Cerebrospinal fluid-lymphatic fistula causing spontaneous intracranial hypotension in a child with kaposiform lymphangiomatosis.

Spontaneous intracranial hypotension is an uncommon etiology of secondary headaches in children. We report a unique case of a girl with kaposiform lymphangiomatosis who developed postural headaches and imaging features of spontaneous intracranial hypotension without a spinal extradural collection. The girl underwent dynamic computed tomography myelography which revealed a cerebrospinal fluid (CSF)-lymphatic fistula related to a lymphatic malformation associated with the right T10 nerve. She underwent surgical ligation of the CSF-lymphatic fistula, resulting in resolution of the headaches. Spinal CSF-lymphatic fistulas are rare and have previously been reported in two patients with Gorham-Stout disease. The current report suggests that patients with systemic lymphatic anomalies who develop postural headaches should undergo evaluation for spontaneous intracranial hypotension and a CSF-lymphatic fistula. If discovered, surgical ligation is a potential treatment.

Definition and severity grading of postoperative lymphatic leakage following inguinal lymph node dissection.

PURPOSE: Lymphatic complications occur frequently after radical inguinal lymph node dissection (RILND). The incidence of lymphatic leakage varies considerably among different studies due to the lack of a consistent definition. The aim of the present study is to propose a standardized definition and grading of different types of lymphatic leakage after groin dissection. METHODS: A bicentric retrospective analysis of 82 patients who had undergone RILND was conducted. A classification of postoperative lymphatic leakage was developed on the basis of the daily drainage output, any necessary postoperative interventions and reoperations, and any delay in adjuvant treatment. RESULTS: In the majority of cases, RILND was performed in patients with inguinal metastases of malignant melanoma (n = 71). Reinterventions were necessary in 15% of the patients and reoperations in 32%. A new classification of postoperative lymphatic leakage was developed. According to this definition, grade A lymphatic leakage (continued secretion of lymphatic fluid from the surgical drains without further complications) occurred in 13% of the patients, grade B lymphatic leakage (persistent drainage for more than 10 postoperative days or the occurrence of a seroma after the initial removal of the drain that requires an intervention) in 28%, and grade C lymphatic leakage (causing a reoperation or a subsequent conflict with medical measures) in 33%. The drainage volume on the second postoperative day was a suitable predictor for a complicated lymphatic leakage (grades B and C) with a cutoff of 110 ml. CONCLUSION: The proposed definition is clinically relevant, is easy to employ, and may serve as the definition of a standardized endpoint for the assessment of lymphatic morbidity after RILND in future studies.

Quantity-guided drain management reduces seroma formation and wound infections after radical lymph node dissection: results of a comparative observational study of 374 melanoma patients.

BACKGROUND: Lymphatic fistulas are common complications after lymph node dissections in melanoma patients. We investigated whether drain management could improve the patient's outcome. METHODS: Patients who underwent axillary or inguinal lymph node dissection (RALND or RILND) for malignant melanoma were recorded in a prospective database. Two different methods of drain management were compared. Either the drain was removed no later than the eighth postoperative day (period I, 2003-2007) or it was left in place until fluid flow was below 50 ml in 24 h for two consecutive days (period II, 2008-2011). The main outcome criterion was the incidence of seroma punctures after drain removal. RESULTS: 374 patients were analysed. The incidence of seroma punctures significantly decreased in period II. The number of patients with elevated lymphatic secretions rose by 41.3% (RALND) and 38.1% (RILND). With the exception of lymphatic fistulas, we observed significantly more local complications with need for treatment in period I (n = 104, 52%) than in period II (n = 31, 18%). In period II, the hospital stays after both procedures were significantly reduced. CONCLUSIONS: We conclude that quantity-guided drain management leads to a prolonged interval of drainage but is associated with a lower incidence of seroma formation and shorter hospital stay.

Central Lymphatic Imaging in Adults with Spontaneous Chyluria.

