scholarly journals Neonatal Abdominal Hemangiomatosis: Propranolol beyond Infantile Hemangioma

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Siu Ying Angel Nip ◽  
Kam Lun Hon ◽  
Wing Kwan Alex Leung ◽  
Alexander K. C. Leung ◽  
Paul C. L. Choi
Keyword(s):  
Cardiac Failure ◽  
Rare Case ◽  
Male Infant ◽  
Infantile Hemangioma ◽  
Vascular Tumor ◽  
Life Threatening ◽  
Cutaneous Hemangioma ◽  
Benign Course ◽  
Hepatic Hemangiomas ◽  
High Output

Hemangioma is the most common vascular tumor of infancy; presentation is often as cutaneous infantile hemangioma (IH). Cutaneous hemangioma is a clinical diagnosis. Most IHs follow a benign course, with complete involution without treatment in the majority of cases. Visceral hemangioma often involves the liver and manifests as a life-threatening disorder. Hepatic hemangiomas may be associated with high output cardiac failure, coagulopathy, and hepatomegaly which generally develop between 1 and 16 weeks of age. Mortality has been reportedly high without treatment. We report a rare case of a male infant with neonatal hemangiomatosis with diffuse peritoneal involvement, which mimicked a malignant-looking tumor on imaging, and discuss therapeutic options and efficacy. Propranolol is efficacious for IH but generally not useful for other forms of vascular hemangiomas, tumors, and malformations. In our case of neonatal peritoneal hemangiomatosis, propranolol appears to have halted the growth and possibly expedite the involution of the hemangiomatosis without other treatments.

MULTINODULAR HEMANGIOMATOSIS OF THE LIVER IN INFANCY

PEDIATRICS ◽  
1972 ◽  
Vol 49 (4) ◽  
pp. 563-573
Author(s):  
Robert H. McLean ◽  
James H. Moller ◽  
Warren J. Warwick ◽  
Leon Satran ◽  
Russell V. Lucas
Keyword(s):  
Cardiac Failure ◽  
Intensive Treatment ◽  
Combined Effect ◽  
Arteriovenous Shunt ◽  
Congestive Cardiac Failure ◽  
Treatment Modalities ◽  
Arteriovenous Fistulae ◽  
Classical Triad ◽  
Hepatic Hemangiomas ◽  
High Output

Multinodular hemangiomatosis of the liver (MHL) is characterized pathologically by multiple hemangiomas, primarily in the liver but also in other organs. The hepatic hemangiomas act as small arteriovenous fistulae and their combined effect may result in a massive peripheral arteriovenous shunt and high output congestive cardiac failure. Patients with MHL may be recognized clinically by the classical triad of congestive cardiac failure, hepatomegaly, and cutaneous hemangiomas. All but 2 of 31 cases of MHL herein reviewed had the onset of symptoms prior to 6 months of age. High output cardiac failure were severe and resulted in a 70% mortality. Hepatomegaly was massive and out of proportion to the degree of cardiac failure. Therapy includes intensive treatment of congestive cardiac failure and judicious transfusion in anemic patients. Irradiation of the liver and corticosteroids have been utilized in attempts to speed evolution of the hepatic arteriovenous fistulae. The data are not sufficient to establish the efficacy of either of these treatment modalities.

scholarly journals Pulmonary Infantile Hemangioma: Clinical and Histopathological Review of Eight Cases

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4209-4209
Author(s):  
Alexindra Wheeler ◽  
Harry PW Kozakewich ◽  
Kumar Shashi ◽  
Whitney Eng
Keyword(s):  
Medical Therapy ◽  
Glucose Transporter ◽  
Conflicts Of Interest ◽  
Early Recognition ◽  
Infantile Hemangioma ◽  
Vascular Tumor ◽  
Histologic Diagnosis ◽  
Glucose Transporter 1 ◽  
Initial Symptoms ◽  
Hepatic Hemangiomas

