Intestinal lymphangiectasia in children

Isa, Hasan M; Al-Arayedh, Ghadeer G.; Mohamed, Afaf

doi:10.15537/smj.2016.2.13232

Cited by 15 publications

(29 citation statements)

References 9 publications

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“…The exclusion of long-chain fatty acids reduces lymphatic ow and pressure, and thus prevents the rupture of malformed lymphatics, while MCTs are directly absorbed into the portal venous circulation and bypass the enteric lymphatics [11]. Several case reports and articles have introduced the long-term effect of dietary therapy [12][13][14][15][16][17][18][19]. One patient in our study responded to dietary therapy (Table 1) and maintained clinical improvement for more than ve years.…”

Section: Discussionmentioning

confidence: 85%

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Systemic Review

Kwon

Kim

et al. 2020

Preprint

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Background: Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. We experienced seven patients with primary intestinal lymphangiectasia who showed clinical improvement with different treatments. Aim: We aimed to introduce the action mechanism of each drug and treatment overview in a single-center experience and a systemic review of second-line therapy for primary intestinal lymphangiectasia.Method: Children under 18 years old diagnosed with intestinal lymphangiectasia from June 2000 to June 2020 were included in the study. Capsule endoscopy, MR lymphangiography, or whole-body MRI for investigating the extent of abnormal lymphatic vessels in addition to endoscopy and biopsy were conducted. The individual treatment approaches depended upon the lymphangiectasis locations involved.Results: Only one patient showed a response to dietary therapy. One patient was successfully cured after two therapeutic lymphatic embolization. Octreotide was tried for two patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide was used for three months in one patient, and there was no effect in the other patient. Sirolimus was tried for four patients. Two of them had abnormal lymphatic lesions only in the intestine, and the others had extensive lymphangiectasis. The former group showed clinical improvement after 3 – 4 months of sirolimus treatment, whereas the latter group showed clinical improvement only after one month of sirolimus treatment.Conclusion: Intervention is a potential therapeutic option for patients with focal abnormal lymphatic lesions. Octreotide is not an optimal choice for patients with extensive lymphangiectasis. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis.

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Section: Discussionmentioning

confidence: 85%

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Systemic Review

Kwon

Kim

et al. 2020

Preprint

View full text Add to dashboard Cite

show abstract

“…The exclusion of longchain fatty acids reduces lymphatic ow and pressure, and thus prevents the rupture of malformed lymphatics, while MCTs are directly absorbed into the portal venous circulation and bypass the enteric lymphatics [11]. Several case reports and articles have introduced the long-term effect of dietary therapy [12][13][14][15][16][17][18][19]. One patient in our study responded to dietary therapy (Table 1) and maintained clinical improvement for more than ve years.…”

Section: Discussionmentioning

confidence: 85%

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Review of the Literature

Kwon

Kim

Choe

et al. 2020

Preprint

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Background: Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. We aimed to introduce the action mechanism of each drug and treatment overview in a single-center experience and a review of the literature on second-line therapy for primary intestinal lymphangiectasia.Method: Children under 18 years old diagnosed with intestinal lymphangiectasia from June 2000 to June 2020 were included and retrospectively reviewed in the study. Capsule endoscopy, MR lymphangiography, or whole-body MRI for investigating the extent of abnormal lymphatic vessels in addition to endoscopy and biopsy were conducted. The individual treatment approaches depended upon the lymphangiectasis locations involved.Results: Only one patient showed a response to dietary therapy. One patient was successfully cured after two therapeutic lymphatic embolization. Octreotide was tried for two patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide was used for three months in one patient, and there was no effect in the other patient. Sirolimus was tried for four patients. Two of them had abnormal lymphatic lesions only in the intestine, and the others had extensive lymphangiectasis. The former group showed clinical improvement after 3 – 4 months of sirolimus treatment, whereas the latter group showed clinical improvement only after one month of sirolimus treatment.Conclusion: Surgery or embolization is a potential therapeutic option for patients with focal abnormal lymphatic lesions. Octreotide is not an optimal choice for patients with extensive lymphangiectasis. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis.

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“…The treatment is based on a diet free of long-chain lipids, enriched with protein and medium-chain triglycerides, this diet may be effective [ 21 , 22 ]. However, sometimes it doesn't always give good results [ 23 ]; thence, the need in some refractory cases to use other treatments such as somatostatin analogue: octreotide.…”

Section: Discussionmentioning

confidence: 99%

Complicated primary intestinal lymphangiectasia (Waldmann's disease) in a child successfully treated with octreotide: A case report from a low-resource setting

Haddar

Sbia

Rkain

et al. 2021

Annals of Medicine &Amp; Surgery

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Introduction and importance The primary intestinal lymphangiectasia is a rare exudative enteropathy of unknown etiology that affects the lymphatic system. It causes lymphedema and malabsorption syndrome by the escape of the lymph and its elements into the intestinal lumen. Case presentation A female patient, diagnosed at the age of 11 with Waldmann's disease, has initially manifested chronic diarrhea with a stature-ponderal delay at the age of 6 months old; she was treated for a long time as celiac disease patient. Edematous syndrome, chronic diarrhea, staturo-ponderal delay and asymmetric lymphedema of the upper limb are the main clinical symptoms in this case. In addition, the exclusion of secondary intestinal lymphangiectasia was important for the diagnosis. Before and during her follow-up, the patient presented two complications of the disease: warts and osteomalacia. The patient did not respond to treatment with the low-fat diet; therefore, the need to add treatment with octreotide was necessary, which has given quite pleasant results. Octreotide was the therapeutic choice to treat the patient as she was resistant to the appropriate regimen with clinical improvement; nevertheless, certain biological elements of lymphatic leakage persisted. Discussion Waldmann's disease is rare. It can be responsible, besides the typical signs, for complications including warts and osteomalacia. The histopathological study of intestinal biopsies may be normal if lymphangiectasias are localized. The treatment is based on a nutritional diet associated with octreotide. During the patient's follow-up, the evolution after almost two years of the introduction of octreotide compared to the diet alone showed improved outcomes. Conclusion The treatment of Waldmann's disease is based on an adapted diet and octreotide. This case highlighted the importance of the long term follow-up in this disease.

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Intestinal lymphangiectasia in children

Cited by 15 publications

References 9 publications

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Systemic Review

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Systemic Review

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Review of the Literature

Complicated primary intestinal lymphangiectasia (Waldmann's disease) in a child successfully treated with octreotide: A case report from a low-resource setting

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