Summary This comprehensive document covers a broad spectrum of vascular and lymphatic disorders, including common venous conditions like varicose veins and deep
Summary This comprehensive document covers a broad spectrum of vascular and lymphatic disorders, including common venous conditions like varicose veins and deep vein thrombosis, and their complications. It details various vascular ectasias, distinguishes between benign and malignant vascular tumours, and explores intermediate forms like Kaposi Sarcoma and Bacillary Angiomatosis. Finally, it outlines the pathology associated with modern vascular interventions, emphasizing stent complications and graft performance. Key Points Varicose veins result from prolonged increased intraluminal pressure, leading to venous wall weakening and valve incompetence, and can cause chronic venous insufficiency. Deep Vein Thrombosis (DVT) is a major source of pulmonary embolism and is governed by Virchow's Triad, while Trousseau Syndrome signals an underlying occult visceral malignancy. Lymphangitis , often caused by Group A Streptococcus, presents as painful red streaks and can dangerously progress to bacteraemia and sepsis if not promptly treated. Lymphoedema can be primary (e.g., Milroy disease) or secondary, with filariasis being the most common worldwide cause, and chronic cases can lead to lymphangiosarcoma. Sturge-Weber syndrome is characterized by a port wine stain in trigeminal distribution, ipsilateral leptomeningeal angiomas, and neurological symptoms like seizures and intellectual disability. Kaposi Sarcoma , caused by HHV8, presents in four distinct epidemiological forms, with AIDS-associated KS being the most common malignancy in HIV-positive individuals prior to ART. Angiosarcoma , a highly malignant endothelial neoplasm, has various aetiologies including arsenic exposure, Thorotrast, PVC, radiation, and Li-Fraumeni syndrome, and carries a poor prognosis. Drug-eluting stents significantly reduce restenosis but delay reendothelialisation, necessitating prolonged dual antiplatelet therapy to prevent catastrophic late stent thrombosis. Detailed Notes SECTION 1 — VENOUS DISORDERS Varicose Veins Definition: Abnormally dilated, tortuous superficial veins from prolonged increased intraluminal pressure causing progressive venous wall weakening and valve incompetence Epidemiology: Superficial veins of the leg affected in up to 20% of men and 33% of women Also occur in: oesophagus (portal hypertension), anorectal region (haemorrhoids), spermatic cord (varicocele), broad ligament of uterus Risk factors: Obesity, pregnancy (compress IVC), prolonged standing, familial predilection (familial valvular incompetence) Pathophysiology: Elevated intraluminal pressure → venous wall dilation → valve incompetence → worsening venous stasis → more dilation (self-perpetuating cycle) Morphology: Tortuous, dilated, thickened veins with irregular lumens Intimal fibrosis, medial smooth muscle hypertrophy, calcifications ( phleboliths ) Marked variation in wall thickness Clinical consequences: Chronic venous insufficiency → stasis dermatitis = brawny induration ( haemosiderin deposits from haemolysis of extravasated RBCs → brownish skin discolouration + fibrosis) Stasis ulcers (especially medial malleolus — most prone to ulceration) Stasis predisposes to secondary thrombophlebitis and secondary infection Embolism from superficial varicosities is very rare — contrast with DVT Deep Vein Thrombosis (DVT) / Thrombophlebitis Two overlapping entities together account for 90% of clinical venous disease: Phlebothrombosis = thrombus in vein with minimal inflammation Thrombophlebitis = thrombosis with significant venous wall inflammation DVT = major source of pulmonary embolism (popliteal, femoral, iliac veins most dangerous) Pathogenesis — Virchow's Triad: Component Examples --- --- Stasis Immobilisation, CCF, pregnancy, obesity, long-haul travel Hypercoagulability Factor V Leiden, protein C/S deficiency, antiphospholipid syndrome, malignancy, OCP Endothelial injury Surgery, trauma, indwelling