Abdominal Lymphatic Malformations Research Articles

Abdominal Lymphatic Malformation Misdiagnosed as Ascites: A Case Report and Review of Literature.

Abdominal Lymphatic Malformation Misdiagnosed as Ascites: A Case Report and Review of Literature.

Abdominal Lymphatic Malformations: A Novel Approach in Management

Aim of the studyAbdominal lymphatic malformations (LM) are a challenging clinical entity. Complete excision can be impossible due to the infiltrative nature of some lesions and high rate of complications. Sclerotherapy may require multiple episodes of general anaesthesia and an inability to be definitive in terms of diagnosis and treatment. Subsequently we have adopted a newer algorithm of treatment involving a joint procedure with interventional radiology to enable an intra-operative treatment plan based on the findings. We present our early results. MethodA retrospective observational study in a single tertiary paediatric centre from January 2019 to December 2023. The details of each patient were recorded along with their treatment pathway and outcome. Results12 children underwent treatment for abdominal LM. Under GA and laparoscopic vision, pigtail catheters were inserted into the lesion to effect maximal drainage and assessment of surgical anatomy. If resection was feasible this was undertaken through a laparotomy and if not, the drains were retained and sclerotherapy was started.Three underwent drainage with sclerotherapy without resection and 9 underwent drainage with resection. Of the 3 who underwent sclerotherapy 2 have had further treatment and remain under review at a mean follow up of 7.6 months. Of the other 9 there were no signs of recurrence of the LM at a mean follow up of 29 (range 6–56) months. ConclusionWe have found a combined procedure involving IR-guided drainage under laparoscopic vision enabling curative surgical resection to be undertaken, where feasible, with currently no significant morbidity. For those in whom this is not possible then direct intralesional sclerotherapy is the next most useful treatment modality for longer term control of symptoms. Level of evidenceII.

Clinical characteristics and risk factors for acute abdomen in patients with abdominal lymphatic malformations

ObjectiveThe diagnosis of abdominal lymphatic malformations (ALMs) is often overlooked in clinical practice. However, reports in the literature about ALMs are limited to case reports and series with small sample sizes. This study aimed to review our currently available data to describe the clinical characteristics of ALMs and evaluate the risk factors for acute abdomen caused by ALMs. MethodsWe reviewed the records of patients with ALMs who were diagnosed between December 2008 and January 2023 in our institution. The associations between acute abdomen and ALMs were analyzed based on single-factor and multivariate logistic regression analyses. ResultsThis study included 345 patients with pathologically confirmed ALMs, with a slight female predominance of 1:1.4. Approximately 39.1% (135/345) of patients were asymptomatic, and 24.6% (85/345) presented with acute abdomen. Among the ALMs in the cohort, 42.6% (147/345) were retroperitoneal lymphatic malformations (LMs). The maximal lesion dimensions in patients with acute abdomen and nonacute abdomen were 10.0 cm and 7.8 cm, respectively, with no significant difference based on multivariate analyses. Children were more likely to develop acute abdomen than adults were (P = .002; odds ratio, 5.128; 95% confidence interval, 1.835-14.326). ALMs accompanying acute abdomen were more common for lesions involving the small intestinal mesentery (P = .023; odds ratio, 2.926; 95% confidence interval, 1.157-7.400). ConclusionsALMs are rare with an insidious onset, and retroperitoneal LMs are the most common ALMs, followed by jejunal mesenteric LMs. Our retrospective analysis suggested that young age and small intestinal mesenteric lymphatic malformation are independent risk factors for acute abdomen with ALMs.

Bowel Perforation Following Sclerotherapy of a Massive Intra-Abdominal Lymphatic Malformation

Lymphatic malformations are vascular anomalies that often arise from congenital defects and can manifest in a variety of locations, including the abdomen. Such lesions are often targeted with sclerotherapy, which has become the first-line treatment due to the limitations of surgical intervention. Sclerotherapy is associated with side effects including local necrosis and edema, with localized tissue destruction seldom reported. Here we describe the case of a neonate female with bowel wall perforation following sclerotherapy with ethanol and doxycycline for macrocystic abdominal lymphatic malformation.

