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Malformations of the Lymphatic System
Published in Waldemar L. Olszewski, Lymph Stasis: Pathophysiology, Diagnosis and Treatment, 2019
Lymphangioma cavernosum, with its larger cavities, has a location deeper in the skin and subcutaneous tissue (see Figure 2). The skin-colored to reddish-bluish vascular tumors have a preferred location on the genitals and lower extremities, where they may become the starting point for elephantiasis. The less frequent localization on the lip and tongue (as also for L. circumscriptum) may lead to macrochilia and macroglossia and even to tracheal stenosis.3 The tongue is affected either on one side or in its anterior third. The cavernous lymphangioma may affect large areas of body and create a picture of elephantiasis.4 In the case of hematolymphangioma, this involves cavities connected with blood vessels, in addition to cystic dilatations of the lymph vessels. Congenital lymphangioma cysticum is usually a generalized malformation which starts from the submaxillary region, preferentially involving the throat, neck, and axilla, and may advance into one half of the face. A localization on the trunk or legs is less frequent. Cystic lymphangioma may protrude to the muscles.6 In addition to a dominant tumor, many smaller lymph ectasias, down to the “lymph-vessel varices”, are found.
Individual conditions grouped according to the international nosology and classification of genetic skeletal disorders*
Published in Christine M Hall, Amaka C Offiah, Francesca Forzano, Mario Lituania, Michelle Fink, Deborah Krakow, Fetal and Perinatal Skeletal Dysplasias, 2012
Christine M Hall, Amaka C Offiah, Francesca Forzano, Mario Lituania, Michelle Fink, Deborah Krakow
Prenatal US features: specific antenatal diagnosis has not been made. However, a case has been reported in which an abdominal cystic lymphangioma was identified at 17 weeks, diagnosed at 26 weeks. Autopsy at 28 weeks confirmed the diagnosis of Proteus syndrome with more typical features (including hemihypertrophy, macrodactyly of one toe and hemimegalencephaly) identified. The mass was confirmed to be a cystic lymphangioma. The authors suggest that the antenatal identification of an unusually sited cystic mass should prompt careful examination for other features of Proteus syndrome.
Prenatal diagnosis of cystic hygroma cases in a tertiary centre and retrospective analysis of pregnancy results
Published in Journal of Obstetrics and Gynaecology, 2022
Sureyya Saridas Demir, Erkan Cagliyan, Derya Öztürk, Samican Özmen, Sabahattin Altunyurt, Tufan Çankaya, Elcin Bora
‘Congenital anomalies’ are the group of diseases shown as the second most important cause of perinatal morbidity and mortality after preterm labour (Bennasar et al. 2018; Sak et al. 2019; Pan et al. 2020). Foetal structural anomalies are observed in approximately 3–5% of all pregnancies (Kınay et al. 2016). ‘Cystic hygroma’, also called cystic lymphangioma, is a benign type of congenital malformation which is seen in 75–90% neck (especially in posterior cervical triangle), 20% in axilla, 5% in mediastinum and retroperitoneal region, rarely in thoracic wall, the frequency of which is reported as one in 6000 live births and one in 750 spontaneous abortions (Kadam et al. 2017; Koç 2020; Canbey Goret 2018; Saghir et al. 2020).
Enterogenous cyst of the small bowel mesentery: a case report and review of the literature
Published in Acta Chirurgica Belgica, 2019
Donatas Danys, Raminta Martinaitytė, Jolanta Zacharic, Eligijus Poskus, Edvardas Zurauskas, Tomas Poskus, Kęstutis Strupas
Ultrasound showed a thin-walled cyst with clear filling in right inguinal region. Based on radiological findings, retroperitoneal cystic lymphangioma was diagnosed and the patient underwent surgery. During laparotomy, 30 cm from duodenojejunal fold, 7 × 7 × 5 cm cystic mass was identified on mesentery and excised. It occurred to be 170 g cystic mass filled with white lymph (Figures (2–4)).
Giant idiopathic benign retroperitoneal cyst: a case report
Published in Acta Chirurgica Belgica, 2021
W. Maenhoudt, S. Hoflack, J. Bontinck, J. Beaurang, K. Charels, H. Bontinck
In this report we discuss a case of a giant cyst originating in the retroperitoneal space outside the major organs. Such “primary retroperitoneal cysts” are uncommon lesions which are usually confined to the retroperitoneal space and typically show no connection with surrounding anatomical structures [1–3]. Most of these cysts are small and incidentally encountered. Such giant dimensions as in our case are very rare [3]. The estimated incidence of primary retroperitoneal cysts lies somewhere in between 1/5750 and 1/250,000 [1,2,4,5]. They can originate from cell inclusions, embryonal-urogenital remnants, retroperitoneal lymphatics or they develop post-traumatic or after a parasitic infection. They can be further classified based on their specific origin [1–3]. Urogenital cysts are thin-walled with an unilocular translucent appearance and are further subdivided into pronephric, mesonephric and Mullerian subtypes. They develop out of remnants of the embryonic urogenital tract [3]. Mullerian cysts are generally more frequent, especially in obese females from 20 to 50 years with menstrual irregularities [6,7]. Lymphatic cysts consist out of two subtypes. Chylous cysts arise from intestinal lymphatics. The other subtype of lymphatic cysts such as lymphangiomas for instance forms out of retroperitoneal lymphatics and are not in continuity with the intestine [8]. Urogenital cysts always contain a clear serous fluid while lymphatic cysts are sometimes filled with a more milky or fatty substance. They are lined with a flattened endothelium and can either be unilocular or multilocular [7]. Cystic lymphangioma can grow to large volumes and tend to cross from one retroperitoneal compartment to an adjacent one [1,2]. Mesocolic cysts are exclusively found below the transverse mesocolon between the vertical segments of the colon and have a fibrous wall. Dermoid cysts can form out of cell inclusions in the retroperitoneal space. They are slow growing structures characterised by a thick cyst wall and contain sebaceous material and mature tissue such as hair [1,2]. A posttraumatic haematoma or parasitic infection can also lead to the cyst formation in the retroperitoneum. When it is not possible to determine the origin of the primary retroperitoneal cyst, diagnosis of an idiopathic retroperitoneal cyst is rendered.