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Laparoscopic Treatment for Antenatally Detected Solitary Giant Cyst of the Liver

de Lagausie P.¹, Potier A.², Gorincourt G.³, Suprano I*, Muller F**, Guys J.M.¹
¹Service de Chirurgie Pédiatrique, ²Diagnostic anténatal, ³Service de radiologie, *Service d’anésthésie-réanimation, Hôpital
d’Enfant la Timone, Marseille, **Service de Biochimie-hormonologie, Hôpital Robert Debré, Paris, France

 

 

Correspondence

Prof Pascal de Lagausie, MD, PhD,
Department of Pediatric Surgery
Hôpital la Timone; 264 rue Saint Pierre
13385 Marseille cedex 05
Tel: +33 (0) 491 384 714 / Fax: +33 (1) 491 384 714
Mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

The antenatal diagnosis of intra-abdominal mass may present some problems especially in females; it may be difficult to estimate the nature of the lesion and its repercussions on foetal development. We report the case of a giant intraabdominal cyst, early diagnosed during the 12th week of pregnancy, with no signs of foetal hydrops or other malformations. In the pursuit and surveillance of pregnancy a partial regression of the cyst took place and at the end of pregnancy it occupied only the right flank. Delivery didn’t present any problem and due to a perfect clinical adaptation this young boy was operated laparoscopically one month later. His mass of right flank attached to liver revealed to be a hepatic cyst. This observation reminds us that hepatic cyst must represent a possible aetiology of abdominal cystic mass diagnosed antenatally. The evolution of a giant cyst, like this, may be completely favourable.

Key words: foetal hepatic cyst, prenatal diagnosis, ultrasound, amniotic fluid enzymes

 

 

Introduction

Fetal intra-abdominal cysts detected by antenatal ultrasound, especially for giant cysts, present some difficulties of diagnosis and clinical management [1]. The size of these cysts can sometimes perturb the development of respiratory and urinary systems. We report the case of a giant cyst in a male foetus that revealed to be a hepatic cyst.

Clinical case

In a primiparous patient the ultrasound performed at the 12 weeks found the evidence of a giant cystic intraabdominal mass of 40 mm. This exam showed no calcification but a central septum ( fig. 1). The diagnosis of intra-abdominal lymphangioma was proposed. The big size of the cyst and the impossibility to visualize the others intra-abdominal organs made us to discuss about interruption of pregnancy.

giant cyst 1 

Fig 1. Longitudinal section through the foetal abdomen showing a giant cyst at 12 weeks of gestation 

The puncture of the cyst (at 14 weeks) showed the elements indicated in table I. The results eliminate a strict urinary origin, an anterior meningocele, a mesentery cyst and a choledochal cyst. Possible aetiologies based on these results were: cloaca, antenatal duplication, amniotic fluid. The result of the karyotype done by amniotic fluid ponction was normal, 46 XY. However, because of a good tolerance from the point of view of growth, absence of foetal hydrops or repercussions on thoracic development, we decided to continue the pregnancy. There was no hydramnios. After that the giant cyst gradually stabilized while the other organs were better visualized. At 17 weeks, an antenatal MRI ( fig. 2) showed a smaller mass, essentially confined to the right flank.

giant cyst 2

Fig. 2: MRI (17 weeks) - smaller mass, in the right flank 

Thoracic development seemed to be appropriate. There wasn’t any repercussion or dilatation of the urinary tract. An ultrasonographic regular follow-up (every month) confirmed our conservative attitude showing a good weight curve. At 38 weeks’ gestation, the patient was admitted to the labor and delivery room. Prenatal ultrasound exam confirmed the possibility of vaginal delivery. She delivered vaginally a male neonate with an Apgar score of 9 and 10 at 1 and 5 min, respectively and weighing 2270 g. The clinical exam was comforting, but the abdominal wall wass creased, like in Prune Belly syndrome ( fig. 3). No palpable mass was found. The ultrasonography showed a lesion confined to the right flank, possibly in contact with the liver. The MRI confirmed the cystic aspect of the lesion but without a central septum, as that had been seen on the first examination. Intestinal transit was normal and discharge was allowed. One month later the size of the mass was decreasing and we programed the laparoscopic excision of this mass that seemed hung to right liver. We had two possible hypotheses of diagnosis: hepatic cyst and lymphangioma. The laparoscopic exploration showed a cystic lesion in continuity to the inferior edge of the right lobe, recovered by two pieces of ectopic parenchyma as an extension of normal liver. 

