line agent for infantile hemangioma requiring systemic treatment. Life-threatening complications such as obstructive infantile hemangioma of the airway or infantile hepatic hemangioma associated with high-output congestive heart failure are major indications for the consideration of early treatment. We present the case of an infant with life-threatening diffuse neonatal hemangiomatosis, including airway obstruction due to subglottic hemangioma, heart failure due to multiple hepatic hemangiomas with portohepatic venous shunts, and severe anemia due to continuous gastrointestinal bleeding, in which treatment with intravenous propranolol proved successful.

Diffuse neonatal hemangiomatosis presenting as congestive cardiac failure - A case report

Infantile hepatic hemangioma has substantial arteriovenous shunting which may lead to cardiovascular compromise and hydrops fetalis. It may present as hepatomegaly since the entire liver is involved in most cases. As mortality is very high, a high index of suspicion is required to make a diagnosis and common complications arising out it, especially in the presence of cutaneous hemangioma. We present a 2-month-old baby born at term presented with features suggestive of sepsis with multiple cutaneous hemangiomas, and on evaluation, there was congestive cardiac failure, which was initially thought of cardiac origin but subsequently came out to be arteriovenous shunting of blood in liver.

Neonatal Diaphragmatic Hemangioma associated with Diffuse Neonatal Hemangiomatosis treated with Coil Embolization

Neonatal Diaphragmatic Hemangioma associated with Diffuse Neonatal Hemangiomatosis treated with Coil Embolization

Diffuse neonatal hemangiomatosis presenting as congestive heart failure.

Diffuse neonatal hemangiomatosis is a rare condition with a very high mortality rate if left untreated. We report a neonate having around 490 cutaneous and multiple diffuse liver hemangiomas presenting as congestive heart failure. Prompt treatment was instituted with decongestive drugs and prednisolone for anticipated life threatening complications due to hepatic hemangiomas. Propranolol was added later as it is known to precipitate congestive failure and also to avoid long-term complications of steroids. The child responded well. However, there is no consensus regarding the initial line of management, which needs to be individualized keeping in mind the efficacy, long-term side effects of the drug and the clinical presentation.

Diffuse Neonatal Hemangiomatosis with Unilateral Cranial Nerve Palsy Improved by Propranolol

Diffuse neonatal hemangiomatosis (DNH) is characterized by multiple capillary or cavernous hemangiomas on the skin and internal organs occurring during the neo­ natal period. It is a life­threatening condition due to high­output heart failure with a mortality rate of 60­85% without proper treatment. The areas that are most commonly involved include the skin (100%), liver (64­100%), and central nervous system (52%). Corticosteroids are the drugs of choice as an initial treatment and have a response rate of 30­60%. We present here a case of a newborn baby with multiple hemangiomas on her skin (scalp, lips, neck, back, shoulder, arm, buttock, and leg), brain (right cere­ bellum, pons, and medulla oblongata), lungs, liver, kidney, and bones. She suffered from 6, 7, 9, 10, and 12 cranial nerve palsy resulting from hemorrhage of the hemangiomas in the brain. The first­line treatment of prednisolone (4 mg/kg/day) was not effective and propranolol (2 mg/kg/day) was administered as a second­line treatment. After 2 weeks of treatment, the hemangio mas had decreased in size with no associated acute hemorrhage. The infant is now 10 months old and both the multiple hemangiomas and cranial nerve palsy have improved. Propranolol was effective without significant adverse effects in treating DNH resistant to corticoste­ roids.

A Case of Diffuse Neonatal Hemangiomatosis Successfully Treated with Propranolol

We present a case of a 41-day-old girl with diffuse hemangiomatosis on her chin, right thigh, occipital area, and liver. She also had hepatomegaly, heart failure, and hypothyroidism. Hemangiomatosis did not respond to 2 weeks of oral administration of prednisolone, but was successfully treated with propranolol. After 2 years of propranolol treatment, the lesions of hemangiomatosis nearly disappeared. Hypothyroidism was controlled by levothyroxine replacement. As hemangiomatosis regressed, thyroid function was normalized. Propranolol may have adverse effects including hypotension, hypoglycemia, bronchoconstriction, and restlessness, but she did not experience such adverse effects. Propranolol could be the first choice for diffuse neonatal hemangiomatosis (DNH). Our case also suggests that thyroid function test is needed in patient with DNH.

