ABSTRACT
The author reports a rare case of umbilical cord hernia with the accessory lobe of the liver entrapped in the amniotic sac with radial limb anomaly with malrotation. The accessory lobe of the liver is a rare anatomical variant of the liver that is often asymptomatic. The accessory lobe of the liver causing nonclosure of the umbilical ring is also very rare. The accessory lobe of the liver was formed during the development of embryonic body wall folds and disturbed the complete closure of the umbilical ring. If a clear-cut diagnosis of the accessory lobe is made, then it should be resected. In case of doubt, it should be fixed to the abdominal wall and regularly followed to look for signs and symptoms of torsion of the accessory lobe of the liver.
KEYWORDS: Accessory lobe of liver, limb anomaly, neonate, umbilical cord hernia
INTRODUCTION
Hernia of the umbilical cord is a protrusion of the small intestine into the umbilical cord due to nonclosure of the umbilical ring. It occurs due to failure of complete return of physiologically herniated bowel into the abdominal cavity during the 10th week of embryonic life.[1] Hernia sac seldom contains the liver as its content. The accessory lobe of the liver causing failure of closure of the umbilical ring and presenting as a main content of sac of umbilical cord hernia is extremely rare. We present a case of hernia of the umbilical cord with the accessory lobe of the liver as its main content. Our patient also had associated malrotation of the gut and radial limb defect.
CASE REPORT
A 1-day-old female child with a birth weight of 2.0 kg was admitted with us with hernia of the umbilical cord with right radial limb defect. The baby was full-term normal vaginal delivery born. The amniotic sac was intact, and the umbilical ring defect was about 2.0 cm in diameter. Contents of the hernial sac could not be reduced into the abdominal cavity [Figure 1]. The baby was hemodynamically stable. The baby also had a right radial limb defect [Figure 2]. Ultrasound of the sac and abdomen revealed that the liver was the main content of the hernial sac and the rest of the abdomen was normal. Echocardiography was normal. No other congenital anomaly was detected. There was no complaint of vomiting, and the baby passed meconium immediately after birth. After proper evaluation and resuscitation, the baby was planned for surgical repair. On exploration, a firm, dark brown solid mushroom-shaped mass 2.0 cm in diameter and 4.0 cm in length was found, typical of a liver. The elongated mass was attached to the left lobe of the liver. On exploration, both the right and left lobes of the liver were found to be placed normally. With the above findings, a diagnosis of the accessory lobe of the liver was made [Figure 3]. The accessory lobe of the liver was dissected free from the amniotic sac and fixed to the inside of the anterior abdominal wall to avoid torsion of the accessory lobe of the liver. Malrotation was noted and corrected, and a small bowel was placed in the left hypochondrium. Primary repair of the abdominal wall defect was accomplished without any difficulty. The patient was extubated and shifted to the postoperative care ward. Orals were started on the 4th postoperative day. The baby is now symptomatic and doing well.
Figure 1.
Umbilical cord hernia
Figure 2.
Radial limb anomaly
Figure 3.
Intraoperative photograph of the accessory lobe of the liver
DISCUSSION
Umbilical cord hernia is a simple failure of the midgut to return to the abdominal cavity at 10–12 weeks. Such hernias are much smaller than omphalocele and have a better prognosis. It is less than 4 cm, and the hernia contents are covered by a membrane. It is often confused with omphalocele. The differences are that it contains only the midgut, but not the liver and the absence of true abdominal wall defect. In hernia of the umbilical cord, there is normal cord insertion, whereas in omphalocele, the umbilical cord insertion is on the top of the herniated sac. Associated chromosomal anomalies are low with umbilical cord hernia when compared with omphalocele. Even though omphalocele is usually associated with chromosomal anomalies, 20% are sporadic cases which do not have any other associated anomalies. The rectus abdominis muscle meets in the midline at the xiphoid. Malrotation is also a common feature, although it may or may not cause intestinal obstruction.[1] An area of normal skin always covers the umbilical ring and proximal part of the cord. The amniotic sac seldom involves the liver, and the condition is characteristic of this type of defect. However, there are several documented cases in which an accessory lobe of the liver was the main content in umbilical cord hernia[2,3,4,5,6,7,8] [Table 1].
Table 1.
