Abnormal Uterine Bleeding in a Patient with Klippel-Trenaunay Syndrome

Milman, Tal; Murji, Ally; Papillon-Smith, Jessica

doi:10.1016/j.jmig.2018.11.004

Cited by 5 publications

(5 citation statements)

References 5 publications

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“…Surgical management for massive obstetric haemorrhage is case dependent but to date emergency hysterectomy has been the most common treatment for uncontrollable bleeding in the presence of diffuse uterine venous malformations 11 21. Uterine artery and internal iliac artery embolisation has also been used with varying degrees of success,21 24 in addition to extraperitoneal pelvic packing, which has also been described in non-obstetric abdominopelvic surgeries 21 25. We believe we are the first to report the use of ‘uterine sandwich’ to successfully control major PPH in the presence of diffuse uterine venous malformations and atony 10…”

Section: Discussionmentioning

confidence: 92%

Management of postpartum haemorrhage in a patient with Klippel-Trènaunay syndrome

et al. 2022

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Klippel-Trènaunay syndrome is a rare congenital disorder characterised by vascular malformations, which may be exacerbated during pregnancy and pose considerable thromboembolic and haemorrhagic risk for obstetric patients. We report on a patient with this syndrome who underwent elective caesarean section which was indicated due to previous obstetric anal sphincter injury. We describe her multidisciplinary preoperative planning and successful management of major postpartum haemorrhage with uterine compression sutures and intrauterine balloon tamponade (‘uterine sandwich’).

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Section: Discussionmentioning

confidence: 92%

Management of postpartum haemorrhage in a patient with Klippel-Trènaunay syndrome

et al. 2022

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“…The radiologic embolization of uterine arteries and subsequent abdominal hysterectomy was ineffective, and finally, bleeding was controlled with the use of intravenous low-dose heparin and antithrombin III. Milman et al ( 8 ) described the case of a 45-year-old woman with KTS with abnormal uterine bleeding treated with total laparoscopic hysterectomy because of the smaller size of her uterus (15.7 × 6.9 × 5.3 cm). Rodriguez Peña and Ovando et al ( 9 ) exposed the case of a 14-year-old female patient with macroscopic hematuria and metrorrhagia due to the presence of multiple angiomatous lesions in the bladder managed with a selective arterial embolization of the internal and external iliac territories and then with laser endo-coagulation of the bleeding foci.…”

Section: Resultsmentioning

confidence: 99%

Uterine Involvement in Klippel–Trenaunay Syndrome: A Rare But Relevant Event. Review of the Literature

et al. 2022

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IntroductionKlippel–Trenaunay syndrome (KTS) is a rare vascular congenital disorder characterized by the classical triad of port-wine stains, abnormal growth of soft tissues and bones, and vascular malformations. The involvement of the genitourinary tract and of the uterus in particular is extremely infrequent but relevant for possible consequences.MethodsWe performed an extensive review of the literature using the Pubmed, Scopus and ISI web of knowledge database to identify all cases of KTS with uterine involvement. The search was done using the MeSH term “Klippel–Trenaunay syndrome” AND “uterine” OR “uterus.” We considered publications only in the English language with no limits of time. We selected a total of 11 records of KTS with uterine involvement, including those affecting pregnant women.ResultsKlippel–Trenaunay syndrome was described for the first time in the year 1900 in two patients with hemangiomatous lesions of the skin associated with varicose veins and asymmetric soft tissue and bone hypertrophy. Uterine involvement is a rare condition and can cause severe menorrhagia. Diagnosis is based on physical signs and symptoms. CT scans and MRI are first-choice test procedures to evaluate both the extension of the lesion and the infiltration of deeper tissues before treatment. The management of Klippel–Trenaunay syndrome should be personalized using careful diagnosis, prevention and treatment of complications.ConclusionKlippel–Trenaunay syndrome is a rare vascular malformation with a wide variability of manifestations. There are no univocal and clear guidelines that suggest the most adequate monitoring of the possible complications of the disease. Treatment is generally conservative, but in case of recurrent bleeding, surgery may be needed.

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“…One case report of heavy menstrual bleeding in patients with KTS showed that the bleeding is controlled mainly by hormonal therapy (e.g., luteinizing hormone-releasing hormone analogs and progestogen) [ 6 ]. However, another case report described a patient who was unresponsive to hormonal therapy [ 7 ]. In the management of KTS, Jacob et al reported that 9 of 252 patients with KTS had a pulmonary embolism associated with invasive procedures, pelvic surgical procedures, oral contraceptives, and pregnancy [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Abnormal uterine bleeding in a female with Klippel Trenaunay syndrome

Pramchand

2022

Arch Clin Cases

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Klippel-Trenaunay syndrome is a rare syndrome that is defined by capillary, venous, and lymphatic malformations associated with limb overgrowth. The author describes a 33-year-old SARS-CoV-2 positive South African Black Zulu female known with Klippel Trenaunay syndrome in this case report. Her presentation with abnormal uterine bleeding and multiple cystic lymphangiomas has been rarely described in existing literature, which provides further understanding of the gynecological manifestations of this syndrome.

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Abnormal Uterine Bleeding in a Patient with Klippel-Trenaunay Syndrome

Cited by 5 publications

References 5 publications

Management of postpartum haemorrhage in a patient with Klippel-Trènaunay syndrome

Management of postpartum haemorrhage in a patient with Klippel-Trènaunay syndrome

Uterine Involvement in Klippel–Trenaunay Syndrome: A Rare But Relevant Event. Review of the Literature

Abnormal uterine bleeding in a female with Klippel Trenaunay syndrome

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