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CASE REPORT
Year : 2020  |  Volume : 13  |  Issue : 3  |  Page : 285-287  

Congenital hernia of umbilical cord masquerading as umbilical cyst and omphalocele on antenatal scans


1 Department of Neonatology, KK Women's and Children's Hospital; Department of Paediatrics, Duke NUS Medical School, Yong Loo Lin School of Medicine, Lee Kong Chian School, of Medicine, Singapore
2 Department of Paediatric Surgery, Monash Children's Hospital, Melbourne, Australia
3 Department for Neonatology, Monash Children's Hospital; Department of Paediatrics, School of Clinical Sciences, Nursing and Health Sciences, Monash University, Melbourne, Australia
4 Department of Paediatric Surgery, Columbia Asia Referral Hospital, Bengaluru, Karnataka, India

Date of Submission26-Mar-2019
Date of Decision03-Sep-2019
Date of Acceptance03-Oct-2019
Date of Web Publication3-Jun-2020

Correspondence Address:
Krishna Revanna Gopagondanahalli
Department of Neonatology, KK Women's and Children's Hospital, 100 Bukit Timah Road
Singapore
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_91_19

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  Abstract 


Congenital hernia of the umbilical cord (CHUC) is a rare, distinctive anomaly with variable presentation and conflicting terminology. We report two newborn infants who had fetal antenatal diagnosis as umbilical cord cyst and omphalocele, respectively, and were subsequently found to have hernia into the umbilical cord. The herniating mass in CHUC may vary greatly in size, making it difficult to differentiate with other conditions such as congenital umbilical cord cyst, cord hematoma, omphalocele, and gastroschisis. CHUC is prone to inadvertent cord clamping and injury to the gut. The failure of retraction of intestinal loops following physiological herniation has been postulated as the embryological basis of this entity. Overall prognosis is good.

Keywords: Congenital hernia, cord hematoma, omphalocele, umbilical cord, umbilical cysts


How to cite this article:
Gopagondanahalli KR, Chang A, Nataraja R, Tan K, Chandran S, Saiprasad T R. Congenital hernia of umbilical cord masquerading as umbilical cyst and omphalocele on antenatal scans. Med J DY Patil Vidyapeeth 2020;13:285-7

How to cite this URL:
Gopagondanahalli KR, Chang A, Nataraja R, Tan K, Chandran S, Saiprasad T R. Congenital hernia of umbilical cord masquerading as umbilical cyst and omphalocele on antenatal scans. Med J DY Patil Vidyapeeth [serial online] 2020 [cited 2020 Dec 5];13:285-7. Available from: https://www.mjdrdypv.org/text.asp?2020/13/3/285/285773




  Introduction Top


Congenital hernia of the umbilical cord (CHUC) results from a failure of return of intestine loops following the physiological gut herniation around 10–12 weeks of gestation.[1] It is a well-recognized entity, though it can be deceptive and baffling for other entities such as umbilical cysts, hematoma, and large omphaloceles. The complications associated with CHUC include intestinal malrotation, atresia, obstruction, and trauma to the intestines due to accidental clamping of umbilical cord owing to its uncharacteristic appearance. We report two cases of congenital umbilical cord hernia in newborn infants to highlight the varied spectrum of presentation and characteristic features which differentiate from other anterior abdominal defects.


  Case Reports Top


Case 1

A routine antenatal scan in a primigravida mother noted a small cyst at the level of umbilicus. No other fetal anomaly was noted. The pregnancy was uneventful with no complications. A healthy baby boy was born at term with Apgar scores of 9 at 5 min of life, with a birth weight of 3831 g. The apparently normal-looking umbilical cord was clamped and cut as routine. On postnatal screening examination, a small nonreducible mass was noted at the base of the umbilical cord [Figure 1]. The remainder of the clinical examination was unremarkable. On surgical exploration, a 2-cm diameter umbilical fascial defect with herniating intestine and patent vitellointestinal duct was visualized. Herniating intestine was reduced, and patent vitellointestinal duct was ligated due to narrow base. The umbilical fascial defect was closed transversely. A purse-string closure was performed at skin level. Screening echocardiogram and renal and cranial ultrasounds (US) were normal.
Figure 1: Nonreducible mass at the base of umbilical cord (arrow)