Кістозні лімфатичні мальформації кишечника та брижі в дітей: особливості клінічного перебігу та лікувальна тактика

Lymphatic malformations (LMs) - are congenital vascular malformations caused by abnormal embryologic development of the lymphatic system with variable clinical manifestation. Abdominal LMs account approximately 5% of all LMs. The main treatment option for abdominal LMs is surgical. Purpose - to analyze our experience in intestinal and omental LMs diagnosis and treatment, to reveal factors that could influence the complications occurrence risks, that could improve abdominal LMs treatment results in children. Materials and methods. Among 240 pediatric patients, who underwent cystic LMs treatment in a period from December 2012 to July 2022, 43 (19.1%) were diagnosed abdominal and retroperitoneal LMs. 18 (41.8%) patients with intestinal and mesentery affection were included into the study group. Median follow up was 20.7 (95% CI: 12-27) months. Results. Male patients predominated in the study group. Median age at admission was 2.5 (95% CI: 0.75-5) years. 12 (66.6%) had an emergency admission. In 7 (38.8%) children LMs were complicated by intestinal obstruction (n=5; 71.4%), intracystic bleeding and LM cyst infection were observed in n=1, 14.3%) cases both. Surgical treatment was applied in 16 (88.8%) patients, among which 7 (43.7%) underwent laparoscopy. In 4 (25%) cases conversion to laparotomy took place, 5 (31.3%) underwent laparotomic surgeries. No recurrences after surgical treatment were observed. Systemic therapy with mTOR-inhibitors was prescribed in a child with symptomatic total mesentery affection, observational tactics was chosen for asymptomatic child. Conclusions. Intestinal and mesenterial LMs can be asymptomatic or manifest complications, which were observed in 7 (38.8%) children of our group. Macrocystic intestinal LMs are of higher risks of complications development (р=0.0236). Surgical treatment is effective for local intestinal and mesentery affection, excellent treatment result was achieved in 94.1% of patients. In cases with total mesentery affection mTOR-inhibitors systemic therapy is the treatment of choice. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

Anatomy-based treatment strategy for abdominal and retroperitoneal cystic lymphatic malformations in children

Lymphatic malformations (LMs) are rare congenital benign malformations of the lymphatic system, consisting of fluid‑filled cysts or channels with unusual growth. The low incidence of LMs leads to a challenging differential diagnosis and a complicated choice of further treatment strategies. In this study, we analyzed our experience in the treatment of abdominal and retroperitoneal cystic LMs and proposed an anatomy‑based treatment strategy that could improve the treatment outcomes.
 Objective — to prove that the anatomical localization of LM influences treatment effectiveness and should be taken into account when making treatment decisions.
 Materials and methods. Out of 240 pediatric patients who underwent treatment for cystic LMs at a single center from December 2012 to December 2020, 43 (19.1 %) were diagnosed with abdominal and retroperitoneal LMs. The follow‑up period was 3.50 ± 2.16 years.
 Results. Surgical resection of abdominal LMs without evident connection with cisterna chyli does not lead to a recurrence. Sclerotherapy is the best treatment option for retroperitoneal LMs. Diffuse mesentery affection can be successfully treated by sirolimus systemic therapy. According to a logistic regression model, initial choice of LM treatment without considering anatomical localization influences the risks of LM treatment failure (p = 0.000503). All patients in our study group received the following treatments: laparoscopic resections (n = 10, 23.2 %), videoassisted resections (n = 11, 25.5 %), laparotomy resections (n = 9, 20.9 %), sclerotherapy (n = 4, 9.3 %), sclerotherapy and surgery combination (n = 1, 2.3 %), sirolimus systemic therapy (n = 2, 4.6 %), and splenectomy (n = 1, 2.3 %). Six (13.4 %) patients are under dynamic observation. The recommended technique for treating abdominal LMs produced excellent outcomes in 35 (81.4 %) patients, good outcomes in 5 (11.6 %) patients, satisfactory outcomes in 2 (4.7 %) patients, and unsatisfactory outcomes in 1 (2.3 %) patient.
 Conclusions. Treatment strategies for abdominal and retroperitoneal LMs should be based on their anatomical localization. Retroperitoneal localization indicates a high risk of surgical treatment failure (p = 0.0006).

