Klippel–Trénaunay Syndrome and Developmental Coxa Vara in the Same Limb: A Case Report with a Review of the Literature

Left Femur ◽

Port Wine Stains ◽

Left Lower Limb ◽

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.

Overgrowth syndrome in neonates: a rare case series with a review of the literature

BMJ Case Reports ◽

10.1136/bcr-2018-225640 ◽

2019 ◽

Vol 12 (1) ◽

pp. e225640

Author(s):

Aakash Pandita ◽

Astha Panghal ◽

Girish Gupta ◽

Kirti M Naranje

Keyword(s):

Lower Limb ◽

Rare Case ◽

Case Series ◽

Port Wine Stain ◽

Port Wine ◽

Term Neonates ◽

Overgrowth Syndrome ◽

We present here two-term neonates presenting with right lower limb hypertrophy, a port-wine stain, acral abnormalities and clubfeet. These neonates had overlapping features of Klippel Trenaunay syndrome and congenital lipomatous overgrowth, vascular malformation, epidermal nevi and scoliosis/skeletal abnormalities. Such overgrowth syndrome has not been previously described in the literature. Both the neonates are doing well and are under regular follow-up.

A neonate with Klippel–Trénaunay syndrome: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-021-03029-4 ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Franck Katembo Sikakulya ◽

Walufu Ivan Egesa ◽

Sonye Magugu Kiyaka ◽

Philip Anyama

Keyword(s):

Lower Limb ◽

Varicose Veins ◽

Color Doppler ◽

Color Doppler Ultrasound ◽

Port Wine ◽

Black African ◽

Port Wine Stains ◽

Left Lower Limb ◽

Clinical Triad ◽

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.

Klippel–Trénaunay Syndrome – A Very Rare and Interesting Syndrome

Clinical Medicine Insights Circulatory Respiratory and Pulmonary Medicine ◽

10.4137/ccrpm.s21645 ◽

2015 ◽

Vol 9 ◽

pp. CCRPM.S21645 ◽

Cited By ~ 11

Author(s):

Deepak Sharma ◽

Sachin Lamba ◽

Aakash Pandita ◽

Sweta Shastri

Keyword(s):

Soft Tissue ◽

Vascular Malformation ◽

Chronic Venous Insufficiency ◽

Venous Insufficiency ◽

Varicose Veins ◽

Lower Limbs ◽

Port Wine ◽

Bone Deformity ◽

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.

Prenatal diagnosis of Klippel–Trenaunay syndrome: Series of four cases and review of the literature

Ultrasound ◽

10.1177/1742271x19880327 ◽

2019 ◽

Vol 28 (2) ◽

pp. 91-102

Author(s):

Olga Ivanitskaya ◽

Elena Andreeva ◽

Natalia Odegova

Keyword(s):

Quality Of Life ◽

Prenatal Diagnosis ◽

Soft Tissue ◽

Varicose Veins ◽

Port Wine ◽

Unique Case ◽

Port Wine Stains ◽

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.

Acute limb ischemia in a patient with pre-fibrotic myelofibrosis complicated by heparin-induced thrombocytopenia and thrombosis – case report and systematic review of dabigatran use

VASA ◽

10.1024/0301-1526/a000876 ◽

2020 ◽

pp. 1-6 ◽

Cited By ~ 1

Author(s):

Marina Di Pilla ◽

Stefano Barco ◽

Clara Sacco ◽

Giovanni Barosi ◽

Corrado Lodigiani

Keyword(s):

Systematic Review ◽

Case Report ◽

Lower Limb ◽

Limb Ischemia ◽

Acute Limb Ischemia ◽

Heparin Induced Thrombocytopenia ◽

Lower Limb Ischemia ◽

Vein Thrombosis ◽

Left Lower Limb

Summary: A 49-year-old man was diagnosed with pre-fibrotic myelofibrosis after acute left lower-limb ischemia requiring amputation and portal vein thrombosis. After surgery he developed heparin-induced thrombocytopenia (HIT) with venous thromboembolism, successfully treated with argatroban followed by dabigatran. Our systematic review of the literature supports the use of dabigatran for suspected HIT.

Internal valvuloplasty combined with sleeve wrapping in the treatment of severe deep venous incompetence: A case report

Vascular ◽

10.1177/1708538121990121 ◽

2021 ◽

pp. 170853812199012

Author(s):

Yingfeng Wu ◽

Libing Wei ◽

Xixiang Gao ◽

Yixia Qi ◽

Zhu Tong ◽

...

