Intra-abdominal lymphatic malformation management in light of the updated International Society for the Study of Vascular Anomalies classification

Elbaaly, Heba; Piché, Nelson; Rypens, Françoise; Kleiber, Niina; Lapierre, Chantale; Dubois, Josée

doi:10.1007/s00247-020-04930-8

Cited by 12 publications

(10 citation statements)

References 36 publications

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“…Most patients presented with mild to moderate symptoms, generally pain, mass, or distension [2]. 95.8% of our patients needed either sclerotherapy or surgery, with interventional treatment rates ranging from 60-100% similar to other reports [16]. Sclerotherapy was highly effective with only 4 of 16 sclerotherapy patients needing subsequent treatment with laparoscopic or open surgery.…”

Section: Figure 2: Magnetic Resonance Imaging Of Extensive Intra-abdo...supporting

confidence: 74%

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2024

IJCMCR

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Background: Intra-abdominal lymphatic malformations are rare entities without a clear consensus on management or prognosis. These malformations are medically and surgically complex and often require a sophisticated, multi-disciplinary approach to treatment. Methods:A single quaternary institutional, retrospective chart review between January 2000-December 2019 was performed on all patients with an initial diagnosis of a non-solid organ intra-abdominal lymphatic malformation. Demographics, presentation, treatment, and outcome were reviewed.Results: Twenty-four patients were identified, 62.5% were male. Median age at time of diagnosis was 2.5 years old, 33% were present at birth, 50% presented before 2 years old, and 29% presented ≥10 years of age. Seventy-five percent were lymphatic (majority macrocystic), 20.8% were mixed lymphatic-venous malformations (LVM). Fifty-four percent had other associated lymphatic or vascular malformations including 4 patients with PIK3CA-Related Overgrowth Spectrum (PROS), 3 with Generalized Lymphatic Anomaly, and 2 with Capillary Venous Lymphatic malformation (CVLM). Patients most often presented with abdominal pain (50%). An intervention was performed in 95.8% and 87.5% were managed initially with sclerotherapy. Most interventions were performed for abdominal symptoms either before three years of age or during adolescence. Laparoscopic or open surgery was performed in 41.7% of patients. Surgery was performed as primary therapy in 8.3% of the group. Conclusion:Intra-abdominal lymphatic malformations appear to have a bimodal distribution pattern of presentation; shortly after birth and again at puberty. Most patients present with abdominal symptoms and have other associated vascular anomalies. MRI appears to be the most accurate modality for treatment planning. Interventional procedures such as sclerotherapy and medical therapy with sirolimus are safe and effective and may be considered first line therapies. Surgical resection may be required when the diagnosis is in question or when more conservative therapies are no longer efficacious and is typically an option of last resort.

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Section: Figure 2: Magnetic Resonance Imaging Of Extensive Intra-abdo...supporting

confidence: 74%

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2024

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“…2 95.8% of our patients needed either sclerotherapy or surgery, with interventional treatment rates ranging from 60-100% similar to other reports. 15 Sclerotherapy was highly effective with only 4 of 16 sclerotherapy patients needing subsequent treatment with laparoscopic or open surgery. Sclerotherapy at our institution is typically image guided by ultrasound and fluoroscopy, the lesion aspirated, fluid collections drained, and doxycycline instilled as the sclerosing agent.…”

Section: Discussionmentioning

confidence: 97%

Intra-abdominal Lymphatic Malformations: A Case Series

Brungardt

Cooke-Barber

Tiao

et al. 2022

Preprint

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Background: Intra-abdominal lymphatic malformations are rare entities without a clear consensus on management or prognosis. These malformations are medically and surgically complex and often require a sophisticated, multi-disciplinary approach to treatment. Methods: A single quaternary institutional, retrospective chart review between January 2000-December 2019 was performed on all patients with an initial diagnosis of a non-solid organ intra-abdominal lymphatic malformation. Demographics, presentation, treatment, and outcome were reviewed. Results: 24 patients were identified, 62.5% were male. Median age at time of diagnosis was 2.5 years old, 33% were present at birth, 50% presented before 2 years old, and 29% presented ≥10 years of age. 75% were lymphatic (majority macrocystic), 20.8% were mixed lymphatic-venous malformations (LVM). 54% had other associated lymphatic or vascular malformations including 4 patients with PIK3CA-Related Overgrowth Spectrum (PROS), 3 with Generalized Lymphatic Anomaly, and 2 with Capillary Venous Lymphatic malformation (CVLM). Patients most often presented with abdominal pain (50%). 95.8% required an intervention. 87.5% were managed initially with sclerotherapy. Most interventions were performed for abdominal symptoms either before three years of age or during adolescence. 41.7% underwent laparoscopic or open surgery. 8.3% underwent surgery as their primary therapy. Conclusion: Intra-abdominal lymphatic malformations have a bimodal distribution pattern of presentation; shortly after birth and again at puberty. Most patients present with abdominal symptoms and have other associated vascular anomalies. MRI appears to be the most accurate modality for treatment planning. Interventional procedures such as sclerotherapy and medical therapy with sirolimus are safe and effective and should be considered first line therapies. Surgical resection is often required when the diagnosis is in question or when more conservative therapies are no longer efficacious.

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“…У черевній порожнині ЛМ локалізуються в брижі, сальнику, шлунковокишковому тракті та заочеревинному просторі. Кістозні ЛМ класифікуються як макрокістозні, мікрокістозні та змішані, що впливає на лікувальну тактику та результати лікування [3][4][5]. У черевній порожнині більшість ЛМ представлені макрокістозними та змішаними [3,10].…”

Section: оригінальні дослідження абдомінальна хірургіяunclassified

“…За іншими оцінками, частота ЛМ оцінюється в 1:2000 госпіталізацій до дитячих лікарень [10]. На інтраабдомінальні ЛМ припадає близько 5% ЛМ усіх локалізацій [4,11,16]. У нашому дослідженні ЛМ, що уражують кишечник та брижу, серед пацієнтів, що отримували лікування з приводу ЛМ різних локалізацій, становили 18/240 (7,5%).…”

Section: обговоренняunclassified

Intestinal and mesenterial cystic lymphatic malformations in children: clinical course features and treatment tactics

Diehtiarova¹,

Benzar²,

Rusyn³

et al. 2023

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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.

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Intra-abdominal lymphatic malformation management in light of the updated International Society for the Study of Vascular Anomalies classification

Cited by 12 publications

References 36 publications

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Intra-abdominal Lymphatic Malformations: A Case Series

Intestinal and mesenterial cystic lymphatic malformations in children: clinical course features and treatment tactics

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