Management of severe symptomatic abdominal lymphatic malformation complicated by abscess formation, protein-losing gastroenteropathy, and bleeding

BackgroundThe optimal strategy for the management of patients with severe symptomatic abdominal lymphatic malformation (ALM) complicated by abscess formation, protein-losing gastroenteropathy, and bleeding has not yet been established. The present study aimed to determine an appropriate management for patients with severe symptomatic ALM, particularly for those with abdominal complications.Materials and methodsEight infants and young adults who underwent emergency surgery for ALM in our department, between the years 1997 and 2020, were selected for the study. We also evaluated and compared the operative procedures, operative timing, and postoperative surgical outcomes.ResultsEmergency resection was performed in all patients with bleeding. Some patients presented with ALMs that invaded the adjacent organs. Resections that included the involved organs were necessary to achieve full resolution in three patients. After evaluating the diagnostic modalities for symptomatic ALM, we also performed elective surgery for patients without bleeding.ConclusionsClinicians should be aware of severe symptomatic ALM with or without bleeding, as well as its associated complications, in order to select the best surgical management plan.

Institutional Management of Abdominal Lymphatic Malformations: Evolution of Treatment Over a Decade

IntroductionAbdominal lymphatic malformations (LM) have been historically managed with surgical resection; however, sclerotherapy and sirolimus have emerged as effective therapies. The purpose of our study is to evaluate our institutional change in management and outcomes for abdominal LM over the past decade. MethodsA retrospective cohort study was performed for all children with an abdominal LM managed at our multidisciplinary Vascular Anomalies Center from 2011 to 2020. Patient demographics, symptoms, treatment, treatment response, and complications were analyzed with descriptive statistics. ResultsTwenty-nine patients with abdominal LM were identified with a median age at treatment of 6 y (interquartile range 3-14). A majority of lesions were identified as macrocystic (n = 18, 62%). The most common intervention was surgery alone (n = 14, 48%) followed by sirolimus alone (n = 4, 14%), and sclerotherapy + sirolimus (n = 4, 14%). Five patients were observed due to lack of symptoms at presentation. Prior to 2017, 91% (10/11) of LM were treated with surgery alone. Following 2017, only 31% (4/13) were treated with surgery alone. Sixty-seven percent (16/24) of treated patients had >95% reduction in LM maximum diameter. A majority of patients (23/24) who received treatment had improvement or resolution of symptoms at median 9-mo follow-up. Only three patients had post-treatment complications, including a drain site infection, small bowel obstruction, and an aspiration event. Complications only occurred after sclerotherapy sessions. ConclusionsOver the study period, our institution has transitioned to initial management of symptomatic abdominal LM with sclerotherapy and/or sirolimus with almost all treated patients having excellent or satisfactory treatment response. Post-treatment complications were rare.

Laparoscopic interventions in children with retroperitonial lymphatic malformations

Introduction. Lymphatic malformations (LM) is the developmental anomaly of lymphatic vessels which is formed at the stage of early embryogenesis. Intraabdominal and retroperitoneal LM are very rare and account for 4 % of all observed LM. Purpose. To clarify the effectiveness of minimally invasive surgical interventions in children with abdominal LM. Material and methods. Over a ten-year period, 15 children with retroperitoneal lymphangiomas , aged from 1 to 17, were operated on in our clinics. To clarify diagnosis, a comprehensive examination was performed: ultrasound, spiral computed tomography, magnetic resonance imaging of the abdominal cavity. Laparoscopic access was applied in all 15 cases for surgical intervention. Results. In all cases, lymphatic malformations were diagnosed before surgery which was later confirmed at laparoscopy. Malformations were excised laparoscopically. In 2 patients, lymphangiomatous tissue was partially left at the site of the greatest fusion with renal and iliac vessels due to a high risk of their damage. Conclusion. Endovideosurgical technologies in children with retroperitoneal LM may be used at any child’s age and at any size of retroperitoneal cystic formation since emptying the cystic cavity creates the necessary working space in the abdominal cavity. Such an approach minimizes surgical trauma, shortens surgical time and facilitates the postoperative period.