Successful Propranolol Treatment of Multiple Infantile Hemangiomas with Predominant Neuroaxial Involvement: a Case Report

Infantile hemangiomas (IH) are the most common vascular tumor of infancy. Occurrences during the neonatal period are divided in multiple congenital, limited to skin involvement, or diffuse neonatal hemangiomatosis (DNH). DNH is considered to be a benign and rare disease, determined by the presence of multiple cutaneous and extracutaneous hemangiomas. The encephalic and medullary involvement of IHs is unusual and rarely reported. The authors report a case of infantile hemangioma (IH) as diffuse neonatal hemangiomatosis with predominant central nervous system involvement on a neonate, treated with propranolol. We discuss the use propranolol as main therapy and review the available data regarding IHs with neuroaxial involvement and its treatment.

Multisystemic Langerhans Cell Histiocytosis Mimicking Diffuse Neonatal Hemangiomatosis

We report the first case of multisystemic Langerhans cell histiocytes mimicking diffuse neonatal hemangiomatosis clinically. This has been described in patients with congenital self-healing reticulohistiocytosis but not in patients with acute, disseminated, and multisystemic disease. In our experience, dermoscopic findings did not help to diagnose the condition.

Diffuse neonatal hemangiomatosis with partial response to propranolol

International Journal of DermatologyVolume 53, Issue 4 p. e247-e250 Correspondence Diffuse neonatal hemangiomatosis with partial response to propranolol Lara Ferrandiz PhD, Corresponding Author Lara Ferrandiz PhD Dermatology Unit, Hospital Universitario Virgen Macarena, Seville, Spain Dr. Lara Ferrandiz, phD Dermatology Unit Hospital Universitario Virgen Macarena Avda, Dr. Fedriani s/n 41073 Seville Spain E-mail: lferrandiz@e-derma.orgSearch for more papers by this authorTomas Toledo-Pastrana MD, Tomas Toledo-Pastrana MD Dermatology Unit, Hospital Universitario Virgen Macarena, Seville, SpainSearch for more papers by this authorDavid Moreno-Ramirez PhD, David Moreno-Ramirez PhD Dermatology Unit, Hospital Universitario Virgen Macarena, Seville, SpainSearch for more papers by this authorLeonor Bardallo-Cruzado MD, Leonor Bardallo-Cruzado MD Neonatal Unit, Hospital Universitario Virgen Macarena, Seville, SpainSearch for more papers by this authorSonia Perez-Bertolez MD, Sonia Perez-Bertolez MD Pediatric Surgery Unit, Hospital Universitario Virgen Macarena, Seville, SpainSearch for more papers by this authorSalud Luna-Lagares MD, Salud Luna-Lagares MD Neonatal Unit, Hospital Universitario Virgen Macarena, Seville, SpainSearch for more papers by this authorJuan J. Rios-Martin PhD, Juan J. Rios-Martin PhD Pathology Unit, Hospital Universitario Virgen Macarena, Seville, SpainSearch for more papers by this author Lara Ferrandiz PhD, Corresponding Author Lara Ferrandiz PhD Dermatology Unit, Hospital Universitario Virgen Macarena, Seville, Spain Dr. Lara Ferrandiz, phD Dermatology Unit Hospital Universitario Virgen Macarena Avda, Dr. Fedriani s/n 41073 Seville Spain E-mail: lferrandiz@e-derma.orgSearch for more papers by this authorTomas Toledo-Pastrana MD, Tomas Toledo-Pastrana MD Dermatology Unit, Hospital Universitario Virgen Macarena, Seville, SpainSearch for more papers by this authorDavid Moreno-Ramirez PhD, David Moreno-Ramirez PhD Dermatology Unit, Hospital Universitario Virgen Macarena, Seville, SpainSearch for more papers by this authorLeonor Bardallo-Cruzado MD, Leonor Bardallo-Cruzado MD Neonatal Unit, Hospital Universitario Virgen Macarena, Seville, SpainSearch for more papers by this authorSonia Perez-Bertolez MD, Sonia Perez-Bertolez MD Pediatric Surgery Unit, Hospital Universitario Virgen Macarena, Seville, SpainSearch for more papers by this author … See all authors First published: 08 July 2013 https://doi.org/10.1111/ijd.12155Citations: 6 Conflict of interests: The authors declare they have no conflict of interests. Read the full textAboutRelatedInformationPDFPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessClose modalShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume53, Issue4April 2014Pages e247-e250 RelatedInformation RecommendedSuccessful propranolol treatment for diffuse neonatal hemangiomatosisTetsuhiko Matsuura, Takafumi Kadono, Hirotaka Koizumi, Tamihiro Kawakami, Yoshinao Soma, The Journal of DermatologyEfficacy of intravenous propranolol for life-threatening diffuse neonatal hemangiomatosisTakamichi Ishikawa MD, PhD, Keigo Seki MD, Hiroki Uchiyama MD, PhD, Pediatric DermatologySevere Hypoglycemia During Successful Treatment of Diffuse Hemangiomatosis with PropranololErnesto Bonifazi M.D., Angelo Acquafredda M.D., Antonella Milano M.D., Osvaldo Montagna M.D., Nicola Laforgia M.D., Pediatric DermatologyThe Successful Treatment of Diffuse Neonatal Hemangiomatosis with Flashlamp Pulsed Dye LaserHideki Endo M.D., Akira Kawada M.D., Ph.D., Yoshinori Aragane M.D., Ph.D., Yuji Yamashita M.D., Ph.D., Rieko Isogai M.D., Tatsuo Yudate M.D., PhD, Tadashi Tezuka M.D., Ph.D., Pediatric DermatologyCongestive heart failure with diffuse neonatal hemangiomatosis – case report and literature reviewMoshe Dotan, Avraham Lorber, Acta Paediatrica