giant cyst 3

Fig. 3: Child at one month, at the moment of operation, the abdominal wall is creased 

Results

The excision of the cystic mass of 12 cm, partially filled by serous fluid and recovered by two contingents of ectopic liver was done easily and section was performed by ultra-scissors. The base of the cyst adhering to hepatic capsule was not excised. The mass was put into a bag and extracted through the umbilical incision. Postoperative evolution was favourable, with reintroduction of oral diet on the first day after surgery and discharge on the third day. An ultrasonography performed three months later didn’t show any residual tissue. The patient will be re-examined again at the age of one year for his right cryptorchidism (the right testis was engaged in the inguinal canal during the operation). The pathologic exam concluded for a cyst of hepatic origin.

Discussion

The antenatal diagnosis of intra-abdominal mass may present some difficulties especially in females in whom the more frequent diagnosis is of an ovarian cyst. Lymphangioma is often one of the first diagnostic hypotheses, especially in presence of a septum. The cyst of hepatic origin is rarer than lymphangioma but it merit being evocated [2, 3]. Moreover its treatment is frequently easier, since there are only few adherences with the inferior face of the liver produced during the growth of the mass through the abdominal cavity. The appearance of these cysts, often of very big size, may present problems about the decision to pursuit pregnancy. Nevertheless, the follow-up allow to evaluate the compression of the digestive tract and to verify the absence of major repercussions on urinary tract and thorax. A puncture may be performed to analyse the nature of intra-cystic fluid [4]. However, like in our case (table 1), it may be useless.

Table I: dosage of intra-cystic fluid at 14 weeks

Proteins 2,1 g/l
Albumin 1,1 g/l
beta 2 micro globulin 6,1 mg/l
GGTP 229 UI/l
tLAP 10 UI/l ( 60% of intestinal origin)
LAP 37 UI/l
Lipase 18 UI/l
Ferritin 126 mg/l
Iron 3,6 mmol/l
Estradiol 106 pg/ml
Electrophoresis of cholinesterases normal
IGFBP-1 high positivity
hCG > 1500 UI/l

 

We found on the other hand 229 UI of GGT in the fluid of puncture (normal = 100 UI). This value a little bit high orientated to a lesion of hepatic origin, even if it excludes a cyst of the biliary tract (in this case the dosage of GGT may be over 1000 UI). At birth it is sometimes necessary to decompress the cystic mass [5] because of its volume, or, like in our case, only to follow up the recovery of intestinal transit of the new-born.

Depending on the clinical tolerance, the resection may be more or less differed. We believe that it is reasonable to proceed to the excision of this type of intra-abdominal cysts in post-natal period in order to facilitate the transit (especially if the hypothesis of lymphangioma has not been excluded, because of the risks of infection). In some cases, the hepatic cyst could be punctured percutaneously under ultrasonographic guidance, with good results [6]. For intraparenchymal hepatic cysts, a simple follow-up may be enough. After laparoscopic management of solitary congenital hepatic cyst, results were successful, even in adult patients [7].

On the whole, the diagnosis of hepatic cyst must be considered one of the possible hypotheses for an antenatal giant cyst. A regular follow-up can progressively show the evolution of the intra-abdominal organs and eliminate a repercussion on thoracic and urinary development. A rise of GGT in the cyst fluid is one of the elements of orientation toward a hepatic cyst. In conclusion, early laparoscopy is useful to confirm the diagnosis and to treat this cyst definitively. 

 

 

 

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