Minimally invasive fenestration for congenital hepatic cyst in infant

ABSTRACT

A 19-year-old woman underwent prenatal ultrasonography, which confirmed the presence of an isolated cystic mass in the upper abdominal cavity of a fetus. A female infant weighing 3085 g was delivered at 36 weeks’ gestation. Ultrasonography and computed tomography examination revealed a clear unilocular cyst and occupying the right side of the abdomen. The infant’s respiratory status was unstable, and she fed poorly, owing to compression by the hepatic cyst. We performed ultrasound-guided aspiration of a hepatic cyst at 15 days old, but it rapidly re-grew. Therefore, we performed laparoscopic findings and fenestration of the hepatic cyst via an umbilical arc incision and the cyst wall was excised at 43 days old. The histopathological diagnosis was mesothelial cell-derived hepatic cyst. Three years after the operation, no recurrence has been observed. Hepatic cyst fenestration by umbilical incision can be performed safely in infants and it is a cosmetically superior method.

INTRODUCTION

The incidence of solitary congenital hepatic cysts in children is rare. They occur more commonly in girls, are not associated with cysts in other organs and do not usually communicate with the biliary tree [1]. Cysts are generally asymptomatic unless a neighboring structure is compressed or if the cysts rupture, hemorrhage or torsion occurs [2]. A surgical excision is indicated when there is symptomatic disease, complications or a malignant tumor is suspected [3].

CASE REPORT

A 19-year-old woman, gravida 1, para 0, had routine prenatal ultrasonography, which confirmed the presence of an isolated cystic mass in the upper abdominal region of a fetus at 27 weeks’ gestation. The cyst measured 54.7 × 33.4 × 55.0 mm (Fig. 1). Normal kidneys and bladder were identified and were separate from the cyst. The amniotic fluid volume was normal. A female infant weighing 3085 g was delivered by spontaneous vaginal delivery at 36 weeks’ gestation. Apgar scores at 1- and 5-min were 8 and 9, respectively. She had poor oxygenation owing to diaphragmatic compression by a giant hepatic cyst and required respiratory assistance with continuous positive airway pressure. Ultrasonography and computed tomography (CT) examination revealed a clear unilocular cyst measuring 98 × 60 × 32 mm and occupying the right side of the abdomen, extending from the inferior aspect of the right liver lobe to the pelvis (Fig. 2). The infant’s respiratory condition gradually improved, but the hepatic cyst did not regress spontaneously. We decided that neonatal treatment was desirable because of the symptoms such as apneic attack related to pressure on related structures caused by the cyst, and performed ultrasound-guided percutaneous hepatic cyst puncture drainage when the infant was 15 days old. The fluid inside the liver cyst was clear and contained no bile components. There was no continuity with the bile duct. The liver cyst decreased in size, but rapidly re-grew. The infant’s respiratory status was unstable, and she fed poorly, owing to compression by the hepatic cyst; therefore, we performed fenestration of the hepatic cyst when she was 43 days old. We performed laparotomy via an umbilical arc incision and punctured and aspirated the hepatic cyst contents (Fig. 3A). The cyst was abducted from the umbilical region, and the cyst wall was excised with an ultrasonic cutting and coagulation device (Fig. 3B). Laparoscopy confirmed that there was no liver injury or biliary fistula (Fig. 3C and D). She received a single-dose of cefazolin intravenously as a prophylactic antibiotic before surgery. Histopathologically, hepatocytes and connective tissue were found in the outer layer of the cyst, and dilated capillaries and lymphatic vessels were scattered throughout. Immunohistologically, the cells supporting the cyst showed the CKAE1/AE3, D2-40, calretinin, WT-1 were positive and CD34 and hepatocyte-1 were negative (Fig. 4). The diagnosis was mesothelial cell-derived hepatic cyst. After the surgery, there were no complications and no respiratory assistance was required. Breastfeeding was resumed on the first postoperative day. One year after the operation, CT showed regeneration of liver tissue (Fig. 5). We regularly follow her with ultrasonography and she has not had a recurrence for 3 years after her surgery. We show the umbilical wound 3 years after the surgery (Fig. 6).

Figure 1.

Liver cyst on prenatal ultrasonograhy at 30 weeks’ gestation. 54 × 33 × 55 mm cyst within the fetal liver (*).

Figure 2.

Liver cyst on CT image at 10 days after birth. The huge cyst located segment 5–6, without blood flow inside (*).

Figure 3.

Laparotomy and laparoscopic findings. (A) The cyst was drained at 16G indwelling needle. (B) We resected cyst wall from the umbilical incision. (C) The cyst wall had been sufficiently resected. (D) The volume of the left lobe of the liver was sufficient.

Figure 4.

Histopathological finding. (A) Hematoxylin and eosin stain. (B) Immunostaining of hepatocyte-1 (C) immunostaining of calretinin.

Figure 5.

CT image at 1 month after fenestration.

Figure 6.

Umbilical incision 3 years after surgery (arrow).

DISCUSSION

Congenital hepatic cysts in fetuses or newborns are relatively uncommon; however, routine ultrasound is detecting an increasing number of them prenatally [4]. Hepatic cysts are usually subdivided into parasitic and nonparasitic cysts. Parasitic cysts do not occur in fetuses. Nonparasitic hepatic cysts can also be classified as solitary or multiple. Multiple hepatic cysts may also occur in cases of congenital kidney and liver polycystic disease, congenital hepatic fibrosis. Solitary nonparasitic hepatic cysts are very rare, do not appear to communicate with the biliary system and are not associated with cystic lesions in other organs [5]. A solitary hepatic cyst should be considered in the differential diagnosis of a fetal or neonatal abdomen with a unilocular cyst. Laparoscopic excision of congenital hepatic cysts is a beneficial procedure in pediatric patients [3]. Laparoscopic-assisted hepatic cyst fenestration by umbilical incision can be performed safely and it is a cosmetically superior procedure. This procedure can be one of the options for the treatment of neonatal congenital hepatic cysts.

CONFLICT OF INTEREST

The authors declare no conflict of interest.

FUNDING

Authors of this article did not receive financial support.

ETHICAL APPROVAL

It does not require approval.

CONSENT

The patient’s parents signed an informed consent form.

GUARANTOR

Yuki Muta.