Klippel–Trenaunay syndrome: endoscopic findings

Barbeiro, Sandra; Brásio, R.; Atalaia-Martins, Catarina; Marcos, Pedro; Gonçalves, Cláudia; Alves, Paulo; Rama, N.

doi:10.1055/s-0042-119038

Cited by 3 publications

(4 citation statements)

References 2 publications

(4 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The sigmoid colon and rectum are the most commonly affected areas. Clinical manifestations range from symptom-free to life-threatening gastrointestinal bleeding [6,10]. Thus, condition awareness is paramount for appropriate clinical management.…”

Section: Discussionmentioning

confidence: 99%

“…In terms of treatment, conservative approaches can be used for patients with self-limiting bleeding. Endoscopic treatments such as endoscopic laser therapy and endoscopic clipping [11] can be applied for localized lesions [9], whereas surgical resection such as sphincterpreserving excision of the affected sigmoid colon and rectum followed by a colon pouch anal anastomosis and protective loop ileostomy may be used for patients with more extensive lesions and severe symptoms [10,12]. As a preoperative management for surgical resection, selective feeding vessels angiographic embolization can help reduce intraoperative bleeding [2].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Gastrointestinal bleeding with Klippel–Trenaunay syndrome: a case report

2021

View full text Add to dashboard Cite

Background Gastrointestinal bleeding caused by gastrointestinal tract involvement in patients with Klippel–Trenaunay syndrome (KTS) is extremely rare and often overlooked. Case presentation A 9-year-old girl who presented with chronic gastrointestinal bleeding was admitted to our hospital. Laboratory examinations revealed microcytic hypochromic anemia and a positive fecal occult blood test. Computed tomography (CT) of the lower limbs combined with physical examination confirmed KTS. The pelvic CT showed concentric thickening of the sigmoid colon and rectum, with progressive enhancement after the administration of a contrast agent. Colonoscopy demonstrated vascular malformations of the sigmoid colon and rectum. The patient was finally diagnosed with KTS with gastrointestinal tract involvement. The patient improved after receiving conservative treatment. Conclusions A suspicion of gastrointestinal tract involvement as an etiology for gastrointestinal bleeding should not be overlooked in patients with KTS. Endoscopy and imaging modalities can synergistically help diagnose this condition.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Gastrointestinal bleeding with Klippel–Trenaunay syndrome: a case report

2021

View full text Add to dashboard Cite

show abstract

“…As far as we know, no case of PRL reported was associated to KTS. It is rare condition, with an unknown etiology and an incidence of approximately 1/100 000 live births [7]. 63% of patients with KLS have the manifestation of the complete triad (port-wine stain, varicose veins, and hypertrophy of soft tissues/bones) [8].…”

Section: Case Reportmentioning

confidence: 99%

Unusual association of two rare entities: Primary rectal linitis and Klippel-Trenaunay syndrome

Imane¹,

S²,

A³

et al. 2019

Arch Clin Gastroenterol

View full text Add to dashboard Cite

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder, characterized by the triad of vascular malformations (angioma), venous and or lymphatic malformations and asymmetrical disturbed growth of soft tissues and/or bone. Primary rectal linitis is a rare digestive tumor with very poor prognosis. We report the case of a 41-year-old patient with Klippel trenaunay syndrome who was diagnosed with primary rectal linitis.

show abstract

“…Endoscopic therapy has a limited role because of the commonly diffuse nature of the potential bleeding sites. Surgical resection with partial or complete excision of the involved organ is the mainstay of the management of bleeding as per the literature [2][3][4][5][6][7]. Our team used a modified phlebography technique to develop malformed veins involving the pelvis.…”

Section: Introductionmentioning

confidence: 99%

Internal Iliac Vein Reflux: An Underrecognized Pathophysiology in Klippel-Trénaunay Syndrome With Pelvis Involvement

Wang¹,

Xie²,

Lin³

et al. 2022

Cureus

View full text Add to dashboard Cite

Background and objective Internal iliac vein reflux (IIVR) has been underrecognized in Klippel-Trénaunay syndrome (KTS) with pelvis involvement. In this study, our aim was to report clinical and radiological characteristics, modified phlebography technique, and diagnostic and treatment algorithm and outcomes of IIVR in KTS patients with pelvis involvement. Materials and methods KTS patients diagnosed between May 2014 and January 2021 were retrospectively analyzed. The medical records and imaging studies of all patients with KTS of the lower extremities were included and reviewed. KTS was defined as the triad of capillary malformation, venous malformation, and limb overgrowth. Pelvis involvement was evaluated with MRI. Phlebography was performed if IIVR was suspected. IIVR ablation and sclerotherapy were performed if IIVR was confirmed in KTS patients with external genitalia/perineum manifestation and bleeding. Patients were followed up via outpatient consultations. Episodes of gross bleeding were specifically investigated. Results A total of 211 patients with lower limb KTS diagnosed by our team were included in the study. Unilateral IIVR was diagnosed in 97 patients, and bilateral IIVR in two patients; 117 KTS patients were managed with radiological intervention and/or hybrid surgery by our team. Eleven patients underwent an IIVR ablation procedure due to recurrent bleeding from pelvic organs. Postprocedural complications included transient fever (n=2) and mild anaphylactic reaction (n=1). A small hyperpigmented scar at the incision and/or accessing site was noticed in patients receiving bleomycin during the procedure (n=6). Bleeding episodes and anemia resolved in all patients during the follow-up period. Correspondingly, the involved IIV and its tributaries were found to have disappeared on imaging during the follow-up. Conclusion IIVR is common in KTS patients, and it can cause bleeding from pelvic organs. Bleeding can be managed with IIVR ablation and sclerotherapy in KTS with pelvis involvement.

show abstract

Klippel–Trenaunay syndrome: endoscopic findings

Cited by 3 publications

References 2 publications

Gastrointestinal bleeding with Klippel–Trenaunay syndrome: a case report

Gastrointestinal bleeding with Klippel–Trenaunay syndrome: a case report

Unusual association of two rare entities: Primary rectal linitis and Klippel-Trenaunay syndrome

Internal Iliac Vein Reflux: An Underrecognized Pathophysiology in Klippel-Trénaunay Syndrome With Pelvis Involvement

Contact Info

Product

Resources

About