Purpose: Chyluria is a rare condition primarily prevalent in developing countries in tropical regions. In chyluria, there exists the communication between lymphatic vessels and the urinary tract, but the specific mechanism of this communication remains undocumented. The objective of this study was to assess the morphology of the main lymphatic vessels including the uro-lymphatic fistula, the thoracic duct using Magnetic Resonance Lymphangiography (MRL) and Intranodal Lymphangiography (IL). Materials and Methods: A retrospective study spanning five years, from January 2020 to January 2024, included 43 patients diagnosed with chyluria through cystoscopy and quantitative urine testing for triglycerides. These patients underwent MRL and then IL for uro-lymphatic fistula embolization. Results: The study involved 43 patients with an average age of 66.1 ± 19.5 years, with a male-to-female ratio of 1:2. Uro-lymphatic fistula occurred predominantly in the left kidney (72.1%), followed by the right kidney (20.9%), and both sides (7%). MRL imaging showed the thoracic duct in 100% of cases but visualized only 84.5% of the uro-lymphatic fistulas. In contrast, IL imaging showed the thoracic duct in 51.5% of patients but visualized uro-lymphatic fistulas in 100% of cases. In the procedure of IL, the average visualization time of the thoracic duct was 45 minutes, with a range of 35 to 69 minutes. Conclusion: MRL and IL complement each other in diagnosing the main lymphatic vessels in chyluria patients. The observed circulatory stasis in the thoracic duct supports the hypothesis that it contributes to increased pressure in the thoracic duct and the formation uro-lymphatic fistula as collateral circulations.

Management of a decade-old recurrent chylothorax with breast fistulization: a case report.

Background: Chylothoraces result from traumatic or non-traumatic insult to the thoracic duct, allowing for lymph to accumulate in the chest. Patients present with increasing dyspnea and fatigue, and the diagnosis is made via chest X-ray, computed tomography (CT), and comparative analysis of the pleural fluid and serum. Management largely entails diet modification and drainage with or without adjunct medications, reserving pleurodesis, percutaneous duct embolization, or thoracic duct ligation for recalcitrant cases. Case Description: A 72-year-old female presented with a 10-year history of recurrent chylothorax. This was precipitated by a rib biopsy in 2013 for concerns of fibrous dysplasia, which was complicated by pneumothorax requiring chest tube placement and recurrent chylous effusion. The patient remained minimally symptomatic despite its chronicity. The lymphatic leak fistulized into her right breast in 2019 to cause significant swelling and exacerbate discomfort. Upon presentation, she noted incessant dyspnea, right arm and breast lymphedema, and exercise intolerance. Initial treatment involved placement of an indwelling pleural catheter (IPC) and interventional radiology (IR)-guided thoracic duct embolization. When persistent, she proceeded with video-assisted thorascopic surgery (VATS) decortication, talc pleurodesis, and ligation of the chylous leak. She was discharged with a drain that remained for one month until sinograms displayed resolution of the effusion. Conclusions: Optimal chylothorax management remains debatable as it is understudied with few high-quality trials guiding treatment. When conservative management is unsuccessful, procedural intervention is often required to minimize morbidity and mortality. A literature review yielded sparse similarities between our case and others, highlighting the irregularity of presentation, challenges faced, and importance of a multidisciplinary approach in management.

[Complications and their management following axillary, inguinal and iliac lymph node dissection]. / Komplikationen und deren Management nach axillärer, inguinaler und iliakaler Lymphknotendissektion.

Irrespective of numerous technical developments, lymphadenectomy remains a necessary component of surgical tumor therapy. Depending on the extent and anatomical localization, complications associated with the lymph vessels such as lymphoceles, lymphatic fistulas or secondary lymphedema can occur with varying frequency, despite a meticulous dissection technique. Chronic lymph fistulas or lymphoceles often require interventional or surgical procedures. Pedicled or free microsurgical flaps are often required in the case of coexisting wound healing disorders or skin soft tissue defects, especially in an irradiated area. For secondary lymphedema a number of conservative and surgical treatment methods have been established. Adequate guideline-based conservative treatment is the method of first choice. If this does not lead to the desired result, microsurgical reconstructive, deviating or resecting procedures are available.

Uro-lymphatic fistula associated with urolithiasis: A case report.

Uro-lymphatic fistulas are rare, and involve communication between the renal collecting system and the lymphatic system. The disorder is usually caused by the obstruction of lymphatic vessels due to several diseases, leading to chyluria. Here, we report the case of a patient with a uro-lymphatic fistula, considered to be associated with urolithiasis.

