Breast Hemangioma: Unique Presentation In a Patient with Klippel-Trenaunay-Syndrome

Author(s):  
Ayesha Shamim Siddiqui ◽  
Ibtesam Zafar ◽  
Ayesha Isani Majeed ◽  
Ramish Riaz

Background: Klippel–Trénaunay-Syndrome (KTS) is characterized by triad of varicose veins, port wine stain and soft tissue or bony hypertrophy and the diagnosis of KTS can be made if any two of these three features are present. Hemangiomas in various location e.g. skull, brain, epidural and vertebral hemangioma, mediastinal, colonic hemangioma, intraneural/intramuscular hemangiomas are reported with KTS. Case Presentation: Benign vascular tumors may rarely develop malignant transformation as Bugarin-Estrada et al reported breast angiosarcoma in a patient diagnosed as Klippel-Trenaunay-Syndrome. We reported a case of a 40-year-old female with known case of Klipple-Trenaunay-Syndrome with left leg varicosities, cutaneous nevus as well as unfortunate development of deep venous thrombosis and markedly enlarged right breast hemangioma. Due to low incidence or lack of early detection of breast hemangioma, its diagnosis is challenging. Conclusion: The history of patient and multi-modality imaging utilization can help in early and accurate diagnosis of diseases leading to better prognosis.

Author(s):  
Deepak Sharma ◽  
Sachin Lamba ◽  
Aakash Pandita ◽  
Sweta Shastri

Klippel–Trénaunay syndrome (KTS or KT) is an infrequently seen dermatological syndrome, which is often viewed as a triad of vascular malformation (capillary malformations or port-wine brands), venous varicosity, and soft tissue and/or bony hypertrophy. We report a case of a 12-year-old male who presented to us with the symptoms of varicose plaques over both lower limbs and was diagnosed as a case of KTS. Management is normally conservative and includes stockings for compression of the branches to reduce edema because of chronic venous insufficiency; modern devices that cause on and off pneumatic compression; and rarely, surgical correction of varicose veins with lifelong follow-up. The orthopedic abnormalities are treated with epiphysiodesis in order to prevent (stop) overgrowing of limb and correction of bone deformity.


Ultrasound ◽  
2019 ◽  
Vol 28 (2) ◽  
pp. 91-102
Author(s):  
Olga Ivanitskaya ◽  
Elena Andreeva ◽  
Natalia Odegova

Klippel–Trenaunay syndrome is a rare disease with a classic triad of port wine stains, varicose veins, and bony and soft tissue hypertrophy of an extremity. The quality of life in these patients is significantly affected, making the prenatal diagnosis of Klippel–Trenaunay syndrome important. We present four prenatally diagnosed cases of this anomaly with a unique case of ectrodactyly of the hand in foetus with Klippel–Trenaunay syndrome. Such a combination has not been previously reported prenatally. A review of the literature for similar cases is also presented.


2005 ◽  
Vol 20 (2) ◽  
pp. 63-81 ◽  
Author(s):  
B McDonagh ◽  
S Sorenson ◽  
A Cohen ◽  
T Eaton ◽  
D E Huntley ◽  
...  

Objective: To study the clinical profile of Klippel–Trenaunay syndrome (KTS) and the management of venous malformations (VMs) with ultrasound-guided foam sclerotherapy using the compass technique. Methods: In this open prospective series, 11 consecutive patients with KTS seeking therapy at a phlebology group practice were included. Seven (64%) of them were post-surgical recurrences. Results: Pain was the commonest presenting complaint (100%), accompanied with VMs/ varicose veins in all except one (91%). Chronic venous insufficiency (CVI) was seen in seven (64%). Soft tissue and bone deformity was present in all (100%). Limb lengthening was frequent and detected in eight (73%), thickening in six (54%); unusually, one patient (9%) presented with severe thinning and shortening of the leg. Venous malformation was the primary vascular problem in nine patients (82%), with capillary malformation (CM)/port wine stain in seven (64%) and isolated lymphatic malformation (LM) in two (18%). The lateral embryonic/Klippel–Trenaunay (KT) vein was detected in 10 patients (91%). Deep vein deformity was not detected in any of them. All the deformities were of the lower limb (100%), while two (18%) had deformities of the thorax and one (9%) had gastrointestinal (GI) malformation. None of the patients gave family history of KTS, while five (45%) had history of varicosities in the family. Eight patients (73%) completed the therapeutic plan with a mean follow-up of 5±3.9 years. An excellent to good type result could be documented in six of the eight cases (75%). These patients had an event-free, good quality of life. Follow-up duplex studies revealed the evolving refluxing tracts requiring sequential therapies. Conclusions: The results in this series demonstrate the utility of foam sclerotherapy using the compass technique in the management of KTS. Chronicity of the VMs in KTS necessitates strict clinical monitoring and sequential therapies.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Franck Katembo Sikakulya ◽  
Walufu Ivan Egesa ◽  
Sonye Magugu Kiyaka ◽  
Philip Anyama

