Long-term outcomes of lymphatic malformations in children: An 11-year experience from a tertiary referral center

Hyvönen, Hanna; Salminen, Päivi; Kyrklund, Kristiina

doi:10.1016/j.jpedsurg.2022.07.024

Cited by 6 publications

(5 citation statements)

References 30 publications

(16 reference statements)

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“…Studies on treatment outcomes of sclerotherapy/embolization often focus on a specific CVM subtype, a specific anatomical location, or a specific agent, and the definition of a successful treatment outcome varies, all of which limits comparability. In recent studies on pediatric patients, a complete response was reported in 11-89% of the patients and improvement in 57-100% [34,48,49,51,54,69,70]. In LMs, bleomycin seems to be the most effective agent, and macrocystic lesions often showed a better response than microcystic and/or mixed lesions [49,53,70].…”

Section: Sclerotherapy/embolizationmentioning

confidence: 97%

“…In recent studies on pediatric patients, a complete response was reported in 11-89% of the patients and improvement in 57-100% [34,48,49,51,54,69,70]. In LMs, bleomycin seems to be the most effective agent, and macrocystic lesions often showed a better response than microcystic and/or mixed lesions [49,53,70]. In AVMs, the success rate of embolization was influenced by the angioarchitecture of the nidus [54].…”

Section: Sclerotherapy/embolizationmentioning

confidence: 99%

“…In AVMs, the success rate of embolization was influenced by the angioarchitecture of the nidus [54]. Complication rates varied from 6-25% on the procedural level and from 10-54% on the patient level in recent studies [34,48,49,51,54,69,70], with historical data reporting complication rates up to 82% on the procedural level and 72% on the patient level [50,59,71]. Complications frequently reported include skin necrosis, infections, and (mostly temporary) nerve complications.…”

Section: Sclerotherapy/embolizationmentioning

confidence: 99%

See 2 more Smart Citations

Congenital Vascular Malformations in Children: From Historical Perspective to a Multidisciplinary Approach in the Modern Era—A Comprehensive Review

Bouwman,

Verhoeven,

Klein

et al. 2024

Children

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Congenital vascular malformations (CVMs) are the result of an aberrant development during embryogenesis. Although these lesions are present at birth, they are not always visible yet. Once symptomatic, patients suffer from pain, bleeding, ulcers, infections or lymphatic leakage, depending on the subtype of vessels involved. Treatment includes conservative management, surgery, sclerotherapy, embolization and pharmacological therapy. The clinical presentation varies widely and treatment can be challenging due to the rarity of the disease and potential difficulties of treatment. This review gives an overview of the historical developments in diagnosis and classification and exposes the key elements of innovations in the past decades on the identification of genetic mutations and personalized treatment. These advances in the field and a multidisciplinary approach are highly valuable in the optimization of clinical care aimed at both curing or stabilizing the CVM and pursuing physical and psychosocial wellbeing.

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Section: Sclerotherapy/embolizationmentioning

confidence: 97%

Section: Sclerotherapy/embolizationmentioning

confidence: 99%

Section: Sclerotherapy/embolizationmentioning

confidence: 99%

See 1 more Smart Citation

Congenital Vascular Malformations in Children: From Historical Perspective to a Multidisciplinary Approach in the Modern Era—A Comprehensive Review

Bouwman,

Verhoeven,

Klein

et al. 2024

Children

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“…This spontaneous regression would be more frequent for cervical localizations of LMs and would occur in just over 10% of cases and 2 to 24 months after the episode [16,17]. Spontaneous regression is possible in up to 16% of cases [18]. Intracystic hemorrhage is also possible: the swelling then becomes suddenly tense, painful, and potentially bluish but without fever or inflammatory signs.…”

Section: Specificities Of Macrocystsmentioning

confidence: 98%

French national diagnosis and care protocol (PNDS, protocole national de diagnostic et de soins): cystic lymphatic malformations