Congestive heart failure with diffuse neonatal hemangiomatosis – case report and literature review

Vascular hemangiomas are considered to be a common finding among neonates and infants. The presence of five or more cutaneous hemangiomas may carry a higher incidence of multiple organ involvement An eleven week old female infant with high cardiac output heart failure and multiple cutaneous hemangiomas was referred to our clinic. Soon after birth she failed to gain weight and developed breathlessness. There was partial response to Captopril and Frusemide therapy, with development of a progressive cough. Echocardiogram and liver and brain ultrasound scans revealed heart chamber enlargement, multiple liver hemangiomas and an isolated cerebellar arterio-venous malformation (AVM). Supportive treatment for congestive heart failure combined with propranolol therapy resulted in rapid clinical response and recovery. The diagnosis of Diffuse Neonatal Hemangiomatosis in infants with failure to thrive and symptoms of heart failure raises the possibility of internal organ involvement. Augmentation of propranolol in such cases may cause regression of Hemangiomatosis with patient clinical improvement. We report our experience with a single patient of Hemangiomatosis induced heart failure which responded to propranolol combined with medication to control heart failure and failure to thrive prevention. This treatment should be further evaluated with special attention to potential adverse effects, tolerance and compliance.

Cerebellar hemangioblastoma associated with diffuse neonatal hemangiomatosis in an infant

Hemangioblastomas (HBLs) comprise approximately 2% of all primary central nervous system (CNS) tumors. Although histological features of this rare tumor are generally benign, its outcome is often unfavorable due to high risk of recurrence and multifocal localization. HBLs can be detected as sporadic or associated with Von Hippel-Lindau disease. Diffuse neonatal hemangiomatosis (DNH) presents with multiple, progressive, rapidly growing cutaneous hemangiomas associated with widespread visceral hemangiomas in the liver, lungs, gastrointestinal tract, brain, and meninges. DNH with predominant CNS involvement is rarely reported. Herein, we present a neonatal case of cerebellar HBL associated with DNH. A 5-day-old male baby was referred with complaints of multiple cutaneous lesions. Purple papules were noted on the trunk, extremities, and the head. Thoracic magnetic resonance imaging demonstrated multiple hyperintense lesions on the chest wall and apex of the right lung. On MRI, a 3×2-cm mass lesion in the right cerebellar hemisphere was detected. Total resection of the mass and ventriculoperitoneal shunting was performed. Histopathologic examination confirmed the diagnosis of HBL. Steroid therapy was administered for disseminated hemangiomatosis, and the lesions showed regression; the patient showed good clinical recovery. The parents refused further treatment, and he was out of our control when he was 9 months old. According to our knowledge, the presented newborn is the second case of cerebellar HBL associated with diffuse skin and visceral hemangiomas in the English medical literature. Clinicians must be vigilant about the predictive value of visceral and/or cutaneous hemangioma for an associated intracranial HBL.

