Autosomal recessive polycystic kidney disease (ARPKD) is characterized by enlarged kidneys with dilated collecting ducts and congenital hepatic fibrosis. There is a variable rate of progression of kidney and liver disease. Portal hypertension and Caroli's disease occur from liver involvement that contributes to morbidity and mortality. Approximately 40 % of patients have a severe disease phenotype leading to rapid onset of end-stage kidney disease (ESKD) and signs of portal hypertension and the rest may have predominant involvement of either the kidney or liver. It is important for the physician to establish the extent of organ involvement before deciding on the ultimate plan of management, especially when transplantation is required. Isolated renal transplantation can be considered when liver involvement is minimal. If hepatobiliary disease is prominent, and kidney function is preserved, management options are based on individual characteristics. In the presence of significant liver disease and ESKD, consideration should be given to combined liver kidney transplantation, which can be beneficial in eliminating the consequences of both kidney and liver disease. However, this is a complex surgical procedure that needs to be performed at experienced transplant centers. Improvement in surgical techniques has considerably improved short-term graft survival with the added advantage of the liver offering immunologic protection to the kidney allograft.
Retinoblastoma is one of the most common ophthalmic neoplasms affecting children worldwide. Since its recent introduction, superselective ophthalmic artery injection of chemotherapy with melphalan has significantly reduced the need for enucleation in patients with advanced disease and also shown to have minimal adverse effects on visual acuity as compared to the conventional therapy. Although no severe complications resulting in strokes or deaths have been reported, this treatment modality is not without difficulties. In this case discussion, we describe an event that has occurred to several pediatric patients undergoing superselective angiography of the ophthalmic artery that may be due to an oculopulmonary type reflex causing significant hemodynamic instability and hypoxemia.
The third eyelid protects the eye, contribute to the aqueous portion of the pre‐ocular film and its distribution over the corneal surface, as well as removal of foreign material from the anterior surface of the eye. It is rather vulnerable during fights and clinicians often have to perform surgery for third eyelid neoplasia. In dogs, this lid has a triangular shape, being widest at its free margin. It is vascularized by branches of the malar artery. Being of such importance as an adnexa of the eye, the authors concentrated their attention on its vascularization, which is not well defined. Methods: Scanning Electronic Microscopy of vascular corrosion casts and intact tissue. Observations: It was quite interesting to notice the presence of two patterns of vascularization inside the third eyelid. One can say that there are two segmental levels of vessels. In one of these there is a predominance of arteries and veins, while in the other a meshwork of third order arteries, capillaries and post‐capillary venules are present. In fact, the branch of the malar artery that reaches the base of the third eyelid divides into smaller branches that cross almost the complete length of the lid giving only a few collateral vessels, in a monopodical way, and only near the free margin of the lid is possible to observe dicothomical ramifications that became rather anastomotic (being anastomoses of type I and II are present) and contribute to the microvascular meshwork. This microvascular net is drained by post‐capillary venules and veins that empty as a brush into bigger veins that occupy the same segmental level as the third eyelid artery. Remarks: This uncommon angioarchitecture is probably an adaptation to the fact that in the medial canthus of the eye this lid has a smaller space and the way of obtaining an efficient vascularization is by going straight to the top of the lid.
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