PENILE NECROSIS IN ASSOCIATION

WITH CHRONIC RENAL FAILURE

CALCIPHLAXIS OR CALCIFIC
UREMIC ARTERIOLOPATHY

A.V Mathur, S. Azad, M. Singh, N. Anand

Department of Surgery Shri Guru Ram Rai Institute of Medical and Health
Sciences,
Patel Nagar,Dehradun 248001 India

INTRODUCTIO
N
Penile necrosis along with necrosis of
the toes as a consequence of a
vasculopathy
Patients with diabetes with chronic renal
failure on long term hemodialysis
Calciphylaxis or Calcemic uremic
arteriolopathy

INTRODUCTION
First described
in uremic
patients by
Bryant and
White in 1898
Deserves
attention
because of the
poor prognosis

Calciphylaxis is
being studied
in Germany
since 2006

CASE PRESENTATION

45 year old male

patient

diabetes presented
with progressive
necrosis of the
terminal part of the
penile shaft and
tips of the toes of
the left foot of one
week duration

diabetic
nephropathy on
maintenance
hemodialysis and on
treatment for
hypertension.

CASE PRESENTATION
Physical
Examination

tachycardia

blood
pressure
was
150/100
mmHg

fasting
blood sugar
was 126
mg/dl

CASE PRESENTATION

Post Prandial
blood sugar
was 189 mg/dl

Anemic and
had puffiness of
the face and
lower limbs

chest had
crepitations at
the base

CASE PRESENTATION
Local Examination
necrosis of the full thickness of the terminal
part of the penile shaft extending to the
urethra and glans penis

gangrene of the tips of the toes of both

lower limbs

passing only about 100 ml of urine daily.

Catheterization revealed blood stained urine

Hemoglobin
of 7 gm/dl

Serum
creatinine
of 10 gm/dl

Serum
calcium
was 8
gm/dl

Serum
phosphorus
was 7
mg/dl

Parathormo
ne level
was normal

Vascular Doppler
revealed generalized
atherosclerotic disease
involving the lower limb
vessels distal to the
common iliac vessels

Plain skiagram
did not reveal
any soft tissue
calcification

After
hemodialysis, he
underwent
partial penile
amputation

Gangrene of
the toes was
managed with
debridement
and repeated
dressings

readmission
about three
weeks later
because of
gangrene in the
region of the
perineum

During
surgery we
found, the
corpora
cavernosa
contained
dark blood
with very
little bleeding

After amputation
there was patchy
necrosis of the
skin of the
ventral penile
flap
conservatively

recovery and
continued to
be on
maintenance
hemodialysis
.

Conclusion
Excluding our patient a total of
about 120 cases of penile
calciphylaxis

Most cases were associated

with end stage renal disease
and diabetes mellitus was the
co-morbidity in 76% patients.

Average patient age has been 58

years

Other conditions associated with calciphylaxis

are malignancy, inflammatory bowel disease,
Acquiredimmunodeficiency syndrome (AIDS),
POEMS syndrome, Alcoholic cirrhosis, Primary
hyperparathyroidism
with
normal
kidney
function, Chronic myelogenous leukemia (CML)
and Systemic lupus erythematosus (SLE)

Starts from a small

area of erythema or
thrombophlebitis
The lesion vary from
rapidly progress to
gangrene beyond the
solitary skin lesions
indurated plaques,
genitalia are found in
multiple eruptions
nodules or frank
two-third of the
covering several body
necrosis, with tissue
patients
regions
loss, eschar, infection,
gangrene and even
sepsis.
skeletal muscle myopathy, heart
valve calcification, ocular
calciphylaxis, necrotizing mastitis,
visceral involvement with
Intense pain
pulmonary calciphylaxis,
pancreatitis, peritoneal
calcification

Pathogenesis may
be related to
metastatic
calcification in
association with
high calcium
phosphate product.
Result from deficiency of inhibitors of
calcification like 2-HeremansSchmid
Vascular injury in
glycoprotein/ fetuin A (AHSG),
association with a hyper
Osteopontin (OPN) and matrix Gla
coagulable state and a
protein (MGP) (1). Differential diagnoses
possible role of Protein C
include pyoderma gangrenosum,
and Protein S deficiency
coagulopathies and arteriosclerotic
ulcers
Average calcium phosphate
product has been found to
be 78.5 mg2/dl2 (range 20.6
to 52.5) and mean
parathormone level has
been 553 pg/ml (range 10 to
65)

Parathyroidectomy has been

performed in 8 patients (with raised
parathyroid hormone (PTH) levels )
Survival has been better in patients
who underwent parathyroidectomy
(75%) than in those treated with
local debridement or penectomy
alone (28%)
overall mortality associated with this
disease has been about 64% with a
mean time to death of 2.5 months

DIAGNOSIS
When debridement is being performed is
aided by performing a skin biopsy and
histopathology demonstrate:
Small vessel calcification, thrombosis and
endovascular fibrosis, intimal hyperplasia,
ultimately leading to tissue necrosis.
Subcutaneous fat inflammation-panniculitis. Calcium
and phosphate deposits demonstrable in x-ray
microanalysis of the
lesions.

Prevention of the development of

calciphylaxis in patients on long
term dialysis therapy
Avoidance of anemia
Hypotension and
excessive peripheral
edema
Corticosteroids and
cytotoxic drugs

Prevention of the development of

calciphylaxis in patients on long
term dialysis therapy

Multidisciplinar
y intervention

Maintainance of a high index of suspicion and

early recognition and aggressive
multidisciplinary intervention

Dialysis

calcium and phosphate levels can be kept

normal
calcium phosphate product should be targeted
at 55 mg2/dl2.

Biophosphonat
es

Early use of biophosphonates

Parathyroidecto
my
Cinacalcet as a
calcimimetic
Thrombolysis

If PTH levels can be demonstrated to be high

Help control calcium metabolic anomalies in

association with calciphylaxis

with low dose tissue plasminogen activator

to treat the vascular occlusion.

Intravenous
sodium
thiosulphate

It could act by dissolving

precipitated calcium salts or by its
antioxidant properties

Antibiotics

To counter sepsis and treat the pain

Insulin and
heparin
injections

NOT be given on the abdominal wall

or thighs if they are the sites of
lesions

The outlook and prognosis of

patients with diabetes and
chronic renal failure has been
reported as poor with mortality
approaching 50% at 6 months

Hypertension and dyslipidemia

may accelerate atherosclerotic
angiopathy in such patients.

REFERENCES
Guldbakke KK, Khachemoune A. Calciphylaxis. Int J Dermatol
2007; 46: 231- 238.
Brandenburg VM, Cozzolino M, Ketteler M. Calciphylaxis: a
still unmet challenge. J Nephrol 2011; 24 : 142-148.
Nina Scola MD, Alexander Kreuter MD. Calciphylaxis: a severe
complication of renal disease, CMAJ 2011; 183.
Arseculeratne G, Evans AT, Morl SM. Calciphylaxis a topical
overview, JEADV; 2006; 20: 493502.
Hayden MR; Goldsmith DJ. Sodium thiosulfate: new hope for
the treatment of calciphylaxis. Semin Dial 2010; 23: 258-262.