Unusual Left Periclavicular Cutaneous Lymphatic Fistula After Port Explantation Without Lymph Vessel Injury: Imaging and Interventional Treatment.

Complex oncological treatment can be associated with lymphatic vascular injury that is burdened by considerable morbidity. Lymphatic imaging and interventional techniques offer new minimally invasive treatment options. We report the case of a 59-year-old woman with an unusual lympho-veno-cutaneous fistula, diagnosed by magnetic resonance lymphangiography and treated by minimally invasive embolization therapy and venous recanalization.

Treatment of postoperative high-volume lymphatic complications using isosulfan blue.

BACKGROUND: Lymphocele (LC) and lymphocutaneous fistula (LF) are infrequent but serious complications that occur when lymphatics are disrupted during a vascular procedure. Conservative management with bed rest, extremity elevation, aspiration, and pressure dressing is often ineffective. This study evaluated the effectiveness of isosulfan blue (ISB) to identify disrupted lymphatics for ligation. METHODS: Between 1998 and 2016, there were 33 lymphatic complications treated with ISB-directed ligation in 32 patients. The patients' records were retrospectively reviewed, recording demographics, comorbid conditions, index vascular operation causing the lymphatic complication, details of the procedure done to treat the lymphatic complication, and outcomes. In each patient, between 1 and 3 mL of ISB was injected in the subcutaneous tissue of the interdigital web space. The wound associated with the lymphatic complication was opened. The appearance of dye within the wound identified disrupted lymphatic ducts for suture ligation. RESULTS: The lymphatic complications were either LC (11 [33%]) or LF (22 [66%]) and were associated with femoral vein harvest (9), great saphenous vein harvest (8), exposure of femoral arteries (13), creation of an upper extremity fistula (1), repeated femoral access for coronary angiography, or excision of an LC (1). Most patients were male (66%), and the mean age was 56.8 ± 13.1 years. In comparing patients with LF and LC, the diagnosis of LF was made earlier (13.8 ± 7.0 days vs 23.4 ± 14.1 days; P = .02), and treatment occurred sooner for LF than for LC (22.1 ± 8.1 days vs 48.8 ± 51.2 days; P = .02). In all patients, ISB identified one or more disrupted lymphatics. The appearance of the ISB dye within the wound after injection was rapid, often within 5 to 10 minutes. After ligation of the lymphatics, most wounds were closed primarily (26 [79%]), but a muscle flap (5 [15%]), negative pressure dressing (1 [3%]), and dressing changes (1 [3%]) were also used. Wound healing was achieved in all patients on average 32.5 ± 21.5 days after lymphatic ligation. CONCLUSIONS: The current series is one of the largest reported experiences using ISB to identify injured lymphatics responsible for LC or LF. Lymphatic complications after a vascular procedure usually occur within 3 weeks of the index vascular procedure, with LF being identified and treated earlier than LC. ISB injection rapidly identifies disrupted extremity lymphatics. Ligation of these lymphatics results in reliable resolution of the lymphatic complication.

Chylous complications of various severity and manifestations within diverse compartments in reconstructive vascular surgery #) #) The manuscript is dedicated to Prof. Dr. Z. Halloul.

INTRODUCTION: Chylous complications, which occur also in the profile of vascularsurgical interventions with considerable frequency, are challenging with regard to their adequate management. Aim & method: Compact short overview on epidemiological, classifying, symptomatic, diagnostic and therapeutic aspects of chylous complications in vascular surgery, based on i) own clinical experiences, ii) a current selection of relevant scientific references and iii) representative case reports from clinical practice. Results (complex patient- & clinical finding-associated aspects): - Basic treatment of lymphedema / postreconstructive edema comprises the complex physical therapy to improve edematous swelling, which need to be usually performed over years. - In case of lymphocele, wait-and-see strategy can be initially pursued to observe spontaneous clinical course. If the lymphocele and its clinical complaints persist, puncture, placement of a drainage or temporary instillation of doxycyclin or ethanol can be attempted. - In case of lymphatic fistula, vacuum-assisted closure dressing, radiation and selective ligation of lymphatic vessels after previous application of methylen blue dye can be used. - Chylascites and chylothorax should be primarily treated - as have been widely established in the mean time - with a consequently conservative approach comprising initially paracentesis / thoracocentesis, protein-enriched and low-fat diet containing middle chain triglycerides (MCT) or total parenteral nutrition combined with the application of a somatostatin analogue (surgical approach as ultima ratio only aiming at ligation of the lesioned lymphatic vessel - if necessary, including preoperative consumption of cream). SUMMARY: Chylous complications can be primarily treated with conservatice measures, which should be exploited using a step-wise approach prior to surgical intervention as ultima ratio. CONCLUSION: The experienced vascular surgeon should be aquainted with a sufficient, finding-adapted management of chylous complications. This requires a well-experienced clinician and surgeon with great expertise regarding the interdisciplinary setting comprising of interventional radiology, vascular (abdominal) surgery and partially surgical intensive care.

