ABSTRACT
INTRODUCTION: Kaposiform hemangioendothelioma (KHE) is a rare and aggressive vascular tumor that can be associated with a consumptive coagulopathy and thrombocytopenia (Kasabach-Merritt phenomenon). Only one case of an intracardiac KHE has been reported which was treated with surgical excision and then expectant management. CASE PRESENTATION: We present a patient with an intracardiac KHE which presented as a large mass surrounding the atria, pulmonary veins, superior vena cava, and infiltrating the atrial septum with moderate compression of the superior vena cava and mild compression of the pulmonary veins. This tumor clinically presented as persistent tachypnea and was unresponsive to conventional therapy with vincristine and steroids but responded dramatically to Sirolimus with almost complete regression on follow-up. CONCLUSIONS: None of the current treatments for KHE, alone or in combination therapy have been found to be effective in a uniform or reproducible manner. Well designed, preferably randomized trials are required for a better understanding of the appropriate dosage and duration as well as response to treatment and a consensus of first and second line therapies.
Subject(s)
Hemangioendothelioma/drug therapy , Kasabach-Merritt Syndrome/drug therapy , Sarcoma, Kaposi/drug therapy , Sirolimus/therapeutic use , Heart Diseases/drug therapy , Hemangioendothelioma/pathology , Humans , Infant , Kasabach-Merritt Syndrome/pathology , Pulmonary Veins/pathology , Salvage Therapy/methods , Sarcoma, Kaposi/pathology , Steroids/pharmacology , Tachypnea , Vincristine/pharmacologyABSTRACT
Pulmonary artery, and rarely aortic, calcifications have been reported in sporadic case reports in the recipient twin of twin-to-twin transfusion syndrome. This presentation is more likely to be secondary to the haemodynamic alterations in the recipient twin, but must be differentiated from idiopathic infantile arterial calcification as the clinical implications, treatment, and prognosis may be drastically different.
Subject(s)
Fetofetal Transfusion/complications , Fetofetal Transfusion/diagnosis , Pulmonary Artery/diagnostic imaging , Vascular Calcification/diagnostic imaging , Adult , Female , Fetofetal Transfusion/surgery , Humans , Infant, Newborn , Male , Pregnancy , Pregnancy, Twin , Tomography, X-Ray Computed , Ultrasonography, PrenatalABSTRACT
Hemoptysis in the children is a rare but serious diagnosis and is even more uncommon in infancy. Mortality is reported and depends on associated illnesses, underlying etiology, and amount of bleeding. A 4-month-old patient presented with significant hemoptysis. Flexible bronchoscopy with differential lavage confirmed the presence and site of origin of hemoptysis. She was managed with cardiac catheterization for embolization of an aorto-pulmonary collateral vessel with immediate complete resolution and no further recurrences. This highlights the importance of cardiac catheterization to detect collateral vessels as a cause for hemoptysis at this age and its successful resolution following embolization. Pediatr Pulmonol. 2016; 51:E31-E33. © 2016 Wiley Periodicals, Inc.
Subject(s)
Aorta/physiopathology , Collateral Circulation , Embolization, Therapeutic , Hemoptysis/etiology , Hemoptysis/therapy , Pulmonary Artery/physiopathology , Bronchoscopy , Cardiac Catheterization , Female , Hemoptysis/diagnosis , Humans , InfantABSTRACT
The objective of the study was to determine differences in optic nerve sheath diameter (ONSD) measurements taken from computed tomography (CT) scans of patients with ventriculoperitoneal shunt (VPS) obstruction versus controls. Inpatients 0-15 years with confirmed VPS obstruction requiring neurosurgical intervention were identified using ICD9 codes. ONSDs, orbit, cranium, and foramen magnum sizes were measured on their pre-surgical CT. Controls included cases at times when their VPS was not obstructed and age and gender matched patients with a CT scan done in the emergency room for head trauma (normal CT findings). Paired T-tests were used for both case-control comparisons. In order to compare the optic nerve sheath size more accurately, the ONSD width was divided by the width of the orbit and by the foramen magnum (antero-posterior) length. Twenty patients were identified with 25 events of VPS obstruction. The right ONSD (RON) was chosen to study. RON/orbit width and RON/foramen magnum diameter for the VPS obstruction versus self-controls, were 0.22 and 0.22, compared to 0.19 and 0.18, respectively, for the non-obstructed self-controls (P = .044 and P = .008, respectively). The same measurements for the VPS obstruction versus age and gender matched controls were 0.22 and 0.21 for the VPS obstruction cases, respectively, compared to 0.17 and 0.16, respectively for the age and gender matched controls (P < .001 and P < .001, respectively). This data confirms that the optic nerve diameter increases during a VPS obstruction. ONSD measurements by ultrasound could add to the evaluation for VPS obstruction.
