Laparoscopic Excision of Solitary Dysontogenetic Liver Cysts in Young Children: Technical Aspects and Outcome

Abstract

Background:

Solitary dysontogenetic liver cysts are rare in young children. However, large cysts can cause symptoms and require therapy. Cyst excision is the therapeutic method least associated with cyst recurrence. Only limited data are available on cyst excision performed laparoscopically in this age group. We present our experience using this surgical approach.

Methods:

Since 2005, 7 children including 5 newborns and infants with solitary dysontogenetic liver cysts have undergone minimally invasive excision of the cyst at our institution. Patient data were analyzed retrospectively.

Results:

Median age of the patients at surgery was 8 months (3 days to 6 years); 5 of them were younger than 1 year. The cysts had varying locations in the segments IV-VIII, and median size was 5.4 cm (3.8–7.9). Complete excision was realized in all cases. Median duration of surgery was 120 minutes (60–171). All procedures could be completed laparoscopically. One intraoperative complication occurred (injury of a bile duct that could be sutured laparoscopically). Median follow-up was 29 months (14–173). Cyst recurrence was not observed in any of the cases.

Conclusion:

Laparoscopic excision of solitary dysontogenetic liver cysts is an effective treatment in young children. Resection is not limited to cysts in anterior and lateral liver segments.

Introduction

Solitary dysontogenetic liver cysts are heterogeneous in histology. Differential diagnoses include simple cysts, intrahepatic choledochal cysts, complex biliary cysts, cystic mesenchymal hamartomas, or ciliated hepatic foregut cysts (CHFC).¹ The overall prevalence of these cysts is reported to be 0.1%–2.5%.^2,3 In adults, the prevalence is 3%–18%,⁴ but it is significantly lower in children. Since the implementation of routine ultrasound investigations during pregnancy, antenatal diagnosis of a solitary liver cyst has become more frequent.⁵ The treatment approach varies from observation to surgery depending on individual conditions. Nonsurgical and surgical procedures have been described for therapy.^6–8 Cyst excision is the preferred method, because it is associated with lowest recurrence rates.⁹

Although there is considerable experience with the laparoscopic excision of solitary liver cysts in adults¹⁰ since this procedure was first performed in 1991,¹¹ there are only a few reports on this technique in children,^12–16 which are usually limited to cysts located in anterior and lateral liver segments. In this study, we present our experience with laparoscopic excision of solitary dysontogenetic liver cysts, including cysts located in posterior liver segments in young children.

Materials and Methods

Patients

Patient records of all children undergoing laparoscopic excision of solitary dysontogenetic liver cysts at our institution since 2005 were retrospectively analyzed. Indications for surgery were clinical symptoms linked to the cyst and substantial increase in cyst size with existing or imminent compression of adjacent structures. Analyzed parameters were patient age and weight at surgery, surgery duration, surgical procedure, size and histology of cysts, and intra- and postoperative complications. Descriptive statistics was applied. Patient data and surgical data are given as median and range. The study was approved by the Ethical Committee of the Medical Faculty of the University of Tuebingen and the University Hospital Tuebingen, Germany (Ref. 039/2020BO2).

Diagnostic and surgical procedure

Preoperatively an ultrasound was carried out in all cases and, if necessary, an additional magnetic resonance imaging (MRI), including magnetic resonance cholangiopancreatography sequences, to plan surgery and to exclude a communication of the cyst with the biliary structures (Fig. 1). Blood analysis included liver enzymes, cholestasis parameters, and tumor markers.

FIG. 1.

MRI and ultrasound of a solitary dysontogenetic liver cyst (Patient No. 6). MRI [(A) coronal plane; (B) axial plane] and ultrasound [(C) sagittal plane; (D) transversal plane] show the cyst in liver Segments VI and VII. MRI, magnetic resonance imaging.

