Telangiectasia of Pembroke Welsh Corgi Dogs

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Vet. Pathol. 20: 203-208 (1983)

                Telangiectasia of Pembroke Welsh Corgi Dogs

                              F. M. MOORE and G. W. THORNTON

 The Department of Pathology and the Department of Internal Medicine, Angell Memorial
                            Animal Hospital, Boston, Mass

   Abstract. Multiple vascular lesions involving kidneys and various other organs consisting
of cavernous, blood-filled spaces lined by endothelial cells with various amounts of mural
collagen are described in eight Pembroke Welsh Corgi dogs. The most common clinical sign
associated with this condition is hematuria.

   Hemangiomas are relatively common in dogs [14]. The differentiation between
these benign neoplasms of endothelial cells, and the developmental anomalies of
vessels (so-called hamartomas) is difficult [10, 17]. Multiple hemangiomatous syn-
dromes have been reported in man [5, 6, 8, 17, 19], and several hemangioma-like
conditions have been recognized in domestic animals [4, 11, 14, 15, 18]. No animal
syndromes have been reported with multiple organ system involvement, however.
This paper describes the occurrence of multiple vascular lesions in a group of
Pembroke Welsh Corgi dogs.

                                                Case Report
   This disease entity has been diagnosed definitively by histological evaluation in eight red
Pembroke Welsh Corgi dogs. Six dogs were necropsied and surgical renal biopsies alone were
examined from the other two dogs. No geneological information was available, but none of
the Corgies were known to be related.
   Five males and three females were affected and all except one had intermittent gross
hematuria, often with several months between episodes. In addition to bloody urine, blood
clots were voided occasionally. Other clinical signs reported were splinting of the abdomen,
whining, vomiting, and occasional dysuria. In one dog, no clinical signs were reported. The
age at onset of the clinical signs varied from two to eight years; however in one dog, the age
at onset was unknown.
   Physical examination frequently revealed no abnormal findings. Occasionally, renal pain
could be elicited upon palpation, or a small, irregular kidney could be felt. The only consistent
laboratory finding was the presence of numerous red blood cells in the urine even when gross
hematuria was absent. Most dogs had bacterial urinary tract infection during some episodes
of hematuria.
   Radiographic evaluation of dogs in the acute phase of the disease commonly revealed no
abnormalities. Renal calculi and renal calcification sometimes were seen in dogs that had
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204                                       Moore and Thornton

                   Table I. Telangiectasia in Pembroke Welsh Corgi dogs
      Dog number            Age (years)                       Sex                      Sites of involvement
         1 5 M                                                                    Kidneys
         2  13 M                                                                  Kidneys, subcutis midbrain
         3   9 F/S                                                                Kidneys, spleen
         4  II F                                                                  Kidneys, subcutis
         5  13 M                                                                  Kidneys, subcutis, duodenal
                                                                                    serosa
          6                        13                         F/S                 Kidneys, subcutis, anterior
                                                                                    mediastinum, spleen, retro-
                                                                                    peritoneum
          7*                        6                         M
          8*                        9                         M
  * Only kidneys were examined microscopically.

hematuria for several years. Intravenous pyelograms revealedpyelorenal backflow and dis-
torted renal pelves in chronically affected dogs. The renal angiograms that were done on two
dogs were not diagnostic.
   Treatment was directed at prevention of bacterial urinary tract infection, renal calculi, and
anemia due to blood loss.
   The age range at death or euthanasia was from five to 13 years (table I). Three dogs died
or were euthanatized due to anemia; one was euthanatized because of hydronephrosis
secondary to obstructing ureteral blood clots; three died or were euthanatized for unrelated
conditions, and in one dog the circumstances of death were unknown. Two corgies having this
condition were not autopsied, and the extent of disease involvement other than the renal
lesions was not determined. Therefore, the following discussion will refer to the six dogs which
were necropsied.
   Tiss~es for histologic examination were obtained at surgery or necropsy, fixed in 10%
formalin, embedded in paraffin, sectioned at 6 urn, and stained with hematoxylin and eosin
(HE); and selected tissues were stained with Masson's trichrome, Verhoeff's elastic, and
Gomori's reticulin stains.

                                                  Results
   Bilateral renal involvement was evident in each dog, but the kidneys were unequal
in size in four. Despite these findings, the weight (as expressed in combined renal
weights/Hx) g body weight) was considered normal. Red-black nodules, ranging
from several millimeters to several centimeters in diameter, were visible on the
capsular surface (fig. I). Some nodules were soft and fluctuant and others were firm.
Occasionally, a single nodule would comprise one pole of the kidney. The intervening
capsular surface was roughened with multiple depressed foci, I to 3 mm in diameter.
On cross section, multiple, soft, red-black regions were visible; the larger of which
corresponded to the capsular nodules. In addition, soft red foci (0.2 to 0.7 em
diameter), many of which were cystic, were located within the medulla. These foci
clustered subjacent to the corticomedullary junction consistently (fig. 2). Clotted
blood was present in these cysts and frequently in the renal pelvis as well. Hydro-
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Telangiectasia in Corgi Dogs                        205

   Fig. I: Kidneys of dog 6 with raised black masses and irregular capsular surface.