Keyword(s):

Lower Limb ◽

Venous Insufficiency ◽

Femoral Vein ◽

Duplex Ultrasound ◽

Vein Wall ◽

Polyester Urethane ◽

Left Lower Limb ◽

Deep Vein ◽

Color Duplex Ultrasound

Background The main cause of severe chronic venous insufficiency is deep venous incompetence. Deep venous reconstructive surgeries are reserved for cases that do not show a good response to conservative therapies. Method We present the case of a 68-year-old man presenting with swelling, pain, and pigmentation in his left lower limb for 14 years and ulcers for 10 years. Descending venography identified a Kistner’s grade IV reflux in the deep vein of the left lower limb. Internal valvuloplasty was performed following Kistner’s method. Meanwhile, external wrapping with a 1-cm-wide polyester-urethane vascular patch was performed to strengthen the vein wall in the venospasm condition. Results Symptoms were immediately relieved postoperatively. Refractory ulcers healed five months after the procedure. At the six-month follow-up, color duplex ultrasound of the deep vein of the left lower limb showed no reflux in the proximal segment of the femoral vein. Conclusion Internal valvuloplasty combined with sleeve wrapping is feasible in the treatment of severe deep venous incompetence with good short-term results.

Neurologic melioidosis presented as encephalomyelitis and subdural collection in two male labourers in India

The Journal of Infection in Developing Countries ◽

10.3855/jidc.6586 ◽

2015 ◽

Vol 9 (11) ◽

pp. 1289-1293 ◽

Cited By ~ 5

Author(s):

Kavitha Saravu ◽

Rajagopal Kadavigere ◽

Ananthakrishna Barkur Shastry ◽

Rohit Pai ◽

Chiranjay Mukhopadhyay

Keyword(s):

Lower Limb ◽

Corticosteroid Treatment ◽

Conus Medullaris ◽

Subdural Empyema ◽

Lower Limbs ◽

High Dose ◽

Limb Weakness ◽

Subdural Collection ◽

Lower Limb Weakness ◽

Left Lower Limb

Two distinct and potentially deceitful cases of neurologic melioidosis are reported. Case 1: A 39-year-old alcoholic and uncontrolled diabetic male presented with cough, fever, and left focal seizures with secondary generalization. An magnetic resonance imaging (MRI) brain scan revealed a small peripherally enhancing subdural collection along the interhemispheric fissure suggestive of minimal subdural empyema. Blood culture grew Burkholderia pseudomallei. Patient was diagnosed with disseminated bacteraemic melioidosis with subdural empyema. He was successfully treated with ceftazidime-cotrimoxazole-doxycycline. Case 2: A 45-year-old male presented with left lower limb weakness, difficulty in passing urine and stool, and back pain radiating to lower limbs. Neurological examination revealed flaccid left lower limb with absent deep tendon reflexes and plantar reflex. Spinal MRI showed T2 hyperintensity from D9 to L1 suggestive of demyelination. Patient was treated with high dose methylprednisolone. By day 3 of steroid treatment, lower limb weakness progressed. Subsequent MRI showed extensive cord hyperintensity on T2 weighted sequence extending from C5 to conus medullaris consistent with demyelination. Cerebrospinal fluid (CSF) culture grew B. pseudomallei, and the patient was given meropenem-cotrimoxazole. After three weeks of parenteral treatment, the lower limbs remained paralyzed. Patient was discharged on oral cotrimoxazole-doxycycline. Conclusions: Melioidosis should be considered as a differential in focal suppurative central nervous system (CNS) lesions, meningoencephalitis, or encephalomyelitis in endemic areas. CNS infections must be ruled out prior to steroid administration. The role of corticosteroids in demyelinating CNS melioidosis has been refuted. This is a rare documentation of effect of unintentional corticosteroid treatment in melioidosis.