Abstract Background Klippel–Trénaunay syndrome is a rare congenital capillary–lymphatic–venous condition characterized by the clinical triad of capillary malformations (port wine stains), varicose veins with or without venous malformations, and bony and/or soft-tissue hypertrophy. It has a very low incidence of about 1:100,000. Case presentation We report the case of 21-day-old neonate Black African female (born in Uganda) with Klippel–Trénaunay syndrome who presented with macrodactyly and ectrodactyly on the left foot, as well as numerous port wine stains on the left thoracoabdominal region and anteroposterior left lower limb. Color Doppler ultrasound examination of the left lower limb and abdomen revealed varicose veins without signs of arteriovenous fistula. Conclusion The report presents the case of a neonate with a rare congenital vascular disorder type Klippel–Trénaunay syndrome.


2020 ◽  
Vol 24 (4) ◽  
pp. 434-438
Author(s):  
Muhammad Khalil Surani ◽  
Asia Noureen ◽  
Umair Khizar ◽  
Summera Tabasum

Background: Klippel-Trenaunay syndrome (KTS) is a rare congenital disease having an incidence of 1/20-40,000, live births. KTS is a congenital, vascular disorder affecting usually one or rarely more than one limbs. It is characterized by port wine stain (capillary malformation), limb hemihypertrophy and other vascular anomalies. Case presentation: We present a case of this rare disorder in an infant who presented at an early stage of this disorder. The index case presented to us with a port wine stain and soft tissue edema of right lower limb. The Doppler scan showed venous malformation of right leg. The diagnosis was made on the basis of clinical and radiological criteria. Conclusion: The case is being reported in order to increase awareness among medical professionals.


Author(s):  
Syarifah Dian Rosa Lubis ◽  
Melati Silvani

Background:  Gynecomastia is defined as the presence of excessive breast tissue in males, which can appear unilateral or bilateral. Bilateral prepubertal gynecomastia in the absence of endocrine abnormalities is extremely rare, with only a few cases in literature. Case Presentation: A  46-year-old man  presented with a  2 years history of bilateral breast enlargement and pain. There is heartburn, shrinking testicles,  and  decreased sexual drive. Vital  examination showed  normal. BMI  : 24. 0 kg/m².  Head  dan neck: normal. On the chest both breasts are enlarged as avocados, palpable time around areola, chewy contingency, press pain, galactorrhea  negative. Lung and cast are  normal. In  the abdominal: hepar, lien and renal are not palpable, and ascites are not found. There is an atrophy of testicles.  Previous history of hepatic cirrhosis with Esophageal Varicose Veins caused by Hepatitis B. Laboratory examination  Estradiol 98.71 pg/mL (range: <62), Testosterone: 8.66 ng/mL (range: 3.0-10.6), LH5.53 mlU/mL, FSH 6.49 mlU/mL, Prolactin 5.08 ng/mL; Total T4 5.57 ng/dL , TSH 1.84 μIU/mL. SGOT U/L 53, SGPT 71 U/L,  Hepatitis B (+), HBsAg Reactive S/CO 414.46; HBeAg: Non Reactive, HBV DNA Undetectable and Albumin 3.6 g/dL. Fibroscan:  Cirrhosis Hepatis. Abdominal ultrasound:  Cirrhosis Hepatis. Gastroscopy:  Esophageal Varicose Veins (F1, LI, CB) + Pan Gastropathy. Conclusion: Reportedly A 46-year-old man presented with  gynaecomastia bilateral with  Cirrhosis Hepatis Child Plug A, given conservative treatment with tamoxiphen for 1 month