Abstract Introduction Infantile hemangioma (IH) is the most common benign vascular tumor of childhood. It typically appears as a single cutaneous mass in the head, neck, and trunk area. IH that does not arise in the skin most commonly presents in the liver. The lesion emerges shortly after birth, rapidly enlarges within the first six months of life, and then spontaneously involutes by 5-10 years of age. Risk factors associated with IH complications include lesional size, location, and growth characteristics. Pulmonary IH is rare with limited reports of clinical presentations and outcomes. Methods An IRB-approved, retrospective review of pediatric patients with a diagnosis of pulmonary IH was conducted. Cases were identified within the Department of Pathology at Boston Children's Hospital from surgical or autoptic specimens evaluated between 1918 and 2021. Analysis of histopathological slides confirmed pulmonary IH in eight infants. We describe the diagnostic workup, radiological, and histopathological findings of these eight patients. Results All patients presented with symptoms of respiratory distress, including tachypnea, subcostal retractions, and hypoxia. The median age at initial symptoms was 1.5 months (range, birth to 12 months). Five patients had a single pulmonary hemangioma ranging in size from 0.2 to 8.0 cm; three patients had multiple pulmonary hemangiomas. Four patients had co-occurrence of multifocal hepatic IH. The median age at histologic diagnosis of pulmonary IH was 6.5 months (range, 5 weeks to 16 months). Glucose transporter-1 (GLUT-1) immunostaining was positive in seven cases. Chest radiography demonstrated nonhomogeneous, mass-like consolidative opacities or rounded nodules. Treatment was primarily supportive. Three patients received medical therapy; two were treated with interferon, and one received propranolol. The infant treated with propranolol responded well with decreased lesional size and resolution of respiratory symptoms. Half of the patients in the cohort died; causes of death included cardiac failure from hepatic involvement, sepsis, hemorrhage, and liver failure. Conclusions Although IH is a common childhood tumor, IH of the lung is rare. Most (80%) IHs are focal, with hepatic co-involvement in 50-60% cases (Zavras et al. Eur J Pediatr, 2020; Hinen et al. Front Pediatr, 2020). Given that all patients who had concomitant hepatic hemangiomas died-albeit before the widespread availability of medical therapy-the presence of hepatic hemangiomas may confer high risk of complications. Patients with hepatic hemangiomas presented with pulmonary symptoms. Biopsy may be necessary to confirm the diagnosis of pulmonary IH and inform treatment. In this series, only treatment with propranolol or surgical resection was curative. Pulmonary IH should be considered in the differential diagnosis of infants with unexplained pulmonary masses, especially when accompanied by hepatic IH. Early recognition is critical for patients to receive proper and potentially life-saving treatment. Disclosures No relevant conflicts of interest to declare.

scholarly journals Infantile Hemangioma: A Review of Current Pharmacotherapy Treatment and Practice Pearls

2020 ◽  
Vol 25 (7) ◽  
pp. 586-599
Author(s):  
Laura Tiemann ◽  
Sarrah Hein
Keyword(s):  
Health Care Providers ◽  
Pediatric Patients ◽  
Medication Management ◽  
Infantile Hemangioma ◽  
Vascular Tumor ◽  
Small Subset ◽  
Care Providers ◽  
First Line ◽  
Life Threatening ◽  
First Line Treatment

Infantile hemangioma (IH) is the most common vascular tumor of infancy, affecting as many as 5% to 10% of all infants. The exact cause is unclear, but specific risk factors, such as low birth weight, prematurity, female sex, white race, and family history are associated with IH development. Most IHs are benign and self-resolving, but a small subset of patients with IHs are at risk of severe or life-threatening outcomes. Systemic and topical β-blockers are effective and safe for use in pediatric patients and considered first-line treatment for both complicated and uncomplicated IHs. Recently published guidelines provide a thorough review of IH and management. This article focuses on IH pharmacotherapy and provides practice pearls to support health care providers in IH medication management.

scholarly journals Kasabach Merritt syndrome in a 28-year-old female: A strange case report

2021 ◽  
Vol 0 ◽  
pp. 1-3
Author(s):  
Swapan Kumar Sinha ◽  
Rishu Vidhatri ◽  
Debes Pal ◽  
Balaram Gupta
Keyword(s):  
Case Report ◽  
Blood Cells ◽  
Coagulation Factors ◽  
Vascular Tumor ◽  
Coagulation Cascade ◽  
Consumption Coagulopathy ◽  
Microangiopathic Hemolytic Anemia ◽  
Older Children ◽  
Life Threatening ◽  
Cutaneous Hemangioma

Kasabach Merritt syndrome (KMS) is a rare disease in which a benign vascular tumor that is hemangioma grows rapidly, entraps red blood cells, platelets, and coagulation factors leading to activation of coagulation cascade resulting in life-threatening disseminated intravascular coagulation and microangiopathic hemolytic anemia. KMS affects newborns and infants. Rarely can affect older children and adults with only a few cases reported in the existing literature. Clinically patients present with large cutaneous hemangioma usually involving the extremities however visceral organs may be involved in some cases along with anemia, thrombocytopenia, coagulopathy, and bleeding. We report a case of KMS in a 28-year-old female who presented with bilateral subdural hematoma, thrombocytopenia, and consumption coagulopathy. She was given seven days course of methylprednisolone to which she responded well.

scholarly journals A life-threatening spontaneous ascending aortic rupture due to a small penetrating aortic ulcer

