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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 2  |  Issue : 4  |  Page : 147-149

Myxoid and pseudocystic degeneration of the umbilical cord


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 Publication17-Jan-2019

Correspondence Address:
Dr. Nnaemeka Thaddeus Onyishi
Department of Histopathology, College of Medicine, Enugu State University of Science and Technology, Enugu
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/LJMS.LJMS_16_18

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  Abstract 


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

How to cite this URL:
Onyishi NT, Ezechukwu P, Ohayi SR. Myxoid and pseudocystic degeneration of the umbilical cord. Libyan J Med Sci [serial online] 2018 [cited 2019 Feb 19];2:147-9. Available from: http://www.ljmsonline.com/text.asp?2018/2/4/147/250299




  Introduction Top


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.[1],[2] 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 Top


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)[3] [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 Top


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;[4],[5],[6],[7],[8],[9],[10],[11],[12],[13] retrospective reviews of sonographically detected umbilical cord cyst and assessment of pregnancy outcome;[14],[15],[16] ultrasonographic screening of large numbers of pregnant women for umbilical cystic lesion;[17],[18],[19] and matched cohort study comparing pregnancy outcomes in women with first trimester umbilical cord cyst and those without.[20]

Studies show that the prevalence of umbilical cyst at prenatal ultrasonography ranges from 0.4 to 3.4%.[17],[18],[19] Cysts which resolve spontaneously within the first trimester are not associated with adverse pregnancy outcome.[7],[20] Persistent second- and third-trimester umbilical cord cysts are associated with varying rates of chromosomal anomalies and/or fetal structural defects.[5],[15],[17] 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.[17] 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.[4],[6],[8],[10],[11],[12],[21] 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 Top


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.



 
  References Top

1.
Baergen RN. Cord abnormalities, structural lesions, and cord “accidents”. Semin Diagn Pathol 2007;24:23-32.  Back to cited text no. 1
    
2.
Kraus D, Gersell F. Diseases of the placenta. In: Kurman RJ, Elleson HL, Ronnet BM, editors. Blaustein's Pathology of the Female Genital Tract. 6th ed. New York: Springer; 2011. p. 1000-69.  Back to cited text no. 2
    
3.
Silver RK, Dooley SL, Tamura RK, Depp R. Umbilical cord size and amniotic fluid volume in prolonged pregnancy. Am J Obstet Gynecol 1987;157:716-20.  Back to cited text no. 3
    
4.
Yavner DL, Redline RW. Angiomyxoma of the umbilical cord with massive cystic degeneration of Wharton's jelly. Arch Pathol Lab Med 1989;113:935-7.  Back to cited text no. 4
    
5.
Smith GN, Walker M, Johnston S, Ash K. The sonographic finding of persistent umbilical cord cystic masses is associated with lethal aneuploidy and/or congenital anomalies. Prenat Diagn 1996;16:1141-7.  Back to cited text no. 5
    
6.
Tennstedt C, Chaoui R, Bollmann R, Dietel M. Angiomyxoma of the umbilical cord in one twin with cystic degeneration of Wharton's jelly. A case report. Pathol Res Pract 1998;194:55-8.  Back to cited text no. 6
    
7.
Sepulveda W, Leible S, Ulloa A, Ivankovic M, Schnapp C. Clinical significance of first trimester umbilical cord cysts. J Ultrasound Med 1999;18:95-9.  Back to cited text no. 7
    
8.
Schiesser M, Lapaire O, Holzgreve W, Tercanli S. Umbilical cord edema associated with patent urachus. Ultrasound Obstet Gynecol 2003;22:646-7.  Back to cited text no. 8
    
9.
Kiran H, Kiran G, Kanber Y. Pseudocyst of the umbilical cord with mucoid degeneration of wharton's jelly. Eur J Obstet Gynecol Reprod Biol 2003;111:91-3.  Back to cited text no. 9
    
10.
Kilicdag EB, Kilicdag H, Bagis T, Tarim E, Yanik F. Large pseudocyst of the umbilical cord associated with patent urachus. J Obstet Gynaecol Res 2004;30:444-7.  Back to cited text no. 10
    
11.
Kita M, Kikuchi A, Miyashita S, Yoshizawa K, Takagi K, Ogiso Y. Umbilical cord cysts of allantoic and omphalomesenteric remnants with progressive umbilical cord edema: A case report. Fetal Diagn Ther 2009;25:250-4.  Back to cited text no. 11
    
12.
Haino K, Serikawa T, Itsukaichi M, Numata M, Kikuchi A, Kojima K, et al. Large pseudocyst of the umbilical cord detected in the second trimester. J Med Ultrason (2001) 2010;37:213-5.  Back to cited text no. 12
    
13.
Whipple NS, Bennett EE, Kaza E, O'Connor M. Umbilical cord pseudocyst in a newborn. J Pediatr 2016;177:333.  Back to cited text no. 13
    
14.
Shipp TD, Bromley B, Benacerraf BR. Sonographically detected abnormalities of the umbilical cord. Int J Gynaecol Obstet 1995;48:179-85.  Back to cited text no. 14
    
15.
Sepulveda W, Gutierrez J, Sanchez J, Be C, Schnapp C. Pseudocyst of the umbilical cord: Prenatal sonographic appearance and clinical significance. Obstet Gynecol 1999;93:377-81.  Back to cited text no. 15
    
16.
Zangen R, Boldes R, Yaffe H, Schwed P, Weiner Z. Umbilical cord cysts in the second and third trimesters: Significance and prenatal approach. Ultrasound Obstet Gynecol 2010;36:296-301.  Back to cited text no. 16
    
17.
Ross JA, Jurkovic D, Zosmer N, Jauniaux E, Hacket E, Nicolaides KH. Umbilical cord cysts in early pregnancy. Obstet Gynecol 1997;89:442-5.  Back to cited text no. 17
    
18.
Ghezzi F, Raio L, Di Naro E, Franchi M, Cromi A, Dürig P. Single and multiple umbilical cord cysts in early gestation: Two different entities. Ultrasound Obstet Gynecol 2003;21:215-9.  Back to cited text no. 18
    
19.
Skibo LK, Lyons EA, Levi CS. First-trimester umbilical cord cysts. Radiology 1992;182:719-22.  Back to cited text no. 19
    
20.
Hannaford K, Reeves S, Wegner E. Umbilical cord cysts in the first trimester: Are they associated with pregnancy complications? J Ultrasound Med 2013;32:801-6.  Back to cited text no. 20
    
21.
Brooks K, Guileyardo J, Le D, Joykutty J. Giant cystic umbilical cord associated with patent urachus and intrauterine fetal demise. Proc (Bayl Univ Med Cent) 2017;30:209-10.  Back to cited text no. 21
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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