Klippel-Trenaunay syndrome or not? An exploration of atypical presentations.

Klippel-Trenaunay syndrome (KTS) is a rare, congenital disorder typically emerging in early infancy or childhood. The classic presentation of KTS is distinguished by a triad of clinical features: a port-wine stain, early-onset varicosities and limb overgrowth. However, a notable variant of KTS has been documented, characterised by limb shortening rather than lengthening, occasionally referred to as 'inverse KTS'. This report details two cases that display this unusual presentation-both patients had classical features of port-wine stain and varicose veins but both experienced shortening of the affected limb. Whether these cases represent a variant of KTS or a new clinical syndrome altogether is uncertain. They however offer valuable insights into the nuances and breadth of clinical manifestations associated with this syndrome.

Identical anatomy, different symptoms: a comparative case study of median arcuate ligament syndrome in monozygotic twins.

Median arcuate ligament syndrome (MALS) is a rare clinical entity arising from the extrinsic compression of the coeliac axis by the median arcuate ligament. In this report, we detail a unique presentation involving monozygotic twins, both of whom demonstrated anatomical extrinsic compression of the coeliac axis by the median arcuate ligament. Intriguingly, only one twin manifested clinical symptoms consistent with MALS, despite comparable anatomical compression of the coeliac axis observed in both. This case highlights the potential interplay of a genetic or anatomical predisposition to coeliac axis compression and secondary, possibly environmental, factors that lead to the development of clinical symptoms. In this report, we explore various determinants potentially influencing symptomatology in MALS and advocate for the publication of similar case studies to further elucidate this rare condition.

Review of the literature supporting international clinical practice guidelines on iliac venous stenting and their applicability to Australia and New Zealand practice.

BACKGROUND: The overall goal of this report is to provide a high-level, practical approach to managing venous outflow obstruction (VOO). METHODS: A group of vascular surgeons from Australia and New Zealand with specific interest, training, and experience in the management of VOO were surveyed to assess current local practices. The results were analyzed and areas of disagreement identified. After this, the group performed a literature review of consensus guidelines published by leading international organizations focused on the management of chronic venous disease, namely, the Society for Vascular Surgery, American Venous Forum, European Society for Vascular Surgery, American Vein and Lymphatic Society, Cardiovascular and Interventional Radiology Society of Europe, and American Heart Association. These guidelines were compared against the consensus statements obtained through the surveys to determine how they relate to Australian and New Zealand practice. In addition, selected key studies, reviews, and meta-analyses on venous stenting were discussed and added to the document. A selection of statements with >75% agreement was voted on, and barriers to the guideline's applicability were identified. The final recommendations were further reviewed and endorsed by another group of venous experts. RESULTS: The document addresses two key areas: patient selection and technical aspects of venous stenting. Regarding patient selection, patients with clinically relevant VOO, a Clinical-Etiologic-Anatomic-Physiologic score of ≥3 or a Venous Clinical Severity Score for pain of ≥2, or both, including venous claudication, with evidence of >50% stenosis should be considered for venous stenting (Level of Recommendation Ib). Patients with chronic pelvic pain, deep dyspareunia, postcoital pain affecting their quality of life, when other causes have been ruled out, should also be considered for venous stenting (Level of Recommendation Ic). Asymptomatic patients should not be offered venous stenting (Level of Recommendation IIIc). Patients undergoing thrombus removal for acute iliofemoral deep vein thrombosis, in whom a culprit stenotic lesion of >50% has been uncovered, should be considered for venous stenting (Level of Recommendation Ib). CONCLUSIONS: Patients with VOO have been underdiagnosed and undertreated for decades; however, in recent years, interest from physicians and industry has grown substantially. International guidelines aimed at developing standards of care to avoid undertreating and overtreating patients are applicable to Australia and New Zealand practice and will serve as an educational platform for future developments.

RETRACTED: Australian and New Zealand Society for Vascular Surgery clinical practice guidelines on venous outflow Obstruction of the femoral-iliocaval veins.

