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Diabetes & Obesity International Journal Research Article 6 min read

“Unreported Nuclear Medicine Findings in Congenital Emangiomas Involving Lower Limb (Klippel-Trenaunay Syndrome)”

Rashid R*, Zohaib B, Ali I, Ambrene Y, Syed Ali RN and Jawad G
* Corresponding author
ISSN: 2574-7770  10.23880/doij-16000108  Received: June 09, 2016  Published: July 01, 2016
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Keywords
Nuclear Medicine Congenital Emangiomas Lower Limb
Abstract

Hemangioma is a prevalent problem among the vascular abnormalities and is the very common benign tumor of infancy and childhood. It is characterized by rapid growth of endothelial cells that commonly arises in the head and neck region (60%), followed in frequency by the trunk (25%) and extremities (15%) [1]. Two types of congenital hemangiomas exist including rapidly involuting congenital hemangiomas and noninvoluting congenital hemangiomas.

Discussion

The patient presented with haemangioma of the lower limband was evaluated with three basic diagnostic tests to the check the extent and severity of the issue and appropriate designing of the therapy.In the management of hemangiomas, plain X-rays of the long bones (scanograms) are most helpful to see bone related abnormalities [3]. Lower limb X-Rays of the patient were taken that clearly showed hypertrophy of the right limb that appeared as a white mass as compared to the remaining image.

Magnetic resonance imaging (MRI) helps in accurate visualization of patient’s soft tissues. Hypertrophy of muscle and bone, presence of dilated veins and arteries, and pelvic and abdominal vascular abnormality can be assessed with MRI [12, 16]. It is alsohelpful in differentiating bone, fat, muscle hypertrophy and lymphedema of muscle, fat and bone [3]. MRI is useful in visualizing the extent of lesions along with infiltration of deep body tissues before treatment [13, 14, 17]. In this way, MRI helps in designing therapy for the patient, monitoring patient’s response to therapy, and to determine the disease prognosis [18]. MRI of the patient was done that showed that the patient hashemangiomas in the right leg, appearing white as compared to remaining part of the image.

Scintigraphy may be utilized to assess regional blood flow, patency of valves and bone vascularity in KTS [12]. When vein stripping, sclerosis, or ligation, or sclerosis are essential radionuclide venography or contrast venography is done to depict perforators, collaterals, and deep connections of the extremities [19, 20]. Various techniques were developed for better visualization of the deep veins, including retrograde venography, ascending venography, with pericalcaneal, femoral, and peritrochanteric injection of contrast material [21]. Scintivenography of the patient was done that showed increased blood flow and bone vascularity in the right limb of patient along with patent deep valves. KTS disease varies greatly from patient to patient, so every patient needs different treatment on the basis of his or her clinical presentation. For better management of the patient and to achieve the aim of improving patient’s quality of life, a multidisciplinary disease management approach is essential to be followed [12, 22, 23]. Patients of KTS should be treated conservatively, with special emphasis on symptomatic relief. Patients with functional limbs and having little or no edema, ulceration, bleeding, or pain are usually managed conservatively [12, 24]. Elastic compression stockings, elevation of the affected limb or intermittent pneumatic compression pumps are extensively used in managing patients with varicose veins, edema and swelling of the extremities [4, 12]. Compression stockings are highly recommended for all KTS patients, especially when they start walking [12, 23]. Patients with cellulitis or thrombophlebitis should be managed with antibiotics, analgesics, and elevation [24]. Corticosteroids should be used when appropriate. Antibiotics are recommended only in the case of infections [12, 25]. Proper skin care and regular cleansing should be done in patients with minor KTS to prevent the development of sever disabling conditions [12, 23]. Our patient was being considered for surgical procedure for varicose veins in a peripheral setup but with proper investigations it became obvious that KTS is genetic disorder that involves no improvement in patient’s quality of life after surgery. As the disease has no permanent cure so no surgery was advised. Over the counter analgesic and anti-inflammatory products were recommended to treat pain and inflammation. The use of elastic compression stockings and elevation of the right leg were emphasized for treating the varicose veins. Some

life style modifications like the use of heel inserts was promoted that help the patient during walking or standing. It was repeatedly emphasized that the patient should routinely clean the affected area to prevent the growth of microbes. Patient was advised for routine follow ups as it can help in accurate and timely management of patient in the case of any infection or other complexities.