Trans-cervical thoracic duct embolization for post-surgical left Chylothorax in a patient with multifocal lymphatic malformations

BackgroundMediastinal and abdominal lymphatic malformations may not be diagnosed until adulthood. Radiologic and pathologic diagnosis is often challenging due to the rarity of the lesion. Surgical excision of these lesions may be curative but lymphatic leak is a known complication. Lymphatic duct embolization may then be required to treat the leak.Case presentationWe describe a patient with post-surgical chylothorax where thoracic duct lymphangiography and embolization was performed by catheterizing the thoracic duct at the venous angle where it drains into the subclavian vein.ConclusionLymphatic duct embolization can be challenging in patients with lymphatic malformations. In these patients, if there is adequate visualization on ultrasound or fluoroscopy, terminal aspect of the thoracic duct can be accessed through the subclavian vein to perform the procedure.

An unusual cause of a toddler with a big belly, abdominal lymphatic malformation case report

: Abdominal pain is one of the most common complaints seen in the pediatric emergency department (PED). Because of the broad range of potential diagnoses, it can pose challenges in the diagnostic evaluation and treatment in the young child. A 3-year-old previously healthy girl presented to our PED with abdominal pain, abdominal distention, poor appetite, and recent weight gain. Physical examination was notable for a significantly distended taut abdomen in an otherwise well appearing child with normal vital signs. Initial evaluation yielded anemia, elevated inflammatory markers, and an abdominal ultrasound (US) showing a large amount of complex intra-abdominal ascites without definite intra-peritoneal mass. Pediatric surgery, pediatric gastroenterology, and pediatric oncology were consulted. A magnetic resonance imaging (MRI) of the abdomen was performed and revealed a large amount of intraperitoneal fluid with a component of internal complexity and no suspicious enhancement to suggest overt malignant process. She was ultimately diagnosed with a large congenital omental cyst that required resection and omentectomy. Pathology was consistent with a macrocystic lymphatic malformation. Lymphatic malformations are uncommon pediatric lesions, accounting for only 5% of benign tumors in childhood. Common locations include the neck, axillae, and rarely involve the gastrointestinal tract. Clinical presentation varies depending on the size and location of the tumor. Many present later in life due to their diagnostic challenges.

Novel treatment of recurrent abdominal lymphatic malformations in a dog

AbstractAn 8‐month‐old neutered male border collie mix was presented for abdominal distension, lethargy and vomiting. Computed tomography revealed a large, lobulated, fluid‐filled cystic mass within the abdomen. Following subtotal surgical resection and histopathology, a diagnosis of lymphangioma (lymphatic malformation) was made. Five months postoperatively, a recurrence of the lymphatic malformation was diagnosed. Multimodal therapy was instituted consisting of subtotal surgical resection, electrochemotherapy, sclerotherapy and radiation therapy. Ten months postoperatively and 7 months post‐radiation therapy, the dog remained without recurrence of clinical signs, and the lymphatic malformation was reduced in size. The combination of surgical resection, electrochemotherapy, sclerotherapy and radiation therapy seemed to be safe therapeutic options for abdominal lymphangiomas and may aid in medium‐term disease control.

Video assisted sclerosis of endoabdominal lymphangiomas with OK432: An effective, safe and minimally invasive choice

Abstract Lymphatic malformations (LM) or lymphangiomas are congenital errors in vascular embryogenesis. Percutaneous sclerosis is considered the best treatment for LM with external localization and yields excellent results. Surgical resection for deep LM is currently the mainstay of therapy, but high recurrence and morbidity rates are reported. Uniform therapeutic protocols are still unavailable. We describe two cases of abdominal LM treated by video-assisted sclerosis with OK432. The first case was a 5-month-old girl with pre-natal diagnosis of a left retroperitoneal LM with vascular encasement. A laparoscopic approach was used to expose the mass and a video-assisted injection of OK-432 in major cysts was performed. A remarkable reduction of the lesion size was observed after 18 months. The second case was a 15-year-old boy who presented at the ER acute abdominal pain. A mesenteric LM was diagnosed and a video-assisted sclerosis of the lesion with OK432 was performed. The LM completely disappeared. Follow-up was of 12 months. In our experience, video-assisted sclerosis of abdominal LM with OK-432 is a valid alternative to surgical resection. The procedure is feasible, safe, effective and minimally invasive; complete resolution may be observed in some cases. Long-term follow-up is required to rule out recurrences.

