|Year : 2018 | Volume
| Issue : 4 | Page : 147-149
Myxoid and pseudocystic degeneration of the umbilical cord
Nnaemeka Thaddeus Onyishi1, Paschalina Ezechukwu2, Samuel Robsam Ohayi1
1 Department of Histopathology, College of Medicine, Enugu State University of Science and Technology, Enugu, Nigeria
2 Department of Obstetrics and Gynaecology, Mother of Christ Specialist Hospital, Enugu, Nigeria
|Date of Web Publication||17-Jan-2019|
Dr. Nnaemeka Thaddeus Onyishi
Department of Histopathology, College of Medicine, Enugu State University of Science and Technology, Enugu
Source of Support: None, Conflict of Interest: None
Cystic, edematous, and myxoid lesions of the umbilical cord are rare conditions which have been sporadically reported in literature, most often detected at prenatal ultrasonography. Most reports of cystic cord conditions are ultrasonographic studies with a few featuring pathological descriptions. This is a report of a rare case of umbilical pseudocystic lesion with full pathological description. The possibility of this extremely myxoid lesion confounding the diagnosis of tumors such as angiomyxoma is highlighted.
Keywords: Cord edema, myxoid umbilical cord, umbilical cord cyst, umbilical cord pseudocyst
|How to cite this article:|
Onyishi NT, Ezechukwu P, Ohayi SR. Myxoid and pseudocystic degeneration of the umbilical cord. Libyan J Med Sci 2018;2:147-9
| Introduction|| |
The umbilical cord is an all-important lifeline connecting the fetus to the placenta and through which the fetus derives nourishment. A variety of abnormal cord conditions such as abnormal coiling, stricture, abnormal length, true knots, entanglements, prolapse, and velamentous insertion are known risk factors for fetal neurological injury, intrauterine growth retardation, and intrauterine fetal death., Cystic lesions of the umbilical cord are rare conditions which have been sporadically reported in literature, most often detected at prenatal ultrasonography. Most journal reports of cystic cord conditions are ultrasonographic studies with only a few featuring pathological descriptions. We report, with full pathological description, a rare case of umbilical cord cyst, discovered at prenatal ultrasonography and followed till delivery, in a 30-year-old woman. This, to the best of our knowledge, is the first reported case of umbilical cord myxoid pseudocyst from Sub-Saharan Africa.
| Case Report|| |
The mother, a 30-year-old G2P1 woman, had no significant medical or obstetric history. A prenatal ultrasonographic scan at 24-week gestational age (GA) revealed a singleton normal fetus with multiple anechoic umbilical cord lesions measuring 5.4 cm × 5.0 cm, 4.6 cm × 4.0 cm, and 3.6 cm × 2.9 cm. Color Doppler imaging confirmed normal flow through the umbilical vessels. Subsequent repeat scans at 29-week and 35-week GA established a progressive increase in the size of the cord lesions as well as good fetal physical condition. At 35 weeks, the conglomeration of cord lesion measured 11.5 cm × 9.67 cm × 7.2 cm and the largest cyst measured 7.0 cm × 5.12 cm. On account of the cord lesion, she underwent an elective cesarean section at 37-week GA and was delivered of a male baby with Apgar score of 10 at 1 min and 5 min. The baby had no physical deformities or abnormal facies and has remained normal 9 months postnatal.
Pathologic examination of the cord and placenta: Grossly, the umbilical cord measured 56 cm in length and was inserted paracentrally. The placental end of the cord was normal up to a length of 3 cm. The rest of the cord was edematous and enlarged with cystic area which ruptured during operative procedure. The average diameter of the cord was 4.5 cm (standard diameter of a cord ranges from 1.25 to 2.00 cm) [Figure 1]. Cut section of the enlarged portion exuded copious mucoid, slimy substance. Three vessels were identified within the cross-section of the cord.
|Figure 1: The gross appearance of the umbilical cord and the placenta (formalin fixed). The cord diameter is increased, and the cord is edematous in most of its length. Cut section of the cord exuded copious amount of mucoid, slimy fluid|
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The placenta was discoid in shape with unremarkable maternal and fetal surfaces [Figure 1]. The placenta weighed 540 g and measured 19 cm × 10 cm × 2.5 cm when trimmed of the membranes and cord. Cut surfaces showed beefy-red parenchyma with no gross abnormalities. The membranes were of normal color and transparency and were inserted at the margins.
