Hernia of umbilical cord: An enigma

Pooja Tiwari; Ram Mohan Shukla; Maneesh Kumar Joleya; Sarvagya Jain; Ashok Kumar Ladda; Shashi Shankar Sharma; Vinod Raj; Brijesh Kumar Lahoti; Manoj Joshi

doi:10.4103/jiaps.jiaps_89_22



ORIGINAL ARTICLE

Year : 2023 \| Volume : 28 \| Issue : 1 \| Page : 14-17

Hernia of umbilical cord: An enigma

Pooja Tiwari¹, Ram Mohan Shukla¹, Maneesh Kumar Joleya¹, Sarvagya Jain², Ashok Kumar Ladda¹, Shashi Shankar Sharma¹, Vinod Raj¹, Brijesh Kumar Lahoti¹, Manoj Joshi¹
¹ Department of Pediatric Surgery, SSH and MGMMC, Indore, Madhya Pradesh, India
² Department of General Surgery, SSH and MGMMC, Indore, Madhya Pradesh, India

Date of Submission	28-Jun-2022
Date of Decision	08-Oct-2022
Date of Acceptance	26-Oct-2022
Date of Web Publication	10-Jan-2023

Correspondence Address:
Maneesh Kumar Joleya
Department of Pediatric Surgery, SSH and MGMMC, Indore, Madhya Pradesh
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jiaps.jiaps_89_22

Abstract

Introduction: Hernia of the umbilical cord (HUC) is an uncommon problem which is often misdiagnosed, leading to inadequate treatment and various complications which cause increased morbidity and mortality in neonates. To address this issue, we took up this study.
Materials and Methods: Patients diagnosed with HUC from January 1, 2017, to December 31, 2021, were retrospectively analyzed. The following data of all these patients were collected and retrospectively analyzed: demography, radiological investigations, echocardiography, contents of hernia, type of surgery performed, and outcome.
Results: Eighteen out of 19 patients included in the study were taken up for surgery after baseline investigations and echocardiography. Out of 19 patients, 15 were discharged successfully and are on regular follow-up. Of the remaining four patients, an anastomotic leak occurred in two and they went into sepsis and succumbed. One of the patients died before any intervention and one succumbed on 3^rd postoperative day due to sepsis.
Conclusion: Timely referral and intervention can save precious lives. We need to educate doctors and health-care providers so that proper diagnosis and timely management can be done for this anomaly which is associated with less morbidity and a lower rate of associated anomalies.

Keywords: Cord clamp injury, gastroschisis, hernia of the umbilical cord, omphalocele, umbilical cord abnormalities

How to cite this article:
Tiwari P, Shukla RM, Joleya MK, Jain S, Ladda AK, Sharma SS, Raj V, Lahoti BK, Joshi M. Hernia of umbilical cord: An enigma. J Indian Assoc Pediatr Surg 2023;28:14-7

How to cite this URL:
Tiwari P, Shukla RM, Joleya MK, Jain S, Ladda AK, Sharma SS, Raj V, Lahoti BK, Joshi M. Hernia of umbilical cord: An enigma. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Feb 2];28:14-7. Available from: https://www.jiaps.com/text.asp?2023/28/1/14/367393

Introduction

Hernia of the umbilical cord (HUC) is a simple failure of the midgut to return to the peritoneal cavity at 10–12 weeks.^[1] HUC is an uncommon problem which is not properly addressed due to missed diagnosis. Due to misdiagnosis, inadequate treatment occurs, leading to various complications resulting in increased morbidity and mortality in neonates. To address this issue, we took up this study in which all the cases of HUC managed over 5 years in the department of pediatric surgery, of our institute were analyzed and reviewed. To the best of our knowledge, it is one of the largest studies reported in English literature.

Materials and Methods

All patients of HUC who were admitted to the department of pediatric surgery, of our institute from January 1, 2017, to December 31, 2021, were included in this study. Those patients whose data were incomplete or missing were excluded from the study. A total of 19 patients were included in our study period of 5 years. The following data of all these patients were collected and analyzed:

Demographic profile
Baseline investigations
X-ray of the abdomen and ultrasound of the umbilical cord with the abdomen
Echocardiography
Contents of HUC on exploratory laparotomy
Surgery performed
Outcomes with morbidity and mortality.

Results

Eighteen out of 19 patients were taken up for surgery after baseline investigations and echocardiography. The characteristic skin collar of HUC was seen in all patients including the one with liver as content [Figure 1]a and [Figure 1]b. Of 19 cases, 18 had small bowel as the content of which four presented with gangrenous changes, three had patent vitellointestinal duct (VID) [Figure 2]a and [Figure 2]b, and one had ileal perforation. One patient had liver as content, one presented with ileal atresia [Figure 3]a, and one presented with a ruptured sac and exposed small bowel [Figure 3]b. All patients underwent reduction of contents with umbilicoplasty except one who presented with septic shock and succumbed.