For surgery, patients were placed in supine position with elevation of the upper abdomen for better exposure of the liver and were brought into a slight anti-Trendelenburg position. A 5 mm optical trocar with a 30° optic was placed through a mini laparotomy below the umbilicus. The capnoperitoneum was installed with a pressure of 10–12 mm Hg at a flow of 3.5–5 mL/min CO₂ depending on age and size of the patient. Two 3 or 5 mm working trocars were placed under laparoscopic vision in the upper abdomen. If necessary, 1 or 2 additional trocars were placed to retract the liver or adjacent organs and/or a transabdominal traction suture around the falciform ligament or through the cyst itself was applied (Fig. 2D). The anterior cyst wall was opened in some cases for better visualization of the border between liver parenchyma and cyst, and for easier dissection of the posterior cyst wall. A monopolar hook or exceptionally an ultracision device was used for the dissection. After separation from the liver parenchyma, cysts were removed through the widened incision of one of the trocars. Hemostasis was achieved by electrocautery and hemostyptical devices. A drainage was installed at the end of surgery. To access cysts in the posterior liver segments, the right triangular and falciform ligament are transected. With additional left lateral and increased anti-Trendelenburg positioning of the patient, the more mobile liver folds to the left and caudally, so that the Segments VII and VIII can be accessed well.

FIG. 2.

Laparoscopic excision of a solitary dysontogenetic liver cyst (Patient No. 6). (A) Liver cyst in Segments VI and VII. (B, C) Puncture of the cyst. (D) Transabdominal traction suture through the cystic wall. (E) Excision of the cyst using monopolar hook cautery. (F) Liver after complete excision of the cyst. Color images are available online.

Follow-up

Postoperative routine follow-up included clinical, ultrasound, and laboratory examinations and was performed 4 weeks, 6 and 12 months after surgery. Further examinations were conducted every 6 months until complete resolution of morphological postoperative changes in the ultrasound.

Results

Patient characteristics

We analyzed data of 7 patients (4 girls and 3 boys), 2 of whom were diagnosed antenatally. An additional MRI was performed in all but one case. In Patient 1, indication for surgery was compression of the common bile duct by a cyst located in liver Segments V and VI with a large extrahepatic portion. A cyst in Segments IV, V, and VIII led to respiratory partial failure due to the immense size in Patient 2. In 5 patients (Patients 3–7), the indication for surgery was a substantial increase of the cyst size. One of these children had episodes of abdominal pain due to a growing cyst in Segments IV b and VIII (Patient 3). In Patient 7, the growing cyst showed additional imminent compression of the porta hepatis and upper hepatic veins (Table 1). The blood tests showed no abnormal liver function, cholestasis, or tumor marker. Radiological investigations revealed a dysontogenetic liver cyst without connection to the biliary system in all cases. The median cyst diameter was 5.4 cm (3.8–7.9). Cysts were located not only in the anterior and lateral liver segments but also in the posterior Segments VII (n = 2) and VIII (n = 3) (Table 1). Median age at surgery was 8 months (3 days to 6 years); 5 of the children were younger than 1 year. Median body weight at surgery was 9.4 kg (2.9–15.5).

Table 1.

Patient and Cyst Characteristics

Patient	Age at surgery	Body weight at surgery (kg)	Indication for surgery	Largest cyst diameter (cm)	Cyst location	Histology details	Hospital stay (days)
1	3 Days	3.3	Compression of common bile duct	4.5	Segments V and VI; mainly extrahepatic	Single layer of flat to cuboidal epithelium	5
2	9 Days	2.9	Partial respiratory failure	7.9	Segments IV, V, and VIII; intrahepatic between left and middle hepatic vein; adjacent to hepatic hilar structures	Cyst wall of connective tissue; epithelium not clearly defined	6
3	76 Months	15.5	Increase in cyst volume (fourfold in 18 months); abdominal pain	4.7	Segments IV b and VIII; intrahepatic	Predominantly flat, squamous epithelium	7
4	55 Months	13.8	Increase in cyst volume (28-fold in 50 months)	6.1	Segment VII; mainly extrahepatic; contact to right diaphragm and upper pole of the right kidney	Single layer of squamous epithelium	5
5	10 Months	9.5	Increase in cyst volume (sevenfold in 4 months)	3.8	Segment V; mainly intrahepatic; contact to middle hepatic vein	Single layer of cuboidal epithelium	9
6	7 Months	9.3	Increase in cyst volume (10-fold in 6 months)	3.8	Segments VI and VII; intrahepatic	Single layer of flat epithelium	5
7	8 Months	7.1	Increase in cyst volume; imminent compression of porta hepatis and upper hepatic veins (2.5-fold in 6 months)	7.7	Segments V and VIII; intrahepatic	Single layer of columnar epithelium	4

Surgery

Three to 5 trocars were used for surgery. Median duration of surgery was 120 minutes (60–171) (Table 2). In 1 patient with a centrally located cyst, the gall bladder was removed for safe access to the cyst and not to risk injury of the portal vein or biliary ducts. All procedures could be completed laparoscopically. The cyst was punctured before resection in 3 patients (Fig. 2B, C), and the anterior cyst wall was opened before removal of the posterior cyst wall in another 3 patients. No intra- or postoperative blood transfusion was required.