[Management of lymphatic fistulas in the groin from a surgeon's perspective]. / Management von Lymphfisteln in der Leistenregion aus chirurgischer Sicht.

BACKGROUND: The postoperative occurrence of lymph fistulas in the groin is a complication that should be taken seriously. These fistulas cause an increase in morbidity and can support local and ascending infections. The treatment of this complication ranges from conservative procedures, such as compression dressings and bed rest to operative treatment with detection of the fistulas and ligation, negative pressure wound therapy (NPWT) or even muscle flaps. This review provides an overview of current therapeutic modalities. MATERIAL AND METHODS: On the basis of a current literature search via PubMed, we identified possible treatment options, which are described in this article. RESULTS: The conservative treatment options presented still have an importance in treating groin fistulas. A selection of safe and effective interventional and operative treatments is presented. CONCLUSION: If there are indications for an interventional or operative treatment a variety of safe and effective therapies are available, which can significantly reduce the length of hospital stay. The option of treatment using a muscle flap is of value as a last resort in the treatment of infected vascular prosthesis in the groin of Szilagyi type III and should be used when necessary.

[Radiotherapeutic management of lymphatic fistulas : An effective but disregarded therapy option]. / Strahlentherapeutisches Management von Lymphfisteln : Eine effektive, aber wenig beachtete Therapieoption.

BACKGROUND: Lymphatic fistulas and lymphoceles are known complications after vascular surgery of the groin and after extended surgical interventions in the pelvic region. Unfortunately, conservative standard therapies are not always successful. OBJECTIVES: Evaluation of the therapeutic efficacy and related side effects of percutaneous low-dose irradiation in patients with lymphorrhea and definition of its importance. MATERIAL AND METHODS: Current presentation of previously published case series, reviews and guidelines. RESULTS: The use of low-dose irradiation therapy with single doses of 0.3-0.5 Gy leads to a cessation of the lymphatic flow in a high percentage of patients when standard therapies do not show a sufficient effect. With cessation of lymphorrhea irradiation should be terminated. Acute side effects have not been reported and the risk of tumor induction is almost negligible. CONCLUSION: Low-dose irradiation is an effective and very well-tolerated therapeutic alternative in the treatment of lymphatic fistulas and lymphorrhea when conservative therapies are unsuccessful.

Lymphangiography to treat postoperative lymphatic leakage: a technical review.

In addition to imaging the lymphatics and detecting various types of lymphatic leakage, lymphangiography is a therapeutic option for patients with chylothorax, chylous ascites, and lymphatic fistula. Percutaneous thoracic duct embolization, transabdominal catheterization of the cisterna chyli or thoracic duct, and subsequent embolization of the thoracic duct is an alternative to surgical ligation of the thoracic duct. In this pictorial review, we present the detailed technique, clinical applications, and complications of lymphangiography and thoracic duct embolization.

Turbid white urine.

Turbid white urine 'albinuria' is defined as a urine discoloration described as milky or cloudy. One of the most frequent causes of turbid white urine is chyluria complicating filariasis (Table 1). The extant causes of albinuria are non parasitic and rare. Amongst their aetiologies stand excessive mineral sediment excretion such as calciuria and phosphaturia, massive pyuria and fungal infections, and rarely congenital malformations of the lymphatic vessels. Malingering is also possible, in patients adding milk to their urine. We observed a case of albinuria in which the diagnostic work up led to diagnosing an exceptional cause of chyluria in a patient living in a region of Colombia where filariasis is not endemic.