Leboulanger

Bisdorff

Boccara

et al. 2023

Orphanet J Rare Dis

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Cystic lymphatic malformations (LMs) are rare chronic conditions which management differs according to the type (macrocystic LMs, microcystic LMs or both). Studies are lacking due to rarity of the pathology. We aimed to establish a French National Diagnosis and Care Protocol (PNDS: Protocole National de Diagnostic et de Soins), to provide health professionals with free open access synthesis on optimal management and care of patients with LMs (https://www.has-sante.fr/upload/docs/application/pdf/2021-03/malformations_lymphatiques_kystiques_-_pnds.pdf). The process included a critical review of the literature and multidisciplinary expert consensus. LMs are congenital but are not always discovered at birth. Nearly 75% of them are located in the head and neck because of the highly dense lymphatic system in this region. Physical examination (showing painless masses with normal skin color and depressible consistency, or cutaneous/mucosal lymphangiectasia) and color Doppler ultrasonography, usually allow for diagnosis. MRI (involving T2 sequences with fat saturation in at least two spatial planes) is the tool of choice for evaluating anatomical extension, characterizing lesions (microcystic and macrocystic), and before considering therapeutic management. A biopsy, coupled to a blood sample, can also be used for molecular biology analyses, to search for activating mutations of the PIK3CA gene, particularly with LM integrating in a syndromic form (CLOVES or Klippel-Trenaunay syndrome) but also in certain isolated (or common) LMs. The spontaneous evolution of LMs, in particular microcystic forms, is often toward progressive aggravation, with an increase in the number of vesicles, thickening, increased oozing and bleeding, while pure macrocystic LMs may regress due to “natural sclerosis”, i.e. fibrosis secondary to an inflammatory reorganization after common infantile infections. In case of voluminous LMs or syndromic forms, functional and psychological repercussions can be major, deteriorating the patient’s quality of life. LMs must be treated by physicians integrated in multidisciplinary teams, and be personalized. Management is a life-long process that involves one or several of these therapies: conservative management, physical therapy (compression), sclerotherapy, surgery, drugs such as mTOR inhibitors (sirolimus), that has shown efficacy in decreasing the volume of LMs, and, more recently, PI3K-inhibitors in syndromic forms. Psychological and social support is necessary, taking into account the patient and his family.

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“…Surgery, oral medications, and sclerotherapy are the three main treatment methods, each with its limitations and advantages. Therefore, treatment options should be individualized for pediatric patients with LMs ( 4 ). Currently, the treatment of retroperitoneal LMs remains challenging due to their rarity.…”

Section: Introductionmentioning

confidence: 99%

Laparoscopic-assisted sclerotherapy in pediatric retroperitoneal lymphatic malformations

Shi,

Lv,

et al. 2024

Front. Pediatr.

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BackgroundRetroperitoneal lymphatic malformations (LMs) are rare. Currently, the treatment of retroperitoneal LMs remains challenging. This study aimed to examine the safety and efficacy of laparoscopic-assisted sclerotherapy for retroperitoneal LMs in pediatric patients.MethodsWe retrospectively reviewed patients treated with laparoscopic-assisted sclerotherapy for retroperitoneal LMs in a single tertiary medical center between July 2020 and February 2023. Doxycycline was prepared into a solution with a concentration of 10 mg/ml for use in sclerotherapy. Demographic data, clinical features, details of management, and outcomes were collected and analyzed.ResultsA total of six patients, comprising three males and three females, were identified. The LMs were categorized into four macrocystic and two mixed-cystic types. The mean age and weight were 52.2 months (range, 11–108 months) and 20 kg (range, 12.5–27.5 kg), respectively. Three patients presented with abdominal pain or distension, while the other three patients were asymptomatic. All six patients underwent a total of eight sclerotherapy sessions. Two patients experienced intra-cystic hemorrhage and required a second sclerotherapy session. Only one patient presented with vomiting after sclerotherapy, which resolved spontaneously. Five patients met the complete response criteria, and one patient met the effective criteria. The mean reduction in lesion size was 92.3% (range, 69.9%–99.6%). No further complications or recurrence were recorded during follow-up.ConclusionLaparoscopic-assisted sclerotherapy is a safe and effective approach for treating retroperitoneal LMs. This technique is applicable for both macrocystic and mixed-cystic retroperitoneal LMs.

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Long-term outcomes of lymphatic malformations in children: An 11-year experience from a tertiary referral center

Cited by 6 publications

References 30 publications

Congenital Vascular Malformations in Children: From Historical Perspective to a Multidisciplinary Approach in the Modern Era—A Comprehensive Review

Congenital Vascular Malformations in Children: From Historical Perspective to a Multidisciplinary Approach in the Modern Era—A Comprehensive Review

French national diagnosis and care protocol (PNDS, protocole national de diagnostic et de soins): cystic lymphatic malformations

Laparoscopic-assisted sclerotherapy in pediatric retroperitoneal lymphatic malformations

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