Pediatric pulmonary lymphatic flow Disorders: Diagnosis and management

Biko, David M.; Dori, Yoav; Savoca, Melanie; Krishnamurthy, G.; Smith, Christopher L.; Laje, Pablo; Rome, Jonathan J.; Escobar, Fernando

doi:10.1016/j.prrv.2019.11.002

Cited by 19 publications

(19 citation statements)

References 27 publications

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“…Brogan et al [ 23 ] proposed the division of patients with PB into three groups, depending on basic disease, i.e., CHD, asthma, or allergy, and finally those without these medical burdens. One hypothesis assumes that an increase in the venous pressure has considerable impact on lymph circulation disorders resulting in significant distension of vessel (lymphangiectasia) or their excessive proliferation (lymphangiomatosis) [ 34 ]. On the one hand, lymphangiectasia is a congenital pathology, which frequently occurs in some clinical syndromes (i.e., Down, Noonan, and Turner syndrome).…”

Section: Discussionmentioning

confidence: 99%

“…The complex of these disorders leads to an improper lymphatic flow through the lymphatic vessels of a respiratory system. Imaging studies such as lymphoscintigraphy, dynamic contrast MR lymphangiography (DCMRL), or conventional lymphangiography have confirmed the relationship between lymphatic disorders and PB in patients after Fontan procedure [ 25 , 26 , 34 ]. One of the treatments of PB include percutaneous lymphatic interventions such as embolization of the thoracic duct [ 6 , 34 ], a surgical intervention for reducing the central venous pressure, and pharmacological management.…”

Section: Discussionmentioning

confidence: 99%

“…Imaging studies such as lymphoscintigraphy, dynamic contrast MR lymphangiography (DCMRL), or conventional lymphangiography have confirmed the relationship between lymphatic disorders and PB in patients after Fontan procedure [ 25 , 26 , 34 ]. One of the treatments of PB include percutaneous lymphatic interventions such as embolization of the thoracic duct [ 6 , 34 ], a surgical intervention for reducing the central venous pressure, and pharmacological management. A diet based on minimizing long-chain triglycerides intake and supplementation of medium chain triglycerides (MCT) associated with lymphatic interventions may enable a reduction in a cast production or other complication of Fontan procedure such as thromboembolism [ 6 , 34 ].…”

Section: Discussionmentioning

confidence: 99%

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Plastic Bronchitis—A Serious Rare Complication Affecting Children Only after Fontan Procedure?

Pałyga-Bysiecka

Polewczyk

Kołodziej

et al. 2021

JCM

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Background: Plastic bronchitis (PB) may occur not only in children following palliative Fontan procedure but also in those without underlying heart disease. We aim to assess the clinical course, therapeutic measures, outcome, and follow-up of PB in children with congenital heart disease (CHD) and children without cardiac problems. Methods: This retrospective case series assessed children with PB admitted to hospital between 2015 and 2019. Parents or guardians of patients were contacted by e-mail or telephone between September 2017 and June 2019 to enquiry about recurrence of PB and strategy of treatment. The diagnosis of PB was based on the expectoration (spontaneous or during bronchoscopy) of endobronchial plugs. Results: This study delineated the clinical, histological, and laboratory features of plastic bronchitis in children following Fontan procedure (Group A) and in those without heart defects (Group B, non-CHD children). The main symptoms were cough accompanied by dyspnea, and hypoxemia with a decrease in oxygen saturation, often leading to acute respiratory failure. In children with CHD, the first episode of PB occurred at a relatively young age. Although chronic, i.e., lasting more than 3 weeks, inhaled therapy was implemented in both groups of patients, the recurrences of PB were observed. The mean time to PB recurrence after the first episode in Group A was longer than that in Group B (1.47 vs. 0.265 years, p = 0.2035). There was no re-episode with recurrence of PB in 3 cases out of 10 in total in Group A (30%) and 1 case out of 4 in total in Group B (25%). While the majority of children in Group A usually developed bronchial casts on the right side, the patients in Group B (without CHD) suffered from bronchial casts located only on the left side. Conclusions: Despite many similarities, clinical, histological, and laboratory studies in the children with plastic bronchitis after Fontan’s surgery and in children without heart defects suggest that there are differences in the course of the disease in patients without CHD, such as a more advanced age of the first episode of PB, the location of plastic casts on the left side, and a stronger role of inflammatory factors and mechanisms. Further research is needed to understand the pathophysiology of PB and choose the most appropriate therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Plastic Bronchitis—A Serious Rare Complication Affecting Children Only after Fontan Procedure?