Figure 1: (a) HUC with the diagnostic skin collar and (b) HUC with liver as a content-intraoperative picture. HUC: Hernia of the umbilical cord

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Figure 2: (a) HUC with patent vitellointestinal duct with reverse intussusception and (b) Type 3 HUC with patent vitellointestinal duct. HUC: Hernia of the umbilical cord

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Figure 3: (a) HUC with ileal atresia and (b) Type 4 HUC. HUC: Hernia of the umbilical cord

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Out of 19 patients, 15 (79%) were discharged successfully and are on regular follow-up. Of the remaining four patients, an anastomotic leak occurred in two and they went into sepsis and succumbed. One of these neonates with an anastomotic leak had a cord clamp injury due to missed diagnosis which led to the patient's demise. Of the other two patients, one came very late with severe dehydration and septic shock but could not be revived in spite of the best of our efforts and died before any surgical intervention. Another patient, which had HUC with ileal atresia with internal hernia with bowel gangrene and sepsis succumbed on the 3^rd postoperative day. The overall mortality in our series was 21%. Our results are shown in [Table 1]. One more patient's cord was clamped due to misdiagnosis but could be managed due to timely intervention and was discharged after surgery.

Table 1: Summary of cases of hernia of umbilical cord

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Discussion

HUC is a very rare anomaly. The reported incidence of HUC is 1 in 5000 live births.^[2] In literature, it is reported to be more common in males (3:1)^[2] but in our study, the ratio was nearly equal (1.1:1) in both sexes.

Embryologically, the bowel herniates into the umbilical cord at the 6^th week of gestation and goes back into the abdominal cavity by the 10^th–12^th weeks of gestation. If the bowel fails to go back it results in congenital umbilical cord hernia, but the actual cause behind the failure of retraction of the bowel is still not known. One hypothesis suggests that HUC occurs due to the failure of normal development of the umbilical ring causing continued communication between the fetal peritoneum and extraembryonic mesoderm.^[3]

It is easy to differentiate HUC from gastroschisis (no covering membrane) and omphalocele major (size >4 cm) but it is really difficult to differentiate it from omphalocele minor.

In HUC, there is a characteristic skin collar (ranging from ½ inch to 1 inch) [Figure 1a] from the wall of the abdomen to the umbilicus^[4] which is not there in omphalocele minor. Furthermore, the presence of a patent umbilical ring^[5] and normally attached rectus abdominis muscle at the xiphoid, i.e. the abdominal wall above the defect is normal, which helps in clearly differentiating HUC from omphalocele minor.^[4],[6]

The HUC can be classified into four types as follows:^[7]

Type 1 is a simple hernia into the cord without any associated complications
Type 2 is associated with intestinal obstruction
Type 3 is a distinct variety associated with mucosal prolapse, and
Type 4 is associated with evisceration.

In our study, eight patients had Type 1 HUC; seven had Type 2 HUC, three had Type 3 HUC and only one had Type 4 HUC [Table 1].

The standard textbook definition of HUC states that it contains only the midgut and never the liver.^[7]

The only contrasting feature to what is mentioned in the textbook was the presence of liver in one of our patients which is similar to many case reports and case series published in the literature^{[5],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17]} which suggests that the standard textbook definition might need revision [Table 2].

Table 2: Various studies of hernia of the umbilical cord with liver as content

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The associated anomalies in our study were malrotation, ileal atresia with an internal hernia in one case, reversed intussusception in one case, and patent VID in three cases. These are similar to other published studies. HUC can also be associated with persistent cloaca, Meckel's diverticulum, short bowel syndrome, bowel stenosis, and rarely with cleft lip, cleft palate, glaucoma, and congenital heart diseases.^[18] HUC is different from exomphalos which is associated with 30%–40% chromosomal anomalies as generally HUC is not associated with chromosomal abnormalities.

Furthermore, it has a better outcome than exomphalos (50%–70% cardiac abnormalities) due to a lower incidence of life-threatening cardiac and other associated anomalies. Careful antenatal ultrasound with transvaginal Ultrasonography (USG) can ascertain this congenital defect before birth and then it can be handled appropriately.^[19]

Conclusion

On clinical examination, it is a must-see for any characteristic skin collar, umbilical ring deformity, and any anterior abdominal wall defect to avoid misdiagnosis of HUC and easily differentiate between HUC and omphalocele minor. Iatrogenic gut injury due to cord clamping as a result of misdiagnosis can be avoided by ligating the cord 5–7 cm above the abdominal wall. Timely referral and intervention can save precious lives. We need to educate doctors and health-care providers so that proper diagnosis and timely management can be done for this anomaly which is associated with less morbidity and a lower rate of associated anomalies.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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