A review of literature on umbilical cord hernia with accessory liver lobe
| Previous literature | Number of cases | Sex | Sac contained | Surgery done | Other anomalies |
|---|---|---|---|---|---|
| Festen et al.[2] | 2 | Male | Accessory lobe of the liver, containing the GB | Resection | None |
| Ito et al.[3] | 1 | Male | Accessory lobe of the liver | Resection | None |
| Hasaniya et al.[4] | 1 | Male | Incarcerated liver tissue and GB | Resection | Malrotation, tetralogy of Fallot, amputation of right thumb |
| Almatar et al.[5] | 3 | Two males One female | Case 1: Left liver lobe Case 2: Part of liver adherent to sac Case 3: The right lobe of the liver | Reduction Reduction Reduction | Atrial septal defect Patent ductus arteriosus and atrial septal defect None |
| Maekawa et al.[6] | 1 | Female | Accessory lobe of the liver | Reduction | None |
| Brandtner et al.[7] | 1 | Male | Accessory lobe of the liver, containing the GB | Resected | Ventricular septal defect Patent foramen ovale and patent ductus arteriosus |
| Mili et al.[8] | 1 | Male | Intact liver tissue with an embedded GB | Resected | None |
| Present study | 1 | Female | Accessory lobe of the liver | Reduction And fixation to the anterior abdominal wall | Radial limb anomaly |
GB: Gallbladder
The accessory lobe of the liver is a congenital ectopic liver tissue resulting from an error during the embryogenetic process of endodermal caudal foregut at the 3rd week of embryonic life. It can be classified into four types according to Collan et al.:[9]
(i) A large accessory liver lobe connected to the main liver by a connective tissue strand
(ii) A small accessory liver lobe connected to the main liver, weighing 10–30 g
(iii) An ectopic liver that is primarily attached to the gallbladder or intra-abdominal ligaments and is not in contact with the liver proper
(iv) A microscopic ectopic liver found in the gallbladder wall.
Another classification by Stattaus et al. divides the accessory liver lobes into only two entities:[10]
(i) One which is connected to the liver, i.e. pedunculated/sessile
(ii) The other completely separated, i.e. true ectopic liver.
The accessory lobe of the liver is considered a rare entity, usually asymptomatic, with a reported incidence of 0.44%.[11] Nonclosure of the umbilical ring due to an accessory lobe of the liver is an extremely rare condition. We found eight such cases in the literature in addition to our own[2,3,4,5,6,7,8] [Table 1]. An apparent attempt at closure of the abdominal wall was noted in all cases, as described by small anterior abdominal wall defect and constriction at the base of the accessory lobe. The accessory lobe of the liver formed the main content of the umbilical cord hernia and was connected by a pedicle containing important vessels of the liver. None of the hepatic anomalies were diagnosed preoperatively.
There has been no clear explanation why this hepatic anomaly occurs in case of umbilical cord hernia. In normal embryogenesis, the liver does not normally participate in the evisceration during embryogenesis. Some authors have postulated that the accessory lobe of the liver is caused by extra-embryonic entrapment of a portion of the liver, while others proposed that free portion of the liver was forced to grow out due to high intra-abdominal pressure by rectus abdominis muscles. As long as the cavity is present anteriorly, free portion of the liver may protrude from the abdominal defect. Later, a leading of the cephalic fold entraps a portion of the developing liver in the septum transversum. Subsequently, the trapped liver becomes elongated over a pedicle, manifesting as an accessory lobe of the liver. Histological examination of the mass shows normal hepatic cells with signs of hematopoiesis.[12]
The radial limb anomaly encompasses a spectrum of complex congenital malformations of the radial side of the forearm which according to Bayne and Klug can be classified into four types:[13]
(i) Type I: Short distal radius (the distal growth plate is present)
(ii) Type II: Hypoplastic radius, smaller and thinner than the ulna, with no growth plate
(iii) Type III: Partially absent radius (a small proximal segment of the radius remains)
(iv) Type IV: Complete absence of the radius.
It is also a rare entity and is usually associated with other congenital syndromes such as VACTERL association, VATER syndrome, Holt-Oram syndrome, thrombocytopenia-absent radius syndrome, and Fanconi anemia.[14] An association of omphalocele with radial limb abnormalities and diaphragmatic hernia was first described by Gershoni-Baruch et al. and few others.[15] However, we have not found any reported case of radial limb anomalies in association with umbilical cord hernia that primarily involves the accessory lobe of the liver except for the one reported Hasaniya et al. with an amputated right thumb[4] [Table 1].
Management includes hemodynamic stabilization and investigations to rule out other associated congenital anomalies. After careful separation of the accessory lobe of the liver from the amniotic sac, the umbilical ring is closed and defect is repaired. Complications of the accessory lobe of the liver with umbilical cord hernia include torsion, so accessory lobe resection is recommended in cases with small pedicle.