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110377
Author(s):  
Yasuhito Nakamura ◽  
Kiyoshi Doi ◽  
Syojiro Yamaguchi ◽  
Etsuji Umeda ◽  
Osamu Sakai ◽  
...  
Keyword(s):  
Rare Case ◽  
Aortic Wall ◽  
Aortic Rupture ◽  
Ascending Aorta ◽  
Bleeding Site ◽  
Penetrating Aortic Ulcer ◽  
Postoperative Course ◽  
Life Threatening ◽  
Aortic Dissections

We reported a rare case of spontaneous frank rupture of a small (4 mm) penetrating aortic ulcer in the ascending aorta resulted in catastrophic bleeding. The ulcer only created a pinhole wound in the adventitia without saccular aneurysms, intramural hematomas, or aortic dissections. Notably, the wound could be directly closed because the aortic wall was intact only 5 mm away from the bleeding site. The postoperative course was uneventful, and the patient was discharged on the 11th postoperative day. After 8 months, follow-up computed tomography showed no abnormality of the aortic wall at the repair site.

scholarly journals Report of a Rare Case of Trauma-Induced Thyroid Storm

2002 ◽  
Vol 81 (8) ◽  
pp. 570-574 ◽  
Author(s):  
Neil M. Vora ◽  
Fred Fedok ◽  
Brendan C. Stack
Keyword(s):  
Nervous System ◽  
Rare Case ◽  
Hepatic Dysfunction ◽  
Motor Vehicle ◽  
Motor Vehicle Accident ◽  
Definitive Treatment ◽  
Thyroid Storm ◽  
Vehicle Accident ◽  
Hyperthyroid State ◽  
Life Threatening

Thyroid storm is a potentially life-threatening endocrinologic emergency characterized by an exacerbation of a hyperthyroid state. Several inciting factors can instigate the conversion of thyrotoxicosis to thyroid storm; trauma is one such trigger, but it is rare. Patients with thyroid storm can manifest fever, nervous system disorders, gastrointestinal or hepatic dysfunction (e.g., nausea, vomiting, diarrhea, and/or jaundice), and arrhythmia and other cardiovascular abnormalities. Treatment of thyroid storm is multimodal and is best managed by the endocrinologist and medical intensivist. Initial medical and supportive therapies are directed at stabilizing the patient, correcting the hyperthyroid state, managing the systemic decompensation, and treating the underlying cause. Once this has been achieved, definitive treatment in the form of radioactive ablation or surgery should be undertaken. We describe a case of thyroid storm in a young man that was precipitated by a motor vehicle accident.

High-Output Cardiac Failure and Coronary Steal With an Arteriovenous Fistula

2018 ◽  
Vol 71 (6) ◽  
pp. 896-903
Author(s):  
Amtul Aala ◽  
Sairah Sharif ◽  
Leslie Parikh ◽  
Paul C. Gordon ◽  
Susie L. Hu
Keyword(s):  
Arteriovenous Fistula ◽  
Cardiac Failure ◽  
Coronary Steal ◽  
High Output

Intra-Peritoneal Pseudocyst as a Rare Complication After Peritoneal Dialysis: The Use of a Staged Surgical Approach in Management

2021 ◽  
pp. 000313482110474
Author(s):  
Ahmad Kharsa ◽  
Kayla Colvill ◽  
Heather Stevenson ◽  
Jeffrey Fair ◽  
Rupak Kulkarni ◽  
...  
Keyword(s):  
Peritoneal Dialysis ◽  
Surgical Approach ◽  
Rare Case ◽  
Diagnostic Laparoscopy ◽  
Rare Complication ◽  
Cyst Excision ◽  
Neoplastic Processes ◽  
Life Threatening ◽  
Pseudocyst Formation

Despite its numerous benefits, peritoneal dialysis (PD) can rarely result in dangerous and even life-threatening complications, including peritonitis, hernias, encapsulating peritoneal sclerosis (EPS), and rarely peritoneal pseudocysts. Herein, we present a rare case of a giant intra-peritoneal pseudocyst that presented four months following the discontinuation of a 5-year course of complicated PD. Despite the initially successful drainages, the patient’s symptoms continued to recur, and the imaging findings were concerning for underlying neoplastic processes. As such, a staged surgical approach was performed, starting with a diagnostic laparoscopy and was subsequently followed with cyst excision and marsupialization to the peritoneal cavity. While previous reports of such rare pseudocyst have been documented in the literature as a complication of PD, to our knowledge, this is the second case of pseudocyst formation to occur months after the discontinuation of PD therapy. This case emphasizes the importance of close follow-up in PD patients and showcases how a staged surgical approach can be utilized to accurately diagnose and manage such complicated cases.

Sign in / Sign up

Export Citation Format

Share Document