Lymphatic malformation of abdomen in children; eight year experience from a tertiary care centre.

Introduction: Lymphatic malformations of abdomen include mesenteric, omental and retroperitoneal cysts. These are benign malformations with variable presentation. These malformations may present as abdominal distension, abdominal pain, features of intestinal obstruction and rarely acute abdomen. Ultrasonography, CT scan and MRI provide characteristics of malformations like size, composition and may at times help in determining the origin of the lesion. Surgical exploration confirms the diagnosis besides being therapeutic. The mesenteric and omental cysts are either enucleated or need bowel resection or sometimes, partial cyst excision with marsupliazation. Intraoperative injection of the sclerosing agent is an option in instances when excision is not possible or feasible. We analysed the clinical characteristics, management and follow-up of these cases in our study. Methods: We analysed 26 cases of abdominal lymphatic malformations. The data regarding epidemiological and clinical characteristics were reviewed and recorded. Operative record and imaging findings were noted. The long and short term results of the surgical treatment were analysed. Results: Twenty six cases (18 Male:8 Female) were included in this study. The most common presentation was chronic abdominal symptoms like pain, vomiting or distension. Three patients had antenatal diagnosis while eleven patients had symptoms three months before being diagnosed. The average age of presentation was 48.64 months. Twelve (46.15%) patients had solitary cyst. Fifteen (57.69%) patients had mesenteric cyst, 6(23.07%) had omental cyst and five (19.23%) patients had cyst extending to the retro peritoneum. Twelve patients underwent enucleation. Serous fluid was the content in ten (38.48%) patients. Two patients needed emergency surgery for intestinal obstruction. The average follow-up was 46 months. Two patients required re-exploration after initial incomplete resection. Overall recurrence rate was seen in four cases (15.38%). Conclusion: Lymphatic malformations of abdomen can have varied presentation. Ultrasonography and CT scan or MRI is useful diagnostic tools. Mesenteric cysts need resection anastomosis. Omental cysts are amenable to resection. Multi-loculated and retroperitoneal cyst may need partial excision or sclerotherapy. Long term outcome is excellent.

A multidisciplinary approach to management of abdominal lymphatic malformations

Background/purposeAbdominal lymphatic malformations (LM) are a rare subset of vascular anomaly caused by abnormal development of the lymphatic system. They are classified as macrocystic, microcystic or combination macrocystic and microcystic. Surgical resection, percutaneous sclerotherapy, and medical therapy are all employed to treat these complex and often symptomatic lesions. No standardized treatment algorithm exists currently. The purpose of this study was to establish a multidisciplinary treatment approach to abdominal LMs. MethodsA retrospective observational study was conducted from 2013 to 2019 on patients with abdominal LMs at a single tertiary children's hospital vascular anomalies center. Demographics, imaging, and treatment modality were recorded. Clinical and/or radiographic response to the primary treatment modality as well as complications was the outcomes measured. ResultsNineteen patients (12 macrocystic, 5 microcystic and 2 combined) were identified, with a median age at diagnosis of 2.2 years (range 0.1–20.8 years). Sclerotherapy was the most common primary treatment, followed by surgical resection and sirolimus. No difference in clinical response (p = 0.58) or complications (p = 0.31) was observed based on primary treatment or subtypes. ConclusionsBased on our institutional experience, we propose an LM subtype-based treatment algorithm for abdominal LMs. It employs a multidisciplinary approach, and results in satisfactory patient outcomes with minimal complications. Level of evidenceLevel III, retrospective comparative study.