BACKGROUND: The overall goal of this report is to provide a high-level, practical approach to managing venous outflow obstruction (VOO) in Australia and New Zealand. METHODS: A group of vascular surgeons from the Australian and New Zealand Society for Vascular Surgery with specific interest, training, and experience in the management of VOO were surveyed to assess current local practice. The results were analyzed and areas of disagreement identified. Following this, the group performed a literature review of consensus guidelines published by leading international organizations focused on the management of chronic venous disease, namely the Society for Vascular Surgery, American Venous Forum, European Society for Vascular Surgery, American Vein and Lymphatic Society, Cardiovascular and Interventional Radiology Society of Europe, and American Heart Association. These guidelines were compared against the consensus statements obtained through the surveys to determine how they relate to Australian and New Zealand practice. In addition, selected key studies, reviews, and meta-analyses on venous stenting were discussed and added to the document. Finally, a selection of statements with >75% agreement was voted on, and barriers to the guideline's applicability were identified. RESULTS: The document addresses two key areas: patient selection and technical aspects of venous stenting. Regarding patient selection, patients with a CEAP (Clinical-Etiologic-Anatomic-Physiologic) score of ≥3 or a venous clinical severity score for pain of ≥2, or both, and evidence of >50% stenosis on venography, computed tomography venography, magnetic resonance venography, and/or intravascular ultrasound should be considered for venous stenting (level of recommendation Ib) Patients undergoing thrombus removal for acute iliofemoral deep vein thrombosis, in whom a culprit stenotic lesion has been uncovered, should be considered for venous stenting (level of recommendation Ib). Patients with chronic pelvic pain, deep dyspareunia, postcoital pain affecting their quality of life, when other causes have been ruled out, should be considered for venous stenting (level of recommendation Ic). Asymptomatic patients should not be offered venous stenting (level of recommendation IIIc). CONCLUSIONS: Patients with deep VOO have been underdiagnosed and undertreated for decades; however, in recent years, interest from physicians and industry has grown substantially. The advent of simpler and safer treatment options has revolutionized its management, but, unfortunately, formal training for venous disease has not grown at the same rate. Simplifying the technology and training required can result in inconsistent outcomes. These guidelines are aimed at developing standards of care and will serve as an educational platform for future developments.

Early supported hospital discharge for foot disease: a co-design study.

BACKGROUND: There are more than 10,000 admissions each year in Australia for foot disease, with an average length of hospital stay of 26 days. Early supported discharge (ESD) has been shown to improve patient satisfaction and reduce length of stay without increasing the risk of 30-day readmissions. This research aims to gain consensus on an optimal model of early supported discharge for foot disease. METHODS: Three focus groups were held where preliminary components for an early discharge model, as well as inclusion and exclusion criteria, were identified with a purposefully sampled group of medical, nursing, allied health staff and consumers. Two researchers independently systematically coded focus group transcripts to identify components of an ESD model using an iterative constant comparative method. These components then formed the basis of a three phase Delphi study, with all individuals from the focus groups were invited to act as panellists. Panellists rated components for their importance with consensus established as a rating of either essential or very important by ≥80% of the panel. RESULTS: Twenty-nine experts (including 5 consumers) participated across the two study phases. Twenty-three (3 consumers) participated in the focus groups in phase one. Twenty-eight of the twenty-nine experts participated in the phase 2 Delphi. 21/28 completed round 1 of the Delphi (75% response rate), 22/28 completed round 2 (79% response rate), and 16/22 completed round 3 (72% response rate). Consensus was achieved for 17 (29%) of 58 components. These included changes to the way patients are managed on wards (both location and timeliness of care by the multidisciplinary team) and the addition of new workforce roles to improve co-ordination and management of the patients once they are at home. CONCLUSIONS: A model of early supported discharge that would allow individuals to return home earlier in a way that is safe, acceptable, and feasible may result in improving patient satisfaction while reducing health system burden. Future trial and implementation of the ESD model identified in this study has the potential to make a significant contribution to the experience of care for patients and to the sustainability of the health system.

Intravascular ultrasound-guided laparoscopic division of the median arcuate ligament.

Median arcuate ligament syndrome is a rare and poorly understood condition that can have a significant impact on the quality of life of patients. Diagnosis is often difficult and delayed because of the need to exclude other pathologic processes. Treatment strategies traditionally involve open or laparoscopic division of the median arcuate ligament, with or without vascular reconstruction. This report portrays a case of median arcuate ligament syndrome with compression of two visceral arteries and distal embolic complications. A novel hybrid technique is described using intravascular ultrasound technology to aid in laparoscopic median arcuate ligament division. This allowed real-time intravascular visualization of the compressive segment, guided release of the ligament fibers, and demonstrated confirmation of decompression.

Synovial sarcoma mimicking a traumatic pseudoaneurysm in the upper extremity.

Synovial sarcomas are a rare but aggressive malignancy that primarily affects young patients. Diagnosis is often difficult and delayed due to its insidious onset, heterogenous presentation and mimicry of other pathologies. We present the case of a patient with a history of a slow-growing left arm mass that arose after a traumatic fracture of the humerus. Multimodal imaging was undertaken and reported the mass as being consistent with a vascular malformation of the brachial artery. The patient underwent surgical repair of the artery and intraoperative biopsies confirmed a diagnosis of synovial sarcoma. This case highlights the importance of maintaining suspicion for soft-tissue sarcomas in young patients presenting with a mass, and demonstrates the way in which these tumours may mimic other pathologies both clinically and radiologically. Early referral to a specialist sarcoma centre is key for further investigative workup.

Profound acute limb ischemia affecting all four limbs following cocaine inhalation.

Recreational drug use is a recognized cause of a number of acute vascular events. Cocaine is associated with a number of cardiovascular diseases, including myocardial ischemia, arrhythmias, and aortic dissection. Cutting agents are commonly used to dilute the amount of cocaine required to enhance the profits of the seller. Such cutting agents themselves often provoke acute vascular disease. We present the case of a 34-year-old female presenting with profound ischemia affecting all four limbs secondary to cocaine inhalation.