Table 2.

Surgical Details and Outcome

Patient	Duration of surgery (minutes)	Trocars (n)	Cyst puncture (n)/opening of anterior wall (n)	Complications	Follow up (months)	Outcome
1	60	3	1/0	None	173	Uneventful; NR
2	112	3	1/0	None	61	Uneventful; NR
3	129	5	0/1	None	32	Uneventful; NR
4	68	4	0/1	None	29	Uneventful; NR
5	159	4	0/1	Bile duct injury	23	Uneventful; NR
6	171	3	1/1	None	17	Uneventful; NR
7	150	3	0/1	None	14	Uneventful; NR

NR, no cyst recurrence.

Intra- and postoperative complications

A tangential injury of a bile duct occurred in 1 patient (Table 2). The defect was sutured laparoscopically. Still, the patient developed a bilioma, which resolved after conservative treatment. According to the Dindo-Clavien classification, this complication represents a grade I complication.¹⁷ No other intraoperative or postoperative complications occurred.

Histology and outcome

Complete cyst excision was achieved in all patients. Histological evaluation revealed a simple dysontogenetic liver cyst without signs of malignancy in all cases; further histology details are listed in Table 1. Median hospital stay was 5 days (4–9) (Table 1). Oral feeding was started on the day of surgery (n = 4) or on the first postoperative day (n = 3) and was ended on postoperative day 1 (n = 4), 2 (n = 1), 3 (n = 1), or 4 (n = 1). Analgesic medication was administered for a median duration of 2 days (0–5) after surgery. No cyst recurrence was observed (Table 2) with a median follow-up of 29 months (14–173).

Discussion

We report our experience with minimally invasive excision of solitary dysontogenetic liver cysts in young children, with most of the patients being newborns and infants. The minimally invasive approach for excision of simple solitary liver cysts is well established for adults and it is considered the therapy of choice in this patient group.^10,18,19 However, there are only few reports in children, mainly reporting on individual cases or small heterogeneous cohorts.^12–16 To our knowledge, we present the largest case series of laparoscopic liver cyst excision in this age group, including newborns and infants performed in one center.

For the treatment of solitary dysontogenetic liver cysts, various nonsurgical procedures have been described including aspiration and alcoholic sclerotherapy.⁷ Surgical procedures include deroofing and excision of the cyst.^6,8 Liver resections²⁰ or even liver transplantation should only be the ultima ratio. A recurrence of cysts has been reported after sclerotherapy as well as after deroofing.^21,22 This adverse event is not expected to occur after complete cyst excision, as confirmed in our case series with a median follow-up of 29 months. A further advantage of a complete cyst excision is the complete removal of suspicious tissue in unclear cases, as for example, a malignant transformation to squamous cell carcinoma has been reported for CHFC.²³

Postnatally most cysts regress and asymptomatic cysts need no therapy.^5,24 However, large cysts may compress adjacent vascular or biliary structures and cause symptoms such as pain, nausea, or obstructive jaundice and therefore require therapy. Indications for surgery in the present study were substantial increase in cyst size and symptoms linked to the cyst. They correspond to those reported in other studies.^12–16

We have observed a good correlation between preoperative diagnostic workup and postoperative histopathological findings in our patients. In no case was a biopsy indicated, and primary surgery was always allowed.

According to recommendations for children¹⁴ and for adults,^25,26 laparoscopic cyst excision should be restricted to cysts located in the more accessible anterior and lateral Segments II, III, IVb, V, and VI according to the Couinaud classification.²⁷ The reported cyst excisions in children are limited to these lesions.^12–16 We included not only patients with liver cysts located in these segments but also patients with a location of cysts in Segments VII and VIII. No complication was observed during surgery or the follow-up period after excision of these cysts. Obviously, location of cysts in these segments is not an exclusion criterion for their laparoscopic excision in this age group. Further, we could demonstrate that the central position of the cysts with close proximity to the porta hepatis is not a contraindication for the laparoscopic approach. Complete excision of the cysts is as well possible in this condition provided that surgeons have a relevant expertise with open hepatic surgery and the minimally invasive approach. A prerequisite to perform the procedure minimally invasively is a preoperative MRI, which provides the exact location of the cyst in the liver tissue and its relationship to the surrounding vascular and biliary structures. In case of ambiguity, for example, regarding the relationship of the cyst to important vascular and biliary structures, and the appropriate surgical approach, the open procedure should always be favored.