Diffuse neonatal haemangiomatosis with a placental chorangioma

<h3>Introduction</h3> Single haemangiomas are common in neonates. Rarely they present as multiple, widely spread lesions also known as haemangiomatosis. In 50% of these there is visceral involvement, which can affect the liver, GI system and CNS. There are reports of haemangiomatosis being associated placental chorangioma. <h3>Case report</h3> A term male infant, BW 2280 g, was admitted to our neonatal unit with hypoglycaemia and a non-blanching rash. At delivery, a mass was found in the placenta, which was found to be a chorangioma. On admission, he was stable but had polycythaemia, thrombocytopenia and coagulopathy with a prolonged INR, APTR and decreased fibrinogen. He was managed with intravenous Vitamin K, a platelet transfusion and fresh frozen plasma. He remained clinically stable and he received one further dose of IV vitamin K before his thrombocytopenia and coagulopathy completely resolved. An abdominal ultrasound scan was performed to look for visceral involvement. This showed a hepatic haemangioma. <h3>Discussion</h3> Two types of neonatal haemangiomatosis are described. Benign neonatal haemangiomatosis affects the skin only and diffuse neonatal haemangiomatosis affects both the skin and viscera. Our case falls into the second group with both skin and liver involved. The most common sites affected by diffuse haemangiomatosis are the liver, GI system, CNS and lungs. Infants can be ill at birth or present later with high output cardiac failure, GI haemorrhage or CNS compression causing hydrocephalus. Mortality rates of up to 50% is reported.2. Large chorangiomas have an incidence of 1:9000 births and are often associated with antenatal complications and 30% fetal mortality. There is a known link between large chorangiomas and neonatal haemangiomatosis. The mechanisms of this link are still being established. In our case the neonate presented with thrombocytopenia and coagulopathy, which quickly resolved with treatment. This suggests that this was secondary to the placental chorangioma rather than the hepatic haemangioma, which would have caused a more prolonged consumptive coagulopathy.

Diffuse neonatal hemangiomatosis: An evidence-based review of case reports in the literature

The term "diffuse neonatal hemangiomatosis" has been used historically to describe multifocal vascular lesions affecting the skin and viscera in infants. We hypothesized that many cases reported as diffuse neonatal hemangiomatosis did not have infantile hemangiomas (IH), but represented more recently described neonatal vascular diseases. A literature search was performed using PubMed database (1950-2009) with the terms "neonatal hemangiomatosis," "benign hemangiomatosis," and "diffuse hemangiomatosis." A total of 180 articles were identified. Exclusion criteria included disease onset later than 3 years of age and absence of multifocal skin involvement. In all, 73 cases were selected and categorized into 3 groups: IH/probable IH; multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT)/probable MLT; and multifocal vascular lesions, not otherwise specified. Of the 73 cases, 43 had IH/probable IH, 17 had MLT/probable MLT, and 13 had multifocal vascular lesions, not otherwise specified. The clinical outcomes of these groups differed in that two of 43 (5%) patients with IH died whereas 11 of 17 (65%) patients with MLT died (odds ratio 37.6, confidence interval 5.6-387.6, P value < .0001). This was a literature-based meta-analysis, which inherently has limitations of incomplete and inconsistently presented information. Many cases reported in the literature as diffuse neonatal hemangiomatosis represent newly described multifocal vascular anomalies such as MLT, which has a strikingly higher mortality than IH. We propose the term "multifocal infantile hemangioma-with or without extracutaneous disease" instead of "diffuse neonatal hemangiomatosis" for multiple cutaneous IH. Accurate diagnosis of multifocal neonatal vascular lesions is imperative to facilitate appropriate evaluation, treatment, and prognosis.

