We define the histopathologic findings and review the clinical and radiologic characteristics of rapidly involuting congenital hemangioma (RICH). The features of RICH are compared to the equally uncommon noninvoluting congenital hemangioma (NICH) and common infantile hemangioma. RICH and NICH had many similarities, such as appearance, location, size, and sex distribution. The obvious differences in behavior served to differentiate RICH, NICH, and common infantile hemangioma. Magnetic resonance imaging (MRI) of the three tumors is quite similar, but some RICH also had areas of inhomogeneity and larger flow voids on MRI and arterial aneurysms on angiography. The histologic appearance of RICH differed from NICH and common infantile hemangioma, but some overlap was noted among the three lesions. RICH was composed of small-to-large lobules of capillaries with moderately plump endothelial cells and pericytes; the lobules were surrounded by abundant fibrous tissue. One-half of the specimens had a central involuting zone(s) characterized by lobular loss, fibrous tissue, and draining channels that were often large and abnormal. Ancillary features commonly found were hemosiderin, thrombosis, cyst formation, focal calcification, and extramedullary hematopoiesis. With one exception, endothelial cells in RICH (as in NICH) did not express glucose transporter-1 protein, as does common infantile hemangioma. One RICH exhibited 50% postnatal involution during the 1st year, stopped regressing, was resected at 18 months, and was histologically indistinguishable from NICH. In addition, several RICH, resected in early infancy, also had some histologic features suggestive of NICH. Furthermore, NICH removed early (2-4 years), showed some histologic findings of RICH or were indistinguishable from RICH. We conclude that RICH, NICH, and common infantile hemangioma have overlapping clinical and pathologic features. These observations support the hypothesis that these vascular tumors may be variations of a single entity ab initio. It is unknown whether the progenitor cell for these uncommon congenital vascular tumors is the same as for common infantile hemangioma.

Rapidly Involuting Congenital Hemangioma: Clinical and Histopathologic Features

Rapid postnatal growth and slow involution in childhood characterize the common infantile hemangioma. There are other rare vascular tumors that present fully grown at birth and behave quite differently, as designated by the acronyms: rapidly involuting congenital hemangioma (RICH) and noninvoluting congenital hemangioma (NICH). RICH and NICH have similarities in appearance, location, size, equal sex ratio, and both have overlapping radiologic and histologic features with infantile hemangioma. However, neither type of congenital tumor immunostains for glucose transporter-1 protein, a marker of infantile hemangioma. This raises the question of whether these congenital vascular lesions are variations in a spectrum of hemangioma or are entirely different tumors. We describe two groups of patients that suggest a linkage between postnatal and congenital vascular tumors: Link I (n=5), children who had either RICH or NICH coexisting with infantile hemangioma, and Link II (n=10), children initially diagnosed as having RICH, but regression was incomplete and the residuum was that of NICH. We conclude that these infants exhibit "missing links" between the rare RICH and NICH, and the common infantile hemangioma.

http://vascularanomalies.hsdm.harvard.edu/Publications/Mulliken8.pdf

Congenital hemangiomas and infantile hemangioma: missing links.

Antenatal hydronephrosis and the risk of pyelonephritis hospitalization during the first year of life.

Vesicoureteral Reflux and Urinary Tract Infection in Children With a History of Prenatal Hydronephrosis—Should Voiding Cystourethrography be Performed in Cases of Postnatally Persistent Grade II Hydronephrosis?

The strategy for the management of children with urinary tract anomalies has changed considerably as a result of the development of ultrasound equipment and techniques that allow for detailed fetal evaluation. Hydronephrosis is the most common urogenital anomaly detected, suggesting that an obstructive process may be potentially present. The goal of postnatal management is to identify and treat those patients whose renal function is at risk, while leaving alone the high percentage of patients who are at no risk of renal damage. This management involves a spectrum of radiological, medical, and surgical interventions for diagnosis, surveillance, and treatment. In this article, we review our current understanding of the natural history of antenatal hydronephrosis and its management.

http://www.nccpeds.com/kidney/articles/Antenatal%20Hydronephrosis%20Ped%20Nephro%20Feb%202013.pdf

Current management of antenatal hydronephrosis

Complications of voiding cystourethrography in the evaluation of infants with prenatally detected hydronephrosis

We describe a case of hepatic hemangioendothelioma that was first suspected based on prenatal sonographic findings at 19 weeks' menstrual age. At 16 weeks, the patient presented with a markedly elevated maternal serum alpha-fetoprotein level. Serial sonographic examinations revealed that the fetus had cardiomegaly, hepatomegaly with a hepatic mass and dilated intrahepatic vessels, a single umbilical artery, and a placental chorioangioma. Arteriovenous shunting within the hepatic mass was seen using color Doppler and pulsed Doppler sonography. An enlarged artery arising from the abdominal aorta supplying the mass was demonstrated. Postnatal physical examination and radiologic studies supported the diagnosis of hepatic hemangioendothelioma. The evolution in the sonographic appearance of this hepatic lesion in utero over a 17-week period is described.

Hepatic hemangioendothelioma: prenatal sonographic findings and evolution of the lesion.

Peliosis hepatis in a child with myotubular myopathy: Successful treatment using hepatic artery embolization

Mesenteric lipoma is a rare benign neoplastic condition that can grow to be very large and mimic other midgut fatty tumors. These benign tumors can cause various gastrointestinal symptoms such as obstruction and abdominal pain. We report the case of a 9-year-old boy who presented with a small bowel obstruction caused by torsion of a large mesenteric lipoma. This is an important but unusual tumor and should be considered in the differential of fatty lesions within the mesentery.

Torsion of a giant mesenteric lipoma.

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Complications of voiding cystourethrography in the evaluation of infants with prenatally detected hydronephrosis

Hepatic hemangioendothelioma: prenatal sonographic findings and evolution of the lesion.

Peliosis hepatis in a child with myotubular myopathy: Successful treatment using hepatic artery embolization

Torsion of a giant mesenteric lipoma.