Journal Information
Vol. 83. Issue 2.
Pages 132-134 (01 August 2015)
Vol. 83. Issue 2.
Pages 132-134 (01 August 2015)
Scientific Letter
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Review of published cases of hepatic choristoma. Differential diagnosis of umbilical cord masses
Revisión de casos publicados de coristoma hepático. Diagnóstico diferencial de masas de cordón umbilical
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Y. González Ruiza,
Corresponding author
yuremagr@gmail.com

Corresponding author.
, L. Cotaina Graciab, M. Ruiz de Temiñoa, A. Joana González Esguedaa, M.R. Delgado Alviraa
a Servicio de Cirugía Pediátrica, Hospital Universitario Miguel Servet, Zaragoza, Spain
b Servicio de Ginecología y Obstetricia, Hospital Universitario Miguel Servet, Zaragoza, Spain
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Table 1. Literature review of published hepatic choristoma cases.
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Dear Editor:

We present the case of a primigravida, 33 years of age, with no medical or surgical history of interest. Ultrasound examination at 28 weeks of gestation confirmed the presence of a 28mm×17mm mass in the umbilical cord, with an umbilical cord diameter of 16mm, and a small anechoic area with thin walls suggestive of hernial oedema.

The patient had a normal delivery at 40+1 weeks of gestation, giving birth to a girl that weighed 3290g and had an Apgar score of 9/10.

At birth, we observed an umbilical cord with a 4.5cm×2cm×1.8cm bulge protruding from its normal insertion site at the abdomen, lined with amniotic membrane through which could be seen a firm, wine-red mass located 1cm away from the navel that was irreducible, with no accompanying symptoms (Fig. 1). Based on the examination findings, we considered the differential diagnosis of abdominal wall defect and umbilical cord mass.

Figure 1.

Transillumination of the wine-red mass in the umbilical cord.

(0.16MB).

The surgery involved the opening of the amniotic membrane in layers, revealing a solid mass in direct contact with the umbilical vein and with an intraperitoneal communication with the round ligament of the liver. The vascular structures and remnants of the umbilical cord were ligated, the mass fully resected, and the umbilical defect closed. There were no postoperative complications and the patient was discharged 5 days after the surgery.

The mass was submitted to the anatomical pathology department for investigation, and gross examination showed a well-defined brownish nodule measuring 2.5cm, with a microgranular appearance upon sectioning that corresponded to hepatic tissue with preserved architecture at the histological level. The tissue surrounded a cyst-like structure consisting of gallbladder wall tissue that was compatible with a hepatobiliary choristoma.

Ectopic liver is a rare condition described as the presence of hepatic tissue outside the liver and with no hepatic connection.1

The literature has reported the gallbladder as the most common location of ectopic liver, and it can also be found in the thorax, pancreas, spleen, hepatic ligaments, pylorus, greater omentum, oesophagus, gastric mucosa, adrenal cortex, retroperitoneum, pericardium, placenta and umbilical cord.

Several theories attempt to explain the appearance of ectopic liver in locations other than the gallbladder, such as the development of an accessory lobe that loses its connection with the main liver body, the migration of part of the pars hepatica to other sites where ectopic tissue then develops, or the trapping of hepatocytes by the adjacent mesenchyma during the formation of the liver sinusoids and their subsequent migration to more distant regions, such as the umbilical cord, while the connection with the main liver may be maintained through the umbilical vein.

The differential diagnosis of umbilical cord masses is complex and must include cyst and pseudocyst, haematoma, umbilical artery aneurysm, haemangioma, teratoma, angiomyomyxoma, patent urachus, ectopic liver, as well as the most common diseases of the umbilical cord, which are umbilical cord hernia, gastroschisis and omphalocoele.

Ectopic liver in the newborn is usually diagnosed by chance following imaging tests or surgical procedures performed for unrelated reasons. However, it may be diagnosed due to complications like torsion, which manifests with abdominal pain, gastric outlet obstruction and respiratory distress syndrome, caused by the presence of hepatic tissue in supradiaphragmatic locations.

Only eight other cases of hepatic tissue in the umbilical cord have been described in the literature2–6 (Table 1), and the diagnosis of the umbilical cord mass was made prenatally in three of the nine cases, with the definitive diagnosis being made by anatomical pathology. On rare occasions it can be accompanied by symptoms of infection and be associated with other abnormalities, such as utrachal or biliary atresia, ectopic pancreas and heart and lung malformations. In our case, as happened in the one described by Horn et al.,2 we observed an intraperitoneal connection with the liver that may correspond to the round ligament, a vestige of the left umbilical vein.

Table 1.

Literature review of published hepatic choristoma cases.