Efficacy of Propranolol in Hepatic Infantile Hemangiomas with Diffuse Neonatal Hemangiomatosis

Efficacy of Propranolol in Hepatic Infantile Hemangiomas with Diffuse Neonatal Hemangiomatosis

Fetal Anemia of Unknown Cause – A Diagnostic Challenge

To assess the spectrum of underlying diseases in cases of fetal anemia in which the cause was unknown at the time of first and second transfusion or thereafter. All patients who underwent intrauterine transfusion were identified in the perinatal databases of two tertiary referral centers for prenatal medicine and treatment between 2002 and June 2010. 82 fetuses received intrauterine transfusion in the study period. A total of 356 transfusions were performed in these patients. The causes of fetal anemia in our cohort were alloimmunization (32), parvovirus infection (23), feto-fetal transfusion syndrome (9), sacrococcygeal teratoma (2) and cytomegalovirus infection (1). In the remaining 15 cases, the cause of fetal anemia was unknown at the time of first and second transfusion, and could only be ascertained in the further course of pregnancy, in the postnatal period or was ultimately left in doubt. In all cases markedly elevated peak systolic velocities in the middle cerebral artery accurately predicted fetal anemia. The final diagnosis in these cases was fetomaternal hemorrhage (4), Blackfan-Diamond anemia (1), diffuse neonatal hemangiomatosis with chorangioma (1), kaposi-like hemangioendothelioma (1), elliptocytosis (1), neonatal hemochromatosis (1), mucopolysaccharidosis type VII (1) and in 5 cases the cause of fetal anemia remained unexplained. The latter 5 cases had an uneventful postnatal course and did not require further transfusions in infancy. In cases of fetal anemia with negative indirect Coombs test and TORCH serology, rare causes of anemia have to be considered. Fetal studies should therefore include reticulocyte count, parameters of hemolysis, peripheral blood smear and fetal liver function tests. Maternal studies should involve a search for fetal red cells using flow cytometry rather than Kleihauer-Betke test.

Efficacy of Propranolol in Hepatic Infantile Hemangiomas with Diffuse Neonatal Hemangiomatosis

We report the rapid and dramatic efficacy of propranolol in 8 infants with infantile hepatic hemangiomas. The degree of response varied from a significant improvement to a complete resolution of hepatic lesions. Heart failure and hypothyroidism resolved, and hepatomegaly decreased. No side-effects of the drug were noted.

Vincristine, an efficacious alternative for diffuse neonatal haemangiomatosis

Diffuse neonatal haemangiomatosis (DNH) is an uncommon condition characterized by multiple cutaneous and visceral haemangiomas frequently causing severe complications. Corticosteroids constitute the first therapeutic line; however, when they fail, other alternatives are available, provided possible side effects are closely monitored during and after treatment. We present a case of life-threatening DNH, non-responsive to corticosteroids, successfully treated with Vincristine with minor side effects. We conclude that Vincristine is a valid alternative in corticosteroid-resistant DNH.

Interstitial laser therapy in hemangiomatosis of the liver – A case report

Abstract Objective Diffuse neonatal hemangiomatosis (DNH) is a severe disease with multiple organ involvement. If left untreated, it results in high mortality rates. Death occurs due to intractable heart failure following arteriovenous shunting or due to massive cerebral or gastrointestinal bleeding. If there are large liver hemangiomas and medical treatment is not sufficient, it is necessary to use interventional or surgical procedures such as embolization or ligation of the hepatic artery. We present another local tumor destruction procedure to treat liver hemangiomas in DNH, the interstitial laser therapy (ILT). Method ILT was performed in a 3-month-old male infant suffering from DNH with intractable heart failure and multiple liver hemangiomas. Due to the severity of the infant's condition and the minimally invasive character of the treatment, the ILT was performed directly in the neonatal intensive care unit. The ILT was carried out percutaneously by guiding a bare fiber (0.6 mm core diameter) via a puncture needle into the pathologically altered liver region. A Nd:YAG laser (1064 nm) was used as a light source for ILT because its light penetrates deeply into biological tissue. Tissue destruction was achieved with moderate laser power of 4–6 W and a long application time of 90–300 s. Using these applied physical laser parameters, almost spherical volumes of coagulation necrosis could be obtained with diameters of up to 1–2 cm. Puncture and the laser application were monitored by color-coded duplex sonography (CCDS). A decrease in color signal intensity at the tip of the bare fiber indicated the increasing tissue coagulation during application time. Results After 2 sessions of ILT of liver manifestation in 10 days the cardiac output was reduced by 50%. Postoperative Doppler imaging showed fibrosis in the treated areas of the liver. The child was transferred back to his country of origin but he died 3 months later of intracranial bleeding. Conclusion In our opinion interstitial laser therapy can be an effective minimally invasive method to treat life-threatening liver hemangiomas in diffuse neonatal hemangiomatosis.