Iliac Venous Obstruction: Surgical Reconstruction

Phlebology The Journal of Venous Disease ◽

10.1177/026835550001500313 ◽

2000 ◽

Vol 15 (3-4) ◽

pp. 144-148

Author(s):

F. Mercier ◽

F. Cormier ◽

J. M. Fichelle ◽

F. Duarte ◽

J. M. Cormier

Keyword(s):

Iliac Artery ◽

Lower Limb ◽

Iliac Vein ◽

Lower Limbs ◽

Surgical Reconstruction ◽

Invasive Technique ◽

Venous Obstruction ◽

Long Term Outcome ◽

First Choice

Aim: To review the investigation and treatment of iliac vein obstruction. Method: A review of current literature in the field of management of iliac venous obstruction has been conducted. Synthesis: Iliac venous obstruction results in chronic or acute symptoms in the lower limb presenting as pain, swelling, oedema and discomfort of the lower limb. Intrinsic or extrinsic obstruction of the iliac veins may be the cause. Cockett syndrome is the classic aetiology for chronic intermittent or fixed left inferior limb venous obstruction. Other causes include tumours, vascular grafts or lymph node compression and retroperitoneal fibrosis. Duplex ultrasound imaging is now the first-choice investigation. CT scanning is useful where external vein compression is suspected. Phlebography is used when an endovascular procedure is to be done. The surgical treatment of Cockett syndrome described by Cormier is transposition of the common right iliac artery in the left internal iliac artery. This is being replaced by endovascular balloon venoplasty completed by stenting of the left iliac vein. We reviewed the experience of surgical correction of Cockett syndrome with Cormier's technique in 70 patients operated on between 1976 and 1990; 55 patients had a follow-up of 12-177 months. Anatomical and functional results were perfect for all patients except when endoluminal synechiae or iliac venous thrombosis were associated with postural compression. In this case a 50% success rate was achieved. The endovascular revolution offers a less invasive technique for treatment of chronic iliac venous obstruction. Follow-up is short at present in the few publications found in the literature. Conclusions: Iliac vein obstruction results in symptoms of swelling in the lower limbs. These may be managed conservatively. Where there is an indication for venous reconstruction, investigation by duplex ultrasonography is the first step. Endovascular procedures including stenting offer significant benefit. The long-term outcome of these interventions has yet to be established.

Young adult with varicosities and recurrent lower gastrointestinal bleeding

International Surgery Journal ◽

10.18203/2349-2902.isj20193363 ◽

2019 ◽

Vol 6 (8) ◽

pp. 3012

Author(s):

Vidhyachandra V. Gandhi ◽

Pratik Gautam ◽

Nitin V. Pai ◽

Sujai Hegde

Keyword(s):

Gastrointestinal Bleeding ◽

Sigmoid Colon ◽

Lower Limb ◽

Low Anterior Resection ◽

Lower Gastrointestinal Bleeding ◽

Lateral Side ◽

Left Lower Limb ◽

Left Buttock ◽

Klippel Trenaunay Syndrome ◽

Mr Angiogram

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.

Extradural developmental dural root sleeve cyst presenting as a lumbar paraspinal mass with renal compression in an infant

Journal of Neurosurgery Pediatrics ◽

10.3171/2010.2.peds09324 ◽

2010 ◽

Vol 5 (6) ◽

pp. 586-590 ◽

Cited By ~ 1

Author(s):

Balaji Srinivas ◽

Vivek Joseph ◽

Geeta Chacko ◽

Vedantam Rajshekhar

Keyword(s):

Marked Reduction ◽

Left Kidney ◽

Lower Limbs ◽

Resonance Imaging ◽

Abdominal Organs ◽

Left Lower Limb ◽

Extradural Cyst ◽

The Right ◽

Slow Growing

Spinal extradural cysts do not normally present as a visible paraspinal mass or cause compression of the abdominal organs. The authors describe the case of a 9-month-old boy with multiple spinal extradural cysts. The largest of these cysts was along the right L-2 nerve root with significant extraspinal extension resulting in a visible slow-growing swelling in the right paraspinal region and radiological evidence of compression of the right kidney with hydronephrosis. Another large cyst along the left T-12 root caused radiologically evident compression of the left kidney but to a lesser degree. The patient also had monoparesis of the left lower limb and phenotypic features of Noonan syndrome. The authors performed marsupialization of the cysts, as well as repair of the fistula between the subarachnoid space and the cyst on the right side along the L-2 root and on the left side along the T-12 root. At 1-year follow-up, there was no paraspinal mass and the lower limbs exhibited normal power. Magnetic resonance imaging confirmed marked reduction in the size of the cysts and relief of the renal compression. To the authors' knowledge, their patient is the youngest reported in literature to have a spinal extradural cyst and also the first with the cyst presenting as a paraspinal mass.