Pałyga-Bysiecka

Polewczyk

Kołodziej

et al. 2021

JCM

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“…Nutritional management: Patients with high output lymphatic leak are at risk for severe nutritional deficiencies including vitamin D 25-hydroxy, zinc, copper and essential fatty acids. [36] While NPO, TPN with intralipids is necessary to prevent essential fatty acid deficiency.…”

Section: Management Of Refractory Lymphatic Leakmentioning

confidence: 99%

How We Approach: Pediatric Congenital Chylous Effusions and Ascites

Nakano¹,

Dori²,

Gumer³

et al. 2021

Preprint

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Congenital lymphatic leak may develop in patients with maldeveloped lymphatics and result in life-threatening fluid and electrolyte imbalance, protein deficiency and immunodeficiency. Rapid diagnosis and therapy are necessary to prevent these complications; however, the field lacks clinical trials to support standardized diagnostic treatment guidelines. We present our current multidisciplinary approach to the diagnosis and management of congenital lymphatic leak including chylous pleural effusion and ascites. Depending on the rate of lymphatic leak, therapy can range from observation with nutritional modifications to surgical and interventional procedures aimed to reduce lymphatic drainage. Modalities to image central and peripheral lymphatics have advanced considerably. Genetic variants and subsequent targets that drive lymphatic maldevelopment have expanded the repertoire of possible pharmacotherapeutic options.

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“…Currently it is still used in lymphangiography and in embolization procedures such as post-operative lymphatic leakages. Lipiodol remains for several hours and days in the lymphatic system, allowing localization of downstream nodes; this feature is used in recent techniques such as direct transabdominal puncture of the cysterna chyli for transabdominal access to the thoracic duct (4). An additional property is its sclerosing effect at the leakage site where lymph leaves the lymphatic vessels (5); as such it has been used therapeutically with variable effect in case of chylothorax, chyloperitoneum, protein losing enteropathy, plastic bronchitis, and other lymphatic diseases.…”

Section: Introductionmentioning

confidence: 99%

Case Report: Disseminated Systemic Embolism of Lipiodol After Lymphography for Plastic Bronchitis After Fontan Repair

Hubrechts¹,

Wåhlander

Olofsson

et al. 2020

Front. Pediatr.

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Lipiodol-based lymphangiography is not only a diagnostic tool for visualization of lymphatic disorders such as plastic bronchitis (PB), but also aims a therapeutic effect by embolizing lymph leakages. We performed such percutaneous lymphatic embolization for PB in a Fontan patient with proven absence of right-to-left shunt, and demonstrated important lymphatic abnormalities in the mediastinum. Shortly after the procedure, the patient developed severe convulsive seizures, revealing multiple cerebral embolisms of Lipiodol. Radiological images were impressive, yet the clinical neurological outcome was favorable. Lipiodol-based lymphography in Fontan patients with plastic bronchitis should be avoided as this subgroup is more likely to have developed lympho-pulmonary venous connections which allow systemic emboli.

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Pediatric pulmonary lymphatic flow Disorders: Diagnosis and management

Cited by 19 publications

References 27 publications

Plastic Bronchitis—A Serious Rare Complication Affecting Children Only after Fontan Procedure?

Plastic Bronchitis—A Serious Rare Complication Affecting Children Only after Fontan Procedure?

How We Approach: Pediatric Congenital Chylous Effusions and Ascites

Case Report: Disseminated Systemic Embolism of Lipiodol After Lymphography for Plastic Bronchitis After Fontan Repair

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