There are some technical details that help to perform the operation. To facilitate exploration of the cyst and the adjacent anatomical structures as well as to save an additional trocar in an already small working space, we have in some cases retracted the liver at the falciform ligament and/or the cyst with a transabdominal traction suture. We frequently punctured the cyst and aspirated the fluid before the actual excision. For cysts that extend considerably deep into the liver parenchyma, we found opening of the anterior cyst wall helpful for better judging the border of the cyst and to simplify dissection of the posterior wall from bile ducts and vessels.

Spillage of the cyst content would be a problem if an echinococcus cyst is erroneously punctured or its anterior wall is opened. In this case, spillage of the cyst contents may lead to peritoneal hydatidosis and/or anaphylaxis. Therefore, this differential diagnosis must be excluded preoperatively. In general, an MRI is conclusive. Serology may support the diagnosis. In addition, a prenatal diagnosis or diagnosis in the first days of life argues against this parasitic liver cyst. We performed minimally invasive excision of the cyst only after an echinococcus cyst had been excluded. In contrast to most of the other liver cyst excisions described, we preferred to use a monopolar hook for separating the cysts from the liver parenchyma even though using other devices (e.g., a harmonic scalpel) is possibly less time-consuming. Using a 3 mm monopolar hook gave us a better sensory haptic feedback, which allowed us to perform a more precise dissection of the cyst from anatomically relevant borders such as veins or bile ducts. The risk of injury is much higher with the harmonic scalpel due to its forceps grip.

The small working space during laparoscopic procedures in infants is potentially associated with an increased risk of injury of adjacent anatomical structures. We experienced injury of the right bile duct during resection of a cyst located mainly intrahepatic in contact to the middle hepatic vein (Table 1; Patient No. 5). The injured duct was sutured, and a subsequent bilioma disappeared over time without requiring any further intervention. The simultaneous removal of the gallbladder together with the cyst in one patient was, in our opinion, rather due to the specific anatomical conditions than to specific laparoscopic conditions such as small working space. Provided that surgeons are experienced with laparoscopic and hepatic operations, contact with large vessels (e.g., Patient No. 5) or the bile ducts does not preclude the minimally invasive approach in our view.

The advantages of minimally invasive procedures are decreased bleeding, reduced surgery time, less pain, reduced hospital stay, and better cosmetic outcome.^15,28,29 In addition, this approach leads to a reduced surgical trauma, which is particularly beneficial in children as less intensive medication is required postoperatively and intensive care can be avoided. No blood transfusion was necessary intra- or postoperatively in our series. We observed a median surgery time and median hospital stay of 120 minutes and 5 days, respectively. Data on open excisions in children are rare. Rygl et al.⁸ report 6 cases of open excised congenital solitary liver cysts. Median hospital stay was 14 days (10–30). More data that show the advantage of the laparoscopic procedure are available for adults: A combined analysis of several published studies on the surgical therapy of liver cysts, which however was not confined to cyst excision, showed that a laparoscopic procedure is associated with a statistically significant reduction in surgery time and hospital stay.¹⁸

Conclusion

Laparoscopic excision of solitary dysontogenetic liver cysts is an effective treatment in young children, including newborns and infants. Specific techniques can facilitate the surgical procedure. With experience in open liver surgery and minimally invasive surgery, and with good preoperative diagnostics, resection is not limited to cysts located in the more accessible anterior and lateral liver segments but can be performed if cysts are located in posterior segments as well. An open procedure should be performed if important vascular and biliary structures interfere with a minimal invasive approach. Our study confirms the observation of studies in adults, indicating an advantage of the laparoscopic procedure compared with the open procedure in terms of surgery duration and hospital stay.

Footnotes

Authors' Contributions

A.S. substantially contributed to the conception of the work and to the interpretation of data for the work, drafted the work, and has finally approved the version to be published. I.T. and V.E. substantially contributed to the acquisition and analyses of data for the work, revised the work critically for important intellectual content, and have finally approved the version to be published. J.F. and S.W.W. substantially contributed to the design of the work and to the interpretation of data for the work, revised the work critically for important intellectual content, and have finally approved the version to be published. All authors agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Disclosure Statement

No competing financial interests exist.

Funding Information

No funding was received for this article.

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