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Case 2

A second-trimester US scan in a primigravida mother revealed a large herniating mass of the intestine at the umbilicus. The rest of the morphology scan was unremarkable. A diagnosis of omphalocele was made, and the mother was referred to tertiary medical center for further management. A healthy male baby was born by vaginal route at 38 weeks of gestation with good Apgar scores. Birth weight was 2320 g. Examination revealed herniating intestinal loops into the umbilical cord through the umbilical ring [Figure 2]. There were no dysmorphic features, and rest of the clinical examination was normal. The screening echocardiogram and renal and head US were normal. The baby underwent surgical exploration of the sac with release of intestinal adhesions, reduction of intestinal loops, and an anatomical closure of umbilical ring. The skin was closed with purse-string suture.
Figure 2: Herniating intestinal loops into the umbilical cord

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In both the above cases, the normal insertion of the cord was noted at the time of surgical exploration. Both babies had uneventful postoperative recovery and doing well on follow-up.


  Discussion Top


The umbilicus is considered as “mirror of abdomen,” which embryologically represents a bottle neck passage for vessels and structures related to the developing digestive and urinary systems. During the 6th week of fetal life, the intestine herniates in the umbilical cord. The enlargement of the abdominal cavity of the fetus retracts the herniated intestinal loops by 10th–12th weeks of gestation. The failure of return of intestines has been postulated as the cause of congenital umbilical cord hernia, but the exact reason behind the failure of retraction of intestines is still unknown. One of the probable mechanisms could be due to failure of the normal development of the umbilical ring, resulting in the persistence of the connection between the fetal peritoneal cavity and the extraembryonic mesoderm.[1] The free-floating bowel loops into the amniotic fluid are prone for volvulus, leading to vascular compromise resulting in intestinal atresia and perforation, and in extreme cases, the fetal demise has been reported with rupture of the umbilical cord.[2],[3]

Congenital umbilical cord hernia vary greatly in size ranging from few millimeters to large masses measuring several centimeters, making this entity either completely missed due to its unimpressive size or misrepresented as large omphaloceles. Hegazy classified the omphaloceles as a type of congenital umbilical hernia. Such cases are differentiated from that of gastroschisis, in which there is associated defect in the anterior abdominal wall.[4],[5]

Mirza and Saleem classified the CHUC into four types: Type 1 is simple hernia into cord without any associated complications; Type 2- associated with intestinal obstruction Type 3, a distinct variety associated with mucosal prolapse and Type- 4 is associated with evisceration.[6] The reported associated anomalies with CHUC are persistent vitellointestinal duct, Meckel's diverticulum, persistent cloaca, bowel atresia, stenosis, and short bowel syndrome.[3],[6],[7],[8],[9] Other uncommon associations are congenital heart disease, cleft lip, palate, and glaucoma.[10],[11],[12] There are no published data on underlying genetic disorder accompanying cord hernia to date.

CHUC can be reliably detected by high-resolution transvaginal fetal ultrasonography as early as the 13–14 weeks of gestation, though the diagnosis of CHUC on antenatal scan is operator dependent. One should be aware of the lesions which can disguise as CHUC such as abdominal wall defects (omphalocele and gastroschisis), cord hematoma, cysts, and teratoma. A normal insertion of cord on US is a key distinguishing feature from other fetal ventral abdominal wall defects.[11] There is a great utility of three-dimensional US imaging and MRI in better delineation of anatomy such as covering membrane (amnion in omphalocele vs. skin in cord hernia), cord insertion in relation to defect, and most importantly, evaluation of associated malformations in ambiguous cases of fetal ventral defects.[13],[14]

A distinctive skin covering extending from the abdominal wall up to the neck of the sac has been reported as a characteristic of CHUC which was seen in one of our babies.[7],[15]