  Fig. 2: Longitudinal section of kidney. dog 6, with numerous black foci; some appear cystic.
Clustering of foci in outer medullary region. Blood clot fills renal pelvis.

 nephrosis was evident in three dogs, and severe hydronephrosis and hydroureter
were associated with a blood clot in dog 1.
   Subcutaneous lesions were well circumscribed, soft to firm, red and 0.4 to 2.5 em
in diameter. Splenic lesions occurred in two dogs (table I), and gross abnormalities
were evident in one of these. Multiple, raised, red-purple, soft masses 0.4 to 0.8 ern
in diameter occurred in dog 6. In dog 5, a firm and red-black duodenal mass (0.3 X
0.2 x 0.1 em) was located on the serosal surface along the antimesenteric border. The
anterior mediastinal masses of dog 6 were well-delineated, soft, red, and 0.1 to 0.3
cm in diameter. A similar lesion, 0.8 em in diameter, was located ventral to the fifth
thoracic vertebra. In this dog, a 2.0-cm diameter, soft, red, nodular mass also occurred
in the retroperitoneal space ventral to the fourth lumbar vertebra. In dog 2, a 0.7 X
0.6 x O.5-cm black, irregular, granular mass was located at the left ventrolateral edge
of the thalamus, immediately rostral to the cerebellar cortex. The lesions were
characterized by multiple, cavernous, blood-filled spaces with simple endothelial

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206                                      Moore and Thornton

3                                                                                                   4

5                                                                                                   6
      Fig.3: Histological section of kidney, dog 6, with multiple, cavernous, blood-filled spaces
    subjacent to cortico-medullary junction. HE.
      Fig. 4: High power magnification of renal vascular lesion with simple endothelial lining.
    HE.
      Fig.5: Duodenum, dog 5. Cavernous, blood-filled space located with tunica muscularis.
    Masson's trichrome.
      Fig. 6: Midbrain lesion, dog 2. HE.

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Telangiectasia in Corgi Dogs                         207

linings and various amounts of mural collagen. Frequently, thrombosis and hemor-
rhage were evident [fig. 3, 4].
   In the kidney, the lesions were located primarily in the medulla adjacent to the
corticomedullary junction. Often, the width of the overlying cortex was decreased
due to a loss of tubules and glomeruli, with interstitial fibrosis and mononuclear
interstitial nephritis. In several kidneys, the overlying cortex was thinned so that the
larger lesions extended close to the capsular surface. No tubules were found inter-
posed between vascular spaces.
   The duodenal lesion of dog 5 was located within the inner portion of the muscularis
externa [fig. 5]. A leiomyoma occupied a similar position in an adjacent portion of
the duodenum.
   No neural tissue was found interposed between the blood-filled spaces within the
midbrain lesion [fig. 6].

                                               Discussion
    Renal hemangiomas have been reported in the dog [3, 12], and in both of these
 dogs a solitary, discrete lesion involving one kidney was associated with hematuria.
 Similarly, unilateral involvement is the most frequent finding in humans with renal
 hemangiomas [I, 2, 7, 9, 13, 16].
    In contrast, the vascular lesions found in these Corgies consistently involved both
 kidneys, and frequently occurred in multiple organs. This finding, in association with
 the consistently simple endothelial lining and lack of endothelial cell proliferation
suggests that these lesions represent malformations. The unique group of animals
affected may evidence a possible congenital or hereditary nature of this disorder.
The term telangiectasia was chosen to designate these lesions to avoid confusion with
terms suggestive of neoplasia.
   Multiple hemangiomatous syndromes have been reported in humans, including
Sturge-Weber's syndrome, von Hippel-Lindau disease, and Osler's disease. These
syndromes are characterized by the development of lesions in the skin, mucous
membranes, retina, leptomeninges, respiratory, urinary, and gastrointestinal tract. In
von Hippel-Lindau disease, the vascular lesions are associated with cysts of the
pancreas, liver, and kidneys. In Sturge-Weber's disease, a lesion within the lepto-
meninges is associated with ipsilateral facial nevi [5, 6, 8, 17, 19].
   In these corgies, the vascular abnormality was not found in association with other
malformations. No syndromes of vascular anomalies with multiple system involve-
ment have been reported previously in animals.
   Further investigation of these telangiectasias is necessary to define more accurately
the abnormality, and to explore the possible use of these dogs as models for the
development of vascular malformations.

                                      Acknowledgements
 This work was supported in part by National Institute of Health, National Research Service
Award 5T32RR07000-07.

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208                                           Moore and Thornton

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Request reprints from Frances M. Moore, Angell Memorial Animal Hospital, 350 S. Hunting-
ton Ave, Boston, MA 02130 (USA).

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