Histologic sections of the umbilical cord showed extensive myxomatous degeneration of substance of the cord with formation of pseudocysts containing mucoid fluid [Figure 2] and [Figure 3]. The vessels of the cord (two arteries and one vein) were normal.
|Figure 2: Photomicrograph of the umbilical cord showing cystic degeneration of the cord. There was liquefaction of Wharton's jelly with dehisces and fragmentation of collagen fibril component of the cord substance forming pseudocystic spaces not lined by epithelial cells (H and E, ×100)|
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|Figure 3: Photomicrograph showing fragmentation of the substance of the cord (H and E, ×400)|
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Histologic sections of the placenta and the membranes were unremarkable. Focal areas of placental parenchymal calcifications in keeping with normal placental aging were present.
| Discussion|| |
Ours is a case of giant umbilical cord cystic lesion which came to attention at 24-week prenatal ultrasound scan. Pathologic study revealed degeneration and pseudocysts of the Wharton's jelly. The baby was physically normal, and the cause of the cord lesion was not found. Umbilical cystic lesions are detected at prenatal ultrasonography and have been the subject of a number of different of reports. These reports have varied in scope and focus and could broadly be categorized into case reports or small case series of cystic lesions seen at birth or detected earlier in pregnancy and followed to delivery;,,,,,,,,, retrospective reviews of sonographically detected umbilical cord cyst and assessment of pregnancy outcome;,, ultrasonographic screening of large numbers of pregnant women for umbilical cystic lesion;,, and matched cohort study comparing pregnancy outcomes in women with first trimester umbilical cord cyst and those without.
Studies show that the prevalence of umbilical cyst at prenatal ultrasonography ranges from 0.4 to 3.4%.,, Cysts which resolve spontaneously within the first trimester are not associated with adverse pregnancy outcome., Persistent second- and third-trimester umbilical cord cysts are associated with varying rates of chromosomal anomalies and/or fetal structural defects.,, In our case, the cyst was discovered at 24 weeks and persisted through pregnancy. We could not get a karyotype due to lack of facilities, but the baby, a male infant, had no features of congenital malformations at birth and has remained healthy 9 months postnatal.
Umbilical cord cysts have been categorized into epithelial-lined true cysts, which arise from remnant of allantois or omphalomesenteric duct, and pseudocysts which result from edema and degeneration of Wharton's jelly. The implication of this is that lesions with similar gross or sonographic cystic appearance may be pathologically different and may have diverse etiologies. In fact, umbilical cord cysts have been associated with various pathologic entities such as angiomyxoma, patent urachus, and abnormality of cord vessels.,,,,,, A thorough pathologic study and classification of umbilical cord cysts is warranted as it is possible that different etiologic types may correlate differently with pregnancy outcome or fetal condition. Unfortunately, but quite understandably, the methodology of many ultrasonographic studies of umbilical cord cysts does not include pathological examination.
The abundant myxoid appearance of the lesion we saw was most probably caused by degeneration and liquefaction of Wharton's jelly which is biochemically composed of the mucopolysaccharides chondroitin sulfate and hyaluronic acid. There was no angiomatous component or stromal proliferation to warrant the diagnosis of angiomyxoma or myxoma. Important to note here is the fact that degeneration and edema of Wharton's jelly could give rise to remarkably myxoid cord which could potentially lead to misclassification of a hemangioma of the cord as an angiomyxoma.
| Conclusion|| |
Umbilical cord cystic and edematous lesion is a very rare condition which can sometimes occur with no obvious associated fetal or maternal abnormality.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
The authors wish to acknowledge the assistance given by Mr. Ozioko Frank Ekene (BSc, MSc) in literature search and retrieval.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]