Prenatal growth characteristics of lymphatic malformations

Peranteau, William H.; Iyoob, Suzanne D.; Boelig, Matthew M.; Khalek, Nahla; Moldenhauer, Julie S.; Johnson, Mark P.; Hedrick, Holly L.; Flake, Alan W.; Coleman, Beverly G.; Adzick, N. Scott

doi:10.1016/j.jpedsurg.2016.10.025

Cited by 16 publications

(6 citation statements)

References 16 publications

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“…Prognosis of fetal lymphatic malformation has been related to lesion size, cystic and solid components, and presence of septations. 20,26,[28][29][30][31] In the 13 cases presented, we found no association whatsoever between location, lesion size or classification 20 and outcome. MRI was helpful in delivery planning in avoiding an unnecessary EXIT procedure, as well as for the differential diagnosis from other cystic lesions.…”

Section: Discussioncontrasting

confidence: 50%

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Prenatal ultrasound and magnetic resonance evaluation and fetal outcome in high‐risk fetal tumors: A retrospective single‐center cohort study over 20 years

Ulm

Muin

Scharrer

et al. 2020

Acta Obstet Gynecol Scand

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Section: Discussioncontrasting

confidence: 50%

Section: Discussionmentioning

confidence: 57%

Prenatal ultrasound and magnetic resonance evaluation and fetal outcome in high‐risk fetal tumors: A retrospective single‐center cohort study over 20 years

Ulm

Muin

Scharrer

et al. 2020

Acta Obstet Gynecol Scand

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“…Fetal ultrasonography and MRI are the imaging tools for the detailed prenatal diagnosis and anatomical evaluation of LMs [11,12]. LMs must be distinguished from increased nuchal translucency, which represents a frequent delay in lymphatic resorption in trisomy 21 and heart disease, etc.…”

Section: Antenatalmentioning

confidence: 99%

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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“…Thorough assessment of lesion growth and amniotic fluid volume by serial ultrasounds is essential to prenatal counselling and delivery planning. Peranteau et al describe a convenient approach for prediction of lesion growth based on the lesion volume ratio by serial US measurements between 19 and 39 weeks of gestation 76 . This helps to define which lesions may be more suitable for surgical excision or sclerotherapy, and which may be more difficult to manage.…”

Section: Vascular Malformations Spectrummentioning

confidence: 99%

Fetal brain, head, and neck tumors: Prenatal imaging and management

2020

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Fetal tumors represent an infrequent pathology when compared to congenital malformations, although their true incidence may be underestimated. A variety of benign and malignant neoplasms may occur anywhere in the neural axis. Imaging plays an important role in the fetal tumor diagnosis and evaluation of their resultant complications. Discovery of a fetal mass on obstetric ultrasound necessitates further evaluation with prenatal magnetic resonance imaging (MRI). New MR sequences and new applications of existing techniques have been successfully implemented in prenatal imaging. A detailed assessment may be performed using a variety of MR. Fetal tumors may be histologically benign or malignant, but their prognosis generally remains poor, especially for intracranial lesions. Unfavorable tumor location or heightened metabolic demands on a developing fetus may result in severe complications and a fatal outcome, even in cases of benign lesions. Nowadays, prenatal treatment focuses mainly on alleviation of secondary complications caused by the tumors. In this article we review congenital tumors of the brain, face, and neck encountered in prenatal life, and discuss diagnostic clues for appropriate diagnosis.

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Prenatal growth characteristics of lymphatic malformations

Cited by 16 publications

References 16 publications

Prenatal ultrasound and magnetic resonance evaluation and fetal outcome in high‐risk fetal tumors: A retrospective single‐center cohort study over 20 years

Prenatal ultrasound and magnetic resonance evaluation and fetal outcome in high‐risk fetal tumors: A retrospective single‐center cohort study over 20 years

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

Fetal brain, head, and neck tumors: Prenatal imaging and management

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