Surgical exploration is usually recommended for large herniating masses to rule out associated intestinal anomalies such as atresia, malrotation, and adhesions. The small hernias can be manually reduced. The conventional technique involves excision of the amniotic sac, ligation of the umbilical vessels, fascial closure, and reconstruction of the skin with a subcuticular purse-string suture. The surgical techniques have evolved in the recent times with emphasis on preserving umbilical elements and better cosmetic outcome.[16]

Prognosis is excellent following surgery with no significant associated anomalies.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hegazy AA. Anatomy and embryology of umbilicus in newborns: A review and clinical correlations. Front Med 2016;10:271-7.  Back to cited text no. 1
    
2.
Haas J, Achiron R, Barzilay E, Yinon Y, Bilik R, Gilboa Y, et al. Umbilical cord hernias: Prenatal diagnosis and natural history. J Ultrasound Med 2011;30:1629-32.  Back to cited text no. 2
    
3.
Mirza B, Saleem M. Hernia of umbilical cord with congenital short gut. J Neonatal Surg 2014;3:26.  Back to cited text no. 3
    
4.
Gajdhar M, Kundal VK, Mathur P, Gajdhar M. Pitfalls in the umbilical pit: Giant hernia of the umbilical cord. BMJ Case Rep 2013;2013. pii: bcr2013009381.  Back to cited text no. 4
    
5.
Hegazy AA. Clinical Embryology for Medical Students and Postgraduate Doctors. Berlin: Lap Lambert Academic Publishing; 2014.  Back to cited text no. 5
    
6.
Mirza B, Ali W. Distinct presentations of hernia of umbilical cord. J Neonatal Surg 2016;5:53.  Back to cited text no. 6
    
7.
Mirza B, Mirza A, Hashim I, Saleem M. Hernia of umbilical cord: Report of three unusual cases. J Neonatal Surg 2015;4:16.  Back to cited text no. 7
    
8.
Gys B, Demaeght D, Hubens G, Ruppert M, Vaneerdeweg W. Herniation of a Meckel's diverticulum in the umbilical cord. J Neonatal Surg 2014;3:52.  Back to cited text no. 8
    
9.
Burns CW, Ogryzlo MA. Congenital hernia into the umbilical cord; two cases, one associated with persistent cloaca. Can Med Assoc J 1938;39:438-41.  Back to cited text no. 9
    
10.
Hasaniya NW, Premaratne S, Varnes PM, Shin D, Shim W. Hernia into the umbilical cord with incarceration of liver and gall bladder in a newborn. J Pediatr Surg Case Rep 2013;1:432-3.  Back to cited text no. 10
    
11.
Achiron R, Soriano D, Lipitz S, Mashiach S, Goldman B, Seidman DS. Fetal midgut herniation into the umbilical cord: Improved definition of ventral abdominal anomaly with the use of transvaginal sonography. Ultrasound Obstet Gynecol 1995;6:256-60.  Back to cited text no. 11
    
12.
Keshorjit Singh I. Hernia of umbilical cord associated with cleft lip and palate, and congenital glaucoma. J Neonatal Surg 2015;4:49.  Back to cited text no. 12
    
13.
Ono K, Kikuchi A, Takikawa KM, Hiroma T, Yoshizawa K, Sunagawa S, et al. Hernia of the umbilical cord and associated ileal prolapse through a patent omphalomesenteric duct: Prenatal ultrasound and MRI findings. Fetal Diagn Ther 2009;25:72-5.  Back to cited text no. 13
    
14.
Victoria T, Andronikou S, Bowen D, Laje P, Weiss DA, Johnson AM, et al. Fetal anterior abdominal wall defects: Prenatal imaging by magnetic resonance imaging. Pediatr Radiol 2018;48:499-512.  Back to cited text no. 14
    
15.
Pal K, Ashri H, Al Wabari A. Congenital hernia of the cord. Indian J Pediatr 2009;76:319-21.  Back to cited text no. 15
    
16.
Ceccanti S, Falconi I, Frediani S, Boscarelli A, Musleh L, Cozzi DA, et al. Umbilical cord sparing technique for repair of congenital hernia into the cord and small omphalocele. J Pediatr Surg 2017;52:192-6.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2]



 

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