Malformación linfática de epiplón mayor con clínica de abdomen agudo inflamatorio en un paciente adolescente: reporte de un caso

Antecedentes: Las malformaciones linfáticas peritoneales son un hallazgo excepcional y benigno. Generalmente se presentan en niños como dilataciones linfáticas en cavidad abdominal. Existen muy pocos casos reportados en los que esta malformación aparece en espacios peritoneales y retroperitoneales, siendo el mesenterio del intestino delgado el más común seguido por el peritoneo. Presentación del caso: Somos los primeros en reportar un caso ecuatoriano sobre una malformación linfática peritoneal en un adolescente de 12.5 años con clínica de abdomen agudo. Un año después el paciente no ha reportado recidivas. Conclusiones: Ante la presencia de un cuadro de abdomen agudo no podemos dejar de lado esta patología, destacamos la ecografía como método diagnóstico y de seguimiento. La resección quirúrgica completa es curativa.

Recurrent abdominal pain in a child caused by mesenteric lymphatic malformation

Recurrent abdominal pain in children is often attributed to functional causes and constipation. In atypical presentations, pathology could be of surgical origin. Nine year old boy with recurrent episodes of abdominal pain associated with non-bilious vomiting presented with many normal radiological and haematological studies. At laparotomy, a lymphatic malformation confined to mesentery getting distended following meals causing mesenteric ischemia identified. Children with abdominal lymphatic malformation presenting with this clinical picture is very rare.

Symptom Resolution and Volumetric Reduction of Abdominal Lymphatic Malformations With Sclerotherapy

BackgroundLymphatic malformations (LMs) are congenital and arise from errors in vascular embryogenesis. LMs are categorized by cyst size as microcystic, macrocystic, or combined. Abdominal LMs are rare. Surgical resection of abdominal LMs has been the mainstay of therapy, but recurrence and morbidity are high. We sought to determine the effectiveness of sclerotherapy treatment for abdominal LM. MethodsA single-center, retrospective review from 2014 to 2018 was conducted evaluating pediatric patients with abdominal LM. ResultsTen patients were included, n = 9 had macrocystic LM and one patient had combined disease. The average age at first treatment was 6.8 y. The most common presenting symptoms were abdominal distention, pain, infection, and anemia. Preprocedural imaging was performed for all patients; median pretreatment volume was 1572.9 cm3 (range, 67.2-13,226.4). LMs were accessed using ultrasound guidance and injected with opacified doxycycline. Patients received a mean of 7.1 sclerotherapy injections. Complications included intraperitoneal doxycycline extravasation (n = 1), managed conservatively, and LM infection (n = 1), treated with intravenous antibiotics and drainage. One patient went on to surgical resection due to inability gain stable intracystic access; follow-up ultrasonography showed no recurrence. Postprocedural imaging was available in n = 8. Volume decreased by 96.7% after sclerotherapy. The median remaining volume was 0 cm3 (range, 0-599.7) (P = 0.016). Postsclerotherapy magnetic resonance imaging was obtained in n = 6, with complete resolution in 83.3%. All patients had resolution of presenting symptoms. Follow-up duration was 12.3 mo. ConclusionsInitial results demonstrate that sclerotherapy is an effective and durable treatment for symptom resolution and volume reduction of abdominal LM.

Abdominal lymphatic malformations.

Abdominal lymphatic malformations (LM) are relatively rare findings in the differential diagnosis of focal abdominal lesions; however, they represent achallenge especially in younger patients. The aim of this review article is to provide up-to-date information about the different kinds of LM manifestations. In addition, related syndromes and typical imaging features to facilitate the diagnosis are discussed. The clinical presentation of abdominal LM is unspecific, whereby most are asymptomatic and comprise incidental findings of thin-walled cystic masses anywhere in the abdomen. The fluid in the cystic masses may be proteinaceous, contain blood, or be infected. Radiological imaging features overlap with other cystic diseases; hallmark in LM is alack of asolid component and exclusive enhancement of the walls and septa. In cystic abdominal masses in early childhood or young adults, abdominal LM must be taken into account by the radiologist. Newly defined entities in this spectrum of diseases are central conducting lymphatic anomaly (CCLA) and generalized lymphatic anomaly (GLA).

Abdominal Lymphatic Malformation in Children

Abdominal Lymphatic Malformation in Children