  Shaw and Pierog  Park et al.  Preminger et al.3  Wax et al.4  Vaideeswar et al.5  Go and Cho  Lee et al.6  Horn et al.2  Our case 
Maternal age  19  29  ND  28  ND  ND  ND  ND  33 
Delivery  Vaginal  Vaginal  Caesarean  Caesarean  Vaginal  Death  ND  Vaginal  Vaginal 
Suggestive manifestations  Yes  Yes  RDS  RDS  Yes  –  ND  Yes  Yes 
Gestational age  28  40  35  38  39  28  38  39  40 
Weight (g)  1247  3240  2180  3314  2460  ND  3000  ND  3290 
Sex  Male  Female  Female  Female  Female  Male  Male  Female  Female 
Prenatal diagnosis  ND  ND  No  19 week US: UC mass, 6.3mm×3.5mm×7mm, no blood flow  32 seek US: hyperechoic mass on base of UC, 2cm×2cm  No  No  No  28 week US: UC mass, de 28mm×17mm, UC diameter 16mm 
Location  Attached to navel by a stalk  CU insertion site  1.5cm from the end of the UC  Proximal to UC and navel  2.8cm from UC insertion  ND  Central at the navel  ND  1cm from navel, proximal to UC 
Size  7.5cm×6cm×3cm  3mm×3mm  2.5cm×3.5cm  Very small  ND  1.8cm×1.2cm×1.2cm  ND  4.5cm×2cm×1.8cm 
Colour  Red-purple  Yellow-green, brown interior  Red  Dark red  Pale brown  Brown  Yellowish grey  Purplish brown  Wine red 
Consistency  ND  Firm, rubbery  Firm  ND  Firm  Soft  Firm, polyp-like  ND  Firm 
Pathological anatomy  ND  ND  Normal hepatic tissue  Urachus cyst with immature hepatic tissue  Hepatic tissue with numerous portal areas  Hepatic cords without bile ducts  Hepatocytes and fibrous connective tissue stroma, blood vessels and nerves  ND  Hepatic tissue surrounded a small cyst-like structure corresponding to gallbladder wall tissue 
Peritoneal communication  Uncertain  No  No  No  No  ND  No  Yes  Yes, with the round ligament of the liver 
Associated anomalies  ND  Biliary atresia+ectopic pancreas in the jejunum  No  Atretic segment of the urachus  Double outlet RV +right lung agenesis+HMD in left lung  ND  Absence of fourth toe in left foot  No  No 

HMD, hyaline membrane disease; ND, not described; RDS, respiratory distress syndrome; RV, right ventricle; UC, umbilical cord; US, ultrasound.

To conclude, we would like to highlight that when ultrasound examination reveals a mass in the umbilical cord we should consider the possibility of rare conditions, like the one described here, along with more common diseases.

Doppler ultrasound of the mass can be helpful to this end, although as we mentioned above, in most cases the definitive diagnosis will be made postnatally. At any rate, the histological characteristics of the lesion should not change the obstetric approach in the absence of intestinal or vascular involvement in the foetus, and the surgical approach will depend on the suspected diagnosis after birth.

References
[1]
Y. Collan, A. Hakkiluoto, J. Hästbacka.
Ectopic liver.
Ann Chir Gynaecol, 67 (1978), pp. 27-29
[2]
F. Horn, E. Kubikova, D. Dubravova, P. Babal, H. El Falougy, P. Selmeciova, et al.
Omphalocele with ectopic liver forming a mesodermal cyst.
Bratisl Lek Listy, 114 (2013), pp. 100-103
[3]
A. Preminger, R. Udassin, O. Pappo, I. Arad.
Ectopic liver tissue within the umbilical cord.
J Pediatr Surg, 36 (2001), pp. 1085-1086
[4]
J.R. Wax, M.G. Pinette, A. Cartin, J. Blackstone.
Ectopic liver: a unique prenatally diagnosed solid umbilical cord mass.
J Ultrasound Med, 26 (2007), pp. 377-379
[5]
P. Vaideeswar, D. Yewatkar, R. Nanavati, P. Bhuiyan.
Ectopic liver tissue in umbilical cord.
J Postgrad Med, 57 (2011), pp. 229-230
[6]
S.Y. Lee, C.W. Nam, J.H. Suh.
Heterotopic liver in the umbilicus: a case report.
Basic Appl Pathol, 4 (2011), pp. 30-32

Please cite this article as: González Ruiz Y, Cotaina Gracia L, Ruiz de Temiño M, González Esgueda AJ, Delgado Alvira MR. Revisión de casos publicados de coristoma hepático. Diagnóstico diferencial de masas de cordón umbilical. An Pediatr (Barc). 2015;83:132–134.

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