Abstract
Fetal mesentric lymphangioma, a congenital fetal abdominal cystic malformation has a rare occurrence. Antenatal detection, its differential diagnosis, prenatal management options and parental counseling regarding postnatal prognosis of such a case are thus rarely discussed. We report two cases of antenatally detected fetal cystic abdominal mass with a provisional diagnosis of abdominal lymphangioma. Postnatally one of the neonates developed features of intestinal obstruction and required surgical intervention. Intra-operative findings and histopathology report confirmed a mesentric lymphangioma. The other neonate had associated subcutaneous lymphangiectasia in left lumbar region, left sided inguinal hernia, undescended testes and was asymptomatic postnatally and managed conservatively.
Author Contributions
Copyright© 2021
Rohilla Minakshi, et al.
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Introduction
The detection rate of fetal abdominal cysts and other anomalies is increasing with wide spread use of antenatal ultrasonography. These cysts usually arise from gastrointestinal or genitourinary system and are more frequent and larger in size in a female fetus as compared to male fetus
Discussion
Fetal abdominal cystic lesions occur in isolation in around two-third of cases, and in other one-third there may be associated anomalies. Presence of associated anomalies, oligohydramnios or polyhydramnios, increased nuchal translucency in a first trimester scan, dysmorphic facies increase the probability of fetal aneuploidy. Fetal abdominal cystic masses usually arise from ovary or gastrointestinal tract. Cysts from gastrointestinal tract (GIT) may be omental or mesenteric in origin and or may arise consequent to intestinal obstruction, gastric duplication. The usual presentation of mesentric cyst is a single, multilocular cyst which may vary in size from less than a centimeter to a huge one. Symptoms vary depending upon the size of the cyst and the content within the cyst may be serous, chylous or hemorrhagic in nature. These should be differentiated from cysts arising from ovary and retroperitoneum.Massive cysts or those appear grave with other major congenital malformations and/or aneuploid fetuses, medical termination of pregnancy could be a reasonable approach, provided gestational age at diagnosis meets the period of legality for medical termination of pregnancy of the country. Surveillance for single, small, innocent cysts can be done by serial ultrasonography and the pattern of progression or regression can be noted. Management either in the prenatal or postnatal period can thus be planned accordingly. Case 1 presented as isolated non lethal cyst antepartum nevertheless need to be operated for intestinal obstruction in immediate neonatal period. Case 2 presented as multiseptated slowly growing fetal abdominal cyst with subcutaneous extension and did not require any surgical intervention till one year of followup. Lymphangioma is a hamartomatous malformation or developmental defect arising due to interruption in communication of embryonic lymph sacs with the venous channels. They have an incidence of 1 in 6000 live-births. These appear as unilocular or multilocular benign cystic mass in head and neck region with a potential to infiltrate into the surrounding viscera. Lymphangioma can be of three types: lymphangioma simplex, cavernous type, and cystic hygroma or lymphangioma. They can originate from any of the lymphatic channels; around 95% occur in the neck (usual site of representation for Cystic hygroma), head or axilla. Cystic hygroma is usually associated with Turners syndrome with XO fetal karyotype. Abdominal lymphangiomas usually arise from small bowel mesentry or from retroperitoneum and can extend upto lower limbs7,8. The size may be variable as a small encysted fluid collection to a complex, huge cyst Spontaneous regression is rare and mesenteric lymphangiomas depending on its location and locoregional spread usually require surgical intervention after birth.7,8 Conservative approach like cystic drainage is not recommended because of their tendency to recur irrespective of the modality chosen. Prognosis is good after surgical excision but follow-up is advised for possibility of recurrence. The postoperative morbidity for diffuse and multiple lesions is high as the procedure involves partial bowel obstruction and anastomoses. Case 1 also had resection of part of the jejunum along with removal of the cyst, and there is no recurrence at five years of follow up. Adjuvant therapy withbiological response modifier with antineoplastic effects like OK-432, are emerging as a potential therapy in preventing further enlargement of small localizedcysts Thus favourable prognostic factors for lymphangiomas are : a single isolated lesion with no extension to lower limbs or surrounding structures, quiescent or slow growth, first appeared in third trimester, not situated in posterior neck and normal karyotype. A multidisciplinary approach with thorough antenatal evaluation and parental counseling with neonatologists and pediatric surgeons is recommended to achieve optimum perinatal outcome.
1.Urogenital cystic lesions (40%)
Hydronephrosis
Secondary to Ureteropelvic Junction Obstruction may be associated with central nervous system , cardiovascular system , gastro-intestinal, skeletal malformations and aneuploidies, trisomy 21 commonly.
Bladder outflow obstruction secondary to posterior urethral valves-sporadic or familial disorder. Associated abnormalities may be tracheo-esophageal fistula, total anomalous pulmonary drainage, mitral stenosis, skeletal abnormalities, imperforate anus and trisomy 13,18.
Renal dysplasia
Multicystic dysplastic kidney may occur in association with many syndromes like Dandy-Walker and Apert's syndromeZellweger's, Meckel-Gruber and Jeune syndrome are associated with nonobstructive cystic dysplasia
Urachal cyst
Can be as an isolated anomaly or in association with prune-belly syndrome.
Ovarian cysts
Polyhydramnios is seen in 5-10%of patients. Complications include torsion, hemorrhage, necrosis, rupture and intestinal obstruction.
Hydrometrocolpos
Secondary to imperforate hymen or as a part of McKusick- Kaufman syndrome
2.Gastrointestinal cystic lesions (15%)
Duodenal atresia or stenosis
May have other intestinal atresias, congenital heart disease and the VACTERL association. About 30% of cases are associated with Down's syndrome.
Small bowel obstruction
Usually associated with polyhydramnios.Abnormal karyotype is rare.
Large bowel obstruction
Hirsch-prung disease- Down's syndrome is seen in around 5% with these.
Duplication cysts
May be associated with developmental disorders of spinal cord and blood vessels.
Mesenteric and Omental cysts
lymphatic hamartomas, described in detail in discussion.
3. Other Cystic mass.
Precise antenatal diagnosis of Cystic mass arising from hepato-biliary system, adrenal glands, pancreas, spleen other abdominal organs and retroperitoneum is challenging.