Young adult with varicosities and recurrent lower gastrointestinal bleeding

2019 ◽  
Vol 6 (8) ◽  
pp. 3012
Author(s):  
Vidhyachandra V. Gandhi ◽  
Pratik Gautam ◽  
Nitin V. Pai ◽  
Sujai Hegde

A 30 years gentleman presented with anaemia since last ten years due to recurrent rectal bleeding. He has had multiple admissions with transfusions in the preceding 10 years without a clear clinical diagnosis. His physical examination revealed gross pallor, prominent veins on the lateral side of the left lower limb with minimal hypertrophy and a hemangiomatous patch over the left buttock. Colonoscopy revealed a few dilated tortuous submucosal vessels in the rectum and sigmoid colon. A MR angiogram showed absent left popliteal vein and angiomatous lesions in the rectum and sigmoid colon. He was diagnosed to have Klippel-Trenaunay syndrome with hemangiomata of the lower gastrointestinal tract causing bleeding. He underwent a low anterior resection with stapled anastomosis, which has effectively controlled his bleeding. Adults with gastrointestinal bleeding due to congenital venous malformations commonly have visible external stigmata, which are often pointers of rare syndromes.

2015 ◽  
Vol 4 (1) ◽  
pp. 73-77
Author(s):  
Mircea Beuran ◽  
Ionut Negoi ◽  
Sorin Paun ◽  
Valentina Negoita ◽  
Bogdan Stoica ◽  
...  

Author(s):  
Balaji Zacharia ◽  
Jittu Alex ◽  
Ashwin Rajmohan

AbstractWe present a case of a 14-year-old girl who was first treated when she was 4 years old. She had progressive limping of the left lower limb from the age of 3 years. She was diagnosed to have developmental coxa vara of the left hip and treated by a subtrochanteric valgus osteotomy of the left femur. Later, she developed hypertrophy of the left upper and lower limbs. There were port-wine stains over the left lower limbs with multiple superficial varicosities. Her diagnosis was Klippel–Trénaunay syndrome (KTS). She is asymptomatic at the final follow-up. Both developmental coxa vara and KTS are rare conditions. We present this case to demonstrate the rarest combination of two rare conditions occurring in the same limb.


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.


Author(s):  
Kanmani K. ◽  
Meena M. ◽  
Narmadha D. ◽  
Geethu V. A.

Klippel Trenaunay Syndrome is a rare congenital disease characterized by triad of extensive cutaneous haemangiomas (most common), venous varicosities and hypertrophy affecting a leg and/or arm on one side. We report two cases of pregnant women with Klippel Trenaunay syndrome of which one patient presented with Kasabach Merritt Syndrome (refractory coagulopathy). 24-year-old primigravida of 29 weeks with KTS presented with bleeding gums for two days. Known case of diabetes, hypothyroid on treatment and treated TB lymphadenopathy. She had hypertrophy with varicosities and haemangiomas on left lower limb and vulvar region. She was treated for thrombocytopenia at 19 weeks gestation with steroids. Laboratory studies revealed thrombocytopenia. Steroids were given as per haematologist opinion for thrombocytopenia. She had emergency caesarean section with platelet transfusion at 35 weeks as per vascular surgeon opinion. Postoperatively she was given anticoagulants and steroids. 28-year-old primigravida of 39 weeks with KTS admitted for safe confinement. She had surgery for haemorrhoids at 14 years of age and had bleeding per rectum two years back. She had hypertrophy of left lower limb and cutaneous nevus in multiple dermatomes on left half of body. Doppler left lower limb shows superficial varicosities. Laboratory studies revealed normal. With multidisciplinary approach, she had elective caesarean in view of primigravida/breech-footling presentation. Post operatively she was given anticoagulants. The success in the management of these patients requires the participation of multidisciplinary approach. The use of prophylactic anticoagulant is generally advised during the pregnancy and postpartum period.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Robin Osofsky ◽  
Cyril Kamya ◽  
Hamza Hanif ◽  
Victor Phuoc

Abstract Background Metastatic melanoma to the colon is rarely diagnosed with an incidence of only 0.3% and more than 95% of cases identified post-mortem. Survival for patients with metastatic melanoma to the colon is poor, with 5-year survival rates of 26.5%. Nonetheless, surgical resection of the colonic metastatic melanoma lesions is recommended as it is associated with improved survival. Additionally, surgical resection is also indicated for palliative reasons, as symptom resolution is achieved in 90% of such patients. Use of the surgical robot has increased dramatically in the past decades, especially in the field of colorectal surgery. Furthermore, recent studies have demonstrated comparable outcomes between patients undergoing either laparoscopic or robotic-assisted colorectal surgery for cancer. Here, we describe the first case, to the authors knowledge, of a robot-assisted sigmoid colectomy for metastatic melanoma. Case presentation A 72-year-old male with a history of metastatic melanoma diagnosed in 2015 with a favorable response to immunotherapy presented to the emergency department with symptomatic lower gastrointestinal bleeding (LGIB). Endoscopy demonstrated a friable melanotic lesion of the sigmoid colon with biopsy demonstrating histopathologic evidence of metastatic melanoma. After further evaluation, the patient consented for an elective robot-assisted segmental colectomy for palliative intent. Diagnostic laparoscopy identified no evidence of further intra-abdominal metastatic disease. After identifying the metastatic lesion in the sigmoid colon, the mesentery of involved segment of sigmoid colon adjacent to the lesion was divided using the bipolar electrosurgical vessel sealer device. The colon was divided both proximal and distal to the lesion using a robotic stapler and a tension-free colo-colonic anastomosis was created intracorporeally. Postoperatively, the patient had an unremarkable course and was discharged home on post-operative day 3. On follow-up, the patient was doing well with resolution of preoperative LGIB. Conclusion  This case highlights a rare presentation of metastatic melanoma to the colon in a patient presenting with LGIB. Furthermore, this case demonstrates the feasibility of the minimally invasive robotic-assisted approach for an uncommon pathology.


2021 ◽  
Vol 14 (3) ◽  
pp. e239420
Author(s):  
Oseen Hajilal Shaikh ◽  
Uday Shamrao Kumbhar ◽  
Ankit Jain ◽  
Sunitha Vellathussery Chakkalakkoombil

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterised by capillary and venous malformations and soft-tissue hypertrophy with or without lymphatic malformation. The involvement of the gastrointestinal tract and genitourinary tract is uncommon in KTS. We present a case of a young adult who presented to us with bleeding per rectum and varicosities of the left lower limb. The patient was evaluated and diagnosed to have KTS. The patient was managed conservatively. To best of our knowledge, this is the first case report in literature where KTS was associated with atypical varicose veins of the left lower limb with mild hypertrophy of the lower limb, localised hypertrophy of the right gluteal region with involvement of pelvis, rectum, bladder and seminal vesicle.


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