Author(s):  
Fui Tin Pang ◽  
Sze Yuen Lee ◽  
Maninderpal Kaur

Abstract Background Angiosarcomas are rare and fast growing tumors that arise from vascular structures. This case report is significant because it is rare and may cause treatment delay if not considered as a differential diagnosis. Hence, we aim to inform efficient, appropriate, and evidence-based imaging practice to the benefit of patients regionally and worldwide. Case presentation Thirty-five-year-old lady, presented with a large left breast swelling. She had recent history of trauma to the breast which initially caused confusion regarding the possible diagnosis. Ultrasound and MRI raised high suspicion of an angiosarcoma. Patient went through embolectomy prior to mastectomy and axillary clearance. Histopathology confirmed the diagnosis of an angiosarcoma. She had no distant metastases at the time of diagnosis and the patient was treated with chemotherapy and radiotherapy. However, she returned with recurrence within 1-year duration. Conclusion Primary breast angiosarcomas are rare aggressive vascular tumors with generally poor prognosis and no pathognomonic signs on radiological imaging, especially mammogram. Ultrasound and MRI do help in characterizing these tumors and raising the suspicion of a vascular tumor. Ultrasound-guided biopsy is recommended to avoid unnecessary complications. In young patients with rapidly growing, highly vascular breast tumors, a differential diagnosis of primary angiosarcomas should be considered until proven otherwise.


2020 ◽  
Vol 5 (3 And 4) ◽  
pp. 155-160
Author(s):  
Mohsen Aghapoor ◽  
◽  
Babak Alijani Alijani ◽  
Mahsa Pakseresht-Mogharab ◽  
◽  
...  

Background and Importance: Spondylodiscitis is an inflammatory disease of the body of one or more vertebrae and intervertebral disc. The fungal etiology of this disease is rare, particularly in patients without immunodeficiency. Delay in diagnosis and treatment of this disease can lead to complications and even death. Case Presentation: A 63-year-old diabetic female patient, who had a history of spinal surgery and complaining radicular lumbar pain in both lower limbs with a probable diagnosis of spondylodiscitis, underwent partial L2 and complete L3 and L4 corpectomy and fusion. As a result of pathology from tissue biopsy specimen, Aspergillus fungi were observed. There was no evidence of immunodeficiency in the patient. The patient was treated with Itraconazole 100 mg twice a day for two months. Pain, neurological symptom, and laboratory tests improved. Conclusion: The debridement surgery coupled with antifungal drugs can lead to the best therapeutic results.


2020 ◽  
pp. 1-5
Author(s):  
Anton Stift ◽  
Kerstin Wimmer ◽  
Felix Harpain ◽  
Katharina Wöran ◽  
Thomas Mang ◽  
...  

Introduction: Congenital as well as acquired diseases may be responsible for the development of a megacolon. In adult patients, Clostridium difficile associated infection as well as late-onset of Morbus Hirschsprung disease are known to cause a megacolon. In addition, malignant as well as benign colorectal strictures may lead to intestinal dilatation. In case of an idiopathic megacolon, the underlying cause remains unclear. Case Presentation: We describe the case of a 44-year-old male patient suffering from a long history of chronic constipation. He presented himself with an obscurely dilated large intestine with bowel loops up to 17 centimeters in diameter. Radiological as well as endoscopic examination gave evidence of a spastic process in the sigmoid colon. The patient was treated with a subtotal colectomy and the intraoperative findings revealed a stenotic stricture in the sigmoid colon. Since the histological examination did not find a conclusive reason for the functional stenosis, an immunohistochemical staining was advised. This showed a decrease in interstitial cells of Cajal (ICC) in the stenotic part of the sigmoid colon. Discussion: This case report describes a patient with an idiopathic megacolon, where the underlying cause remained unclear until an immunohistochemical staining of the stenotic colon showed a substantial decrease of ICCs. Various pathologies leading to a megacolon are reviewed and discussed.


Author(s):  
Andwi Setiawan Kokok ◽  
Selly Christina Anggoro ◽  
Siti Chandra Widjanantie

Introduction: Complete resection of aspergilloma in chronic pulmonary aspergillosis (CPA), may has several health problem after surgery berupa sesak napas, batuk yang tidak efektif. Methods: A case presentation of 45 years old woman, after lobectomy on right upper of lung due to aspergilloma, with history of cough and haemoptysis for 3 months. Pulmonary Rehabilitation were breathing retraining exercise (BE), mobilization technique (MT), chest mobility exercise (CM), active cycle breathing technique (ACBT), postural correction exercise (PC) for three weeks. Results: There were dyspnea, peak flow rate (PFR:60-70-60), peak cough flow (PCF: 70-90-60 L/m), and abnormal chest expansion (CE: 2.5 – 3 – 2) cm. After three weeks of Pulmonary Rehabilitation, there were no dyspnea, increased the PCF: 193L/m, and CE: 2.5-4 -3. Conclusion: Pulmonary Rehabilitation programs for three weeks were relieved dyspnea, increased cough capacity and CE.


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