The main purpose of presenting this case is that the accessory lobe of the liver if diagnosed preoperatively should be excised to prevent postoperative complications of accessory lobe of liver torsion and acute abdomen. However, when the preoperative diagnosis is not clear or there is any doubt regarding the nature of mass, it should be fixed to the inside of the anterior abdominal wall and the patient should be kept on regular follow-up. Any presentation as an acute abdomen should be viewed with caution in view of high chances of torsion of the accessory lobe of the liver.
To conclude, the accessory lobe of the liver as content in hernia of the umbilical cord with radial limb anomaly in neonates is a rare association, and care should be taken while ligating the cord in these children to avoid injury to the contents of the sac.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given consent for images and other clinical information to be reported in the journal. The guardian understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Conflicts of interest
There are no conflicts of interest.
Funding Statement
Nil.
REFERENCES
- 1.Gajdhar M, Kundal VK, Mathur P, Gajdhar M. Pitfalls in the umbilical pit: Giant hernia of the umbilical cord. BMJ Case Rep. 2013;2013:bcr2013009381. doi: 10.1136/bcr-2013-009381. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Festen C, Severijnen RS, vd Staak FH. Gallbladder embedded in an accessory liver lobe in umbilical cord hernia. J Pediatr Surg. 1988;23:978–9. doi: 10.1016/s0022-3468(88)80399-3. [DOI] [PubMed] [Google Scholar]
- 3.Ito F, Ando H, Watanabe Y, Seo T, Murahashi O, Harada T, et al. An accessory lobe of the liver disturbing closure of the umbilical ring. Pediatr Surg Int. 1999;15:394–6. doi: 10.1007/s003830050609. [DOI] [PubMed] [Google Scholar]
- 4.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. [Google Scholar]
- 5.Almatar Z, Jalil S, Huseynova RA, AlNafisah T, Wahid FN. Liver as sole content of umbilical cord hernia. J Pediatr Surg Case Rep. 2021;65:101774. [Google Scholar]
- 6.Maekawa S, Kogata S, Sasaki T. A case of hernia of the umbilical cord with an accessory liver. J Neonatal Surg. 2021;10:26. [Google Scholar]
- 7.Brandtner MG, Stundner-Ladenhauf HN, Lapointe-Rohde S, Schimke C, Kluth D, Metzger R. Pediculated accessory liver lobe with gallbladder in a preterm with umbilical cord hernia. Children (Basel) 2022;9:1754. doi: 10.3390/children9111754. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Mili T, Ben Ahmed Y, Ben Younes A, Charieg A, Marzouki M, Chibani I, et al. Hernia of umbilical cord containing an accessory liver lobe and the gallbladder: A case report. J Pediatr Surg Case Rep. 2023;93:102632. [Google Scholar]
- 9.Collan Y, Hakkiluoto A, Hästbacka J. Ectopic liver. Ann Chir Gynaecol. 1978;67:27–9. [PubMed] [Google Scholar]
- 10.Stattaus J, Kühl H, Forsting M. Diagnosis of an Accessory Liver Lobe Established by Magnetic Resonance Imaging-Guided Core Biopsy. Z Gastroenterol. 2008;46:351–4. doi: 10.1055/s-2007-963526. [DOI] [PubMed] [Google Scholar]
- 11.Sommariva A, Pasquali S, Stramare R, Montesco MC, Tropea S, Rossi CR. Laparoscopic diagnosis and treatment of a twisted accessory liver lobe. [[Last accessed on 2024 Apr 01]];CRSLS MIS Case Rep SLS. 2014 18 e2014.00170. Available from: https://crsls.sls.org/2014-00170 . [Google Scholar]
- 12.Wang C, Cheng L, Zhang Z, Xie T, Ding H, Deng Q, et al. Accessory lobes of the liver: A report of 3 cases and review of the literature. Intractable Rare Dis Res. 2012;1:86–91. doi: 10.5582/irdr.2012.v1.2.86. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 13.Bayne LG, Klug MS. Long-term review of the surgical treatment of radial deficiencies. J Hand Surg Am. 1987;12:169–79. doi: 10.1016/s0363-5023(87)80267-8. [DOI] [PubMed] [Google Scholar]
- 14.Goldfarb CA, Wall L, Manske PR. Radial longitudinal deficiency: The incidence of associated medical and musculoskeletal conditions. J Hand Surg Am. 2006;31:1176–82. doi: 10.1016/j.jhsa.2006.05.012. [DOI] [PubMed] [Google Scholar]
- 15.Gershoni-Baruch R, Machoul I, Weiss Y, Blazer S. Unknown syndrome: Radial ray defects, omphalocele, diaphragmatic hernia, and hepatic cyst. J Med Genet. 1990;27:403–4. doi: 10.1136/jmg.27.6.403. [DOI] [PMC free article] [PubMed] [Google Scholar]