Figure 1: c, d). Pain and swelling increase with standing and walking. Cutaneous pigmentation was obvious throughout the limb. The patient visited surgeon and he advised him to undergo the Doppler ultrasound of right leg. On the basis of the results, vascular abnormality was noted and varicose veins were ruled out. Spider like hemangioma was found on clinical examination.All the baseline investigations of the patient including Complete Blood Count (CBC), Differential Leukocyte Count (DLC), Liver Function Tests (LFTs) and Renal Function Tests (RFTs) were normal. X ray of pelvis and both femora showed that hip bone and right femur of involved side are larger than the contralateral side (Figure1: A). This discrepancy is also evident on angiography which showed hypertrophied and slightly large right femur when compared to left femur (Figure 1 B).
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Figure 1: c, d). Pain and swelling increase with standing and walking. Cutaneous pigmentation was obvious throughout the limb. The patient visited surgeon and he advised him to undergo the Doppler ultrasound of right leg. On the basis of the results, vascular abnormality was noted and varicose veins were ruled out. Spider like hemangioma was found on clinical examination.All the baseline investigations of the patient including Complete Blood Count (CBC), Differential Leukocyte Count (DLC), Liver Function Tests (LFTs) and Renal Function Tests (RFTs) were normal. X ray of pelvis and both femora showed that hip bone and right femur of involved side are larger than the contralateral side (Figure1: A). This discrepancy is also evident on angiography which showed hypertrophied and slightly large right femur when compared to left femur (Figure 1 B).
Figure 2: (A-F) showing MRI scans for comparison of the two legs with hemangiomas, hypertrophied muscles and femur in right thigh.
Click to enlarge
Figure 2: (A-F) showing MRI scans for comparison of the two legs with hemangiomas, hypertrophied muscles and femur in right thigh.
Figure 3: (A and B) showing Scintivenography of superficial and deep veins of leg.
Click to enlarge
Figure 3: (A and B) showing Scintivenography of superficial and deep veins of leg.

Conclusion

The patient presented to hospital was having Klippel Trenaunay Syndrome with varicose veins. Because of the genetic origin of KTS, no surgery was performed and the patient was recommended to use analgesics and follow other life style modifications for improvement of his quality of life. Therefore in the management of suspected KTS it may be suggested that clinical examination should follow with x-rays and if still found ambiguous either MRI or Scintigram may be of help to rule out KTS.

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@article{rashid2016,
  title   = {“Unreported Nuclear Medicine Findings in Congenital Emangiomas Involving Lower Limb (Klippel-Trenaunay Syndrome)”},
  author  = {Rashid R, Zohaib B, Ali I, Ambrene Y, Syed Ali RN and Jawad G},
  journal = {Diabetes & Obesity International Journal},
  year    = {2016},
  volume  = {1},
  number  = {2},
  doi     = {10.23880/doij-16000108}
}
Rashid R, Zohaib B, Ali I, Ambrene Y, Syed Ali RN and Jawad G (2016). “Unreported Nuclear Medicine Findings in Congenital Emangiomas Involving Lower Limb (Klippel-Trenaunay Syndrome)”. Diabetes & Obesity International Journal, 1(2). https://doi.org/10.23880/doij-16000108
TY  - JOUR
TI  - “Unreported Nuclear Medicine Findings in Congenital Emangiomas Involving Lower Limb (Klippel-Trenaunay Syndrome)”
AU  - Rashid R, Zohaib B, Ali I, Ambrene Y, Syed Ali RN and Jawad G
JO  - Diabetes & Obesity International Journal
PY  - 2016
VL  - 1
IS  - 2
DO  - 10.23880/doij-16000108
ER  -