Lymphatic disorders
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie in Bailey & Love's Short Practice of Surgery, 2018
These are associated with megalymphatics. The diagnosis may be obvious if accompanied by lymphoedema and lymphangioma. However, some patients develop chylous ascites and/or chylothorax in isolation, in which case the diagnosis can be confirmed by aspiration and the identification of chylomicrons in the aspirate. Cytology for malignant cells should also be carried out. A CT scan may show enlarged lymph nodes and CT with guided biopsy, laparoscopy or even laparotomy and biopsy may be necessary to exclude lymphoma or other malignancy. Lymphangiography may indicate the site of a lymphatic fistula that can be surgically ligated. Even if no localised lesion is identified, it may be possible to control leakage at laparotomy or even remove a segment of affected bowel. If the problem is too diffuse to be corrected surgically, a peritoneal venous shunt may be inserted, although occlusion and infection are important complications. Medical treatment comprising the avoidance of fat in the diet and the prescription of medium-chain triglycerides (which are absorbed directly into the blood rather than via the lymphatics) may reduce swelling. Chylothorax is best treated by pleurodesis, but this may lead to death from lymph-logged lungs as the excess lymph has nowhere to drain.
Lymphatic malformations
Prem Puri in Newborn Surgery, 2017
Although safe surgical excision is possible in most small LMs, some reports in the past decade have advocated injection sclerotherapy for cases that are located in regions where resection would be too hazardous, for cases that have been incompletely resected, and for recurrent disease. Many studies suggest that in such cases, sclerotherapy and surgical therapy have similar results and the treatment selected should reflect upon the strengths and experience of the treating team.81,82 No carefully controlled study comparing the efficacy of sclerotherapy to surgery has ever been reported. Most articles describing surgical treatment use it as the primary treatment modality, whereas sclerotherapy is described as primary therapy in most cases (approximately 70%) but as secondary therapy following surgical resection in 25%.82 The successful surgical management of complex (often mixed or microcystic) LM is often staged. In each resection, a surgeon should focus on a defined anatomic region, attempt to limit blood loss, perform as thorough a dissection as possible and be prepared to operate as long as necessary. Even with such an intensive approach to resection, subsequent “recurrence” is as high as 40% after an incomplete excision and 17% after a macroscopically complete excision.83 In some patients, persistent disease at the margins can recur as vesicles along the suture line, so-called lymphangioma circumscriptum (Figure 87.6). Multiple methods have been described to treat lymphangioma circumscriptum including surgical excision, lasers, sclerotherapy, electrocoagulation therapy, and topical sirolimus.
Basic dermatology in children and adolescents
Joseph S. Sanfilippo, Eduardo Lara-Torre, Veronica Gomez-Lobo in Sanfilippo's Textbook of Pediatric and Adolescent GynecologySecond Edition, 2019
The preferred treatment of lymphangioma is complete surgical excision, though recurrence rates are high. Carbon dioxide lasers have been used to treat superficial lymphatic malformations, and sclerotherapy has been used to successfully treat deeper lymphatic malformations. If lymphatic malformation is suspected, referral to dermatology is warranted.42
Isolated cranio-orbitofacial neurofibroma mimicking vascular malformation
Published in Orbit, 2018
Harinder S. Chahal, Brandon Kuiper, Puneet S. Braich, A. Tyrone Glover
We were led to a presumptive diagnosis of lymphangioma because of the cystic-lobulated appearance of the tumor, along with bony remodeling and extension into various anatomic compartments, combined with the initial presentation in early childhood. Despite the presence of pain, the absence systemic signs of neurofibromatosis made the diagnosis of a neurofibroma less likely. The presumptive diagnosis of lymphangioma, along with the patient’s initial reluctance, led us to postpone surgery. The final diagnosis of isolated neurofibroma, therefore, may have had significant diagnostic, prognostic, and treatment implications if discovered earlier in the management of this unusual case.
Eyelid and Orbital Involvement in HIV Infection – An African Perspective
Published in Ocular Immunology and Inflammation, 2020
David Meyer, Derrick P. Smit
Clinical presentation: On the eyelid, KS usually presents as painless, violet-brown single or multiple lobules of varying size (Figure 6a). On the conjunctiva (mostly inferior fornix and tarsal plate) it appears as reddish-blue, vascularized, nodular or diffuse subconjunctival bulbar lesions (Figure 6b). A localized or diffuse subconjunctival hemorrhage is often the presenting complaint. This may mimic a lymphangioma. Ophthalmologists often see oral mucosal lesions associated with eyelid KS (Figure 7a,b).
Clinical, radiological and histological correlation in diagnosis of orbital tumours
Published in Cogent Medicine, 2019
Ralene Sim, Stephanie Ming Young, Rupesh Agrawal, Gangadhara Sundar
Illustrative case 1 (Figure 2) is a 7-year-old girl who presented with fullness of the right lower lid on primary gaze which became more prominent on looking up. On examination of the right inferior forniceal conjunctiva, there was a light pink coloured mass which appeared compressible. Clinical impression was that of lymphangioma. However, the radiologist reported that no intraorbital or periorbital mass lesion was detected. Excision biopsy revealed a capillary haemangioma on histopathological examination.
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