catheters, previous phlebitis Trousseau Syndrome: Migratory thrombophlebitis simultaneously or sequentially involving different venous sites Classically associated with occult visceral malignancy (pancreatic, GI, lung cancers) Mechanism: tumour secretes procoagulant factors (tissue factor, mucins activating Factor X/VII) Unexplained migratory thrombophlebitis requires mandatory malignancy work-up. Clinical features of DVT: Leg pain, swelling, erythema, warmth, Homan's sign (unreliable) Superficial DVTs: tender, indurated cord-like vein palpable Deep DVTs: often clinically silent until PE Chronic post-thrombotic syndrome: chronic oedema, pain, skin changes, ulceration SECTION 2 — LYMPHATIC DISORDERS Lymphangitis Definition: Acute inflammation of lymphatics, most commonly from group A β-haemolytic Streptococcus (also Staphylococcus aureus ) Clinical features: Painful red streaks tracking proximally from a wound/infection Regional lymphadenitis (tender enlarged draining nodes) If bacteria are not contained within draining lymph nodes, they can escape into the venous circulation, leading to bacteraemia and sepsis — this is the feared complication. Treatment: urgent antibiotics Lymphoedema Definition: Accumulation of interstitial fluid from impaired lymphatic drainage → progressive accumulation of protein-rich fluid → fibrosis Classification: Type Subtypes Mechanism --- --- --- Primary Milroy disease (heredofamilial) Congenital lymphatic agenesis/hypoplasia; hereditary; present from birth Simple congenital lymphoedema Present at birth, non-hereditary Lymphoedema praecox Develops before age 35, most common primary form Lymphoedema tarda Develops after age 35 Secondary Post-surgical Axillary clearance → arm lymphoedema Radiation-induced Fibrosis of lymphatics after cancer treatment Malignant obstruction Tumour invasion/compression of lymph nodes Filariasis Most common worldwide cause — Wuchereria bancrofti obstructs lymphatics → massive " elephantiasis " Morphology: Pitting oedema in early stages → progressive accumulation Protein-rich fluid stimulates fibroblast activity → brawny (non-pitting) induration + peau d'orange skin changes Chronic fibrosis with subcutaneous fibrous thickening Complications: Skin ulceration and secondary infection Lymphangiosarcoma (Stewart-Treves syndrome) — malignant transformation in chronic longstanding lymphoedema, especially post-mastectomy Chylous complications: chylous ascites, chylothorax, chylopericardium from ruptured thoracic/abdominal lymphatics SECTION 3 — VASCULAR ECTASIAS Definition: Localised dilatations of pre-existing vessels — NOT true neoplasms Nevus Flammeus (Port Wine Stain) Most common form of vascular ectasia Flat, pink-to-purple macular patch on the head/neck composed of dilated dermal capillaries and venules Port wine stains: persist and progressively grow with the child; the overlying skin may thicken and darken with age — do NOT regress (unlike most naevi) Most are sporadic with no systemic association A port wine stain in the trigeminal distribution should prompt consideration of Sturge-Weber syndrome . Sturge-Weber Syndrome (Encephalotrigeminal Angiomatosis) Somatic mosaic GNAQ missense mutation in the Gα subunit (causes constitutive G-protein signalling) Triad: 1. Port wine stain in the trigeminal nerve distribution (V1/V2) 2. Ipsilateral leptomeningeal angiomas (venous malformations in pia mater) 3. Neurological: seizures, intellectual disability , contralateral hemiplegia, glaucoma Skull radiograph: "tram-track" calcifications (cortical calcifications following gyral patterns) Not inherited — sporadic somatic mutation occurring early in development Not all patients have the complete triad; variable expression is common. Spider Telangiectasia (Spider Angioma) Pulsatile, radiating subcutaneous arterioles emanating from a central arteriole Blanch with direct pressure (due to arterial supply — unlike venous lesions) Associated with hyperestrogenic states : pregnancy, liver cirrhosis, oral contraceptives Oestrogen → vascular smooth muscle relaxation → arte