|Year : 2016 | Volume
| Issue : 2 | Page : 72-74
Giant congenital solitary nonparasitic cyst of the liver causing respiratory distress in a neonate
Minakshi Bhosale, Dasmit Singh
Department of Pediatric Surgery, BJ Government Medical College, Pune, Maharashtra, India
|Date of Web Publication||18-Feb-2016|
G/101, Sudarshan Apartments, Behind Spencer's Daily, Karve Nagar, Pune - 411 052, Maharashtra
Source of Support: None, Conflict of Interest: None
| Abstract|| |
A 3-day-old male neonate delivered at 34 weeks of gestational age was brought with breathing difficulty since birth. The abdomen was massively distended. A soft cystic mass was occupying almost the entire abdomen and causing obvious respiratory distress. On exploration, a huge, solitary, unilocular cyst was found between the two lobes of the liver. Growing extrahepatically between the two lobes, it had displaced them laterally on either side. Enucleation of the cyst and marsupialization of its base was done. Histopathology showed evidence of congenital solitary nonparasitic cyst of the liver. Symptomatic presentation of CSNCL in children, especially in a neonate is extremely rare and not considered as a differential diagnosis of an abdominal mass. Hence, the case report.
Keywords: Abdominal mass, congenital solitary nonparasitic cyst of liver, neonate, respiratory distress
|How to cite this article:|
Bhosale M, Singh D. Giant congenital solitary nonparasitic cyst of the liver causing respiratory distress in a neonate. J Indian Assoc Pediatr Surg 2016;21:72-4
|How to cite this URL:|
Bhosale M, Singh D. Giant congenital solitary nonparasitic cyst of the liver causing respiratory distress in a neonate. J Indian Assoc Pediatr Surg [serial online] 2016 [cited 2018 May 21];21:72-4. Available from: http://www.jiaps.com/text.asp?2016/21/2/72/161032
| Introduction|| |
The majority of the congenital solitary nonparasitic cysts of the liver (CSNCLs) are found in adults, are asymptomatic and detected incidentally at autopsy or laparotomy.  Their presence has been reported in <1% of routinely performed autopsies in adults.  However, they (CSNCLs) are quite rare in infants and children.  The symptomatic presentation is even rare.
| Case report|| |
A 3-day-old male neonate delivered vaginally at 34 weeks of gestation was brought with progressive abdominal distension since birth. His birth weight was 2.2 kg. The baby had cried immediately after birth. On examination, the cry, tone, and activity were normal for the gestational age. He had obvious respiratory distress with a respiratory rate of 80/min. The abdomen was massively distended [Figure 1]. He had a vaguely demarcated, palpable lump, occupying almost the entire abdomen. It was compressible in nature and soft cystic in consistency. The baby had passed urine and meconium. The mass was not detected antenatally. Maternal (antenatal) history was not contributory.
|Figure 1: Clinical photograph of the neonate showing massively distended abdomen|
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Erect X-ray abdomen showed bowel loops displaced to left hypochondrium. Ultrasonography of the abdomen showed a solitary, large cystic lesion 9.7 cm × 6 cm × 9.4 cm in size, incumbent on the left and right lobes of liver extending from the epigastrium to the pelvis. However, the origin of the mass was uncertain. It was causing displacement of the bowel, liver and right kidney. Basic hematologic investigations were normal. Computerized tomography scan of the abdomen was not done since the child's condition was quite unstable.
The working diagnosis was bowel duplication cyst or a lymphangioma. Hence, the decision was taken to explore the child. On exploration, a huge, unilocular, thin-walled, transparent cyst almost prolapsed out [Figure 2]. It was 15 cm × 12 cm ×12 cm in size. It was located on the right and left lobes of the liver. Growing extrahepatically, it was displacing them on either side and had completely replaced the falciform ligament and the ligamentum teres. The gallbladder was normally located and well-distended. The small and large bowel, spleen, and kidneys were normal. Enucleation of the cyst along with marsupialization of the portion within liver parenchyma was done. Careful assessment of the hilar structures was done after dealing with the cyst. There was no obvious dilatation of the hepatic or bile ducts. Blood loss of ~30cc was replaced on the table. ~600 cc of amber colored fluid was drained from the cyst. It turned into jelly-like consistency on exposure to atmospheric air. Fluid cytology showed the presence of albumin; but no bile salts, pigments or urobilinogen. The baby was stable for 36 h postoperatively. However, thereafter he succumbed to death. The histopathology of the excised cyst showed the cyst wall composed of loose connective tissue with many thin-walled dilated blood vessels, few clusters of lymphocytes, and bile ducts. The cyst wall was lined by columnar epithelium, and there was sparse mononuclear cell infiltrate; suggestive of a CSNCL.
|Figure 2: Operative photograph showing the huge prolapsed cyst between liver lobes|
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| Discussion|| |
Congenital solitary nonparasitic cysts of the liver lack uniform nomenclature. Different reports describe this entity using various names such as simple liver cysts,  congenital hepatic cysts,  solitary nonparasitic cysts of liver,  congenital solitary liver cysts,  and CSNCL. , This could be because of the rarity of the lesion and calls for uniform nomenclature worldwide in order to know the exact incidence of these cysts.
The term solitary cyst refers to unilocular cysts that are typically single and are unassociated with cysts in other organs.  Studies indicate that these cysts arise from congenital or secondary obstruction of the peribiliary glands, which normally arise from the ductal plate at the hepatic hilum around 7 th week of gestation and continue to proliferate until adolescence.  Persistence of small remnants of the ductal plate malformation (Von Meyenburg complexes) that separate out from the biliary tree and become dilated are supposed to give rise to CSNCLs. They are generally lined by a single layer of cuboidal or columnar epithelium, characteristic of the bile ducts.  The cyst fluid is typically clear or amber colored and rarely contains bile. , Most cysts are superficially located just under the liver capsule, although they may be found in the falciform ligament.  The majority of them are asymptomatic and are managed by simple unroofing, marsupialization, sclerotherapy or laparoscopic fenestration. ,,
Stehr and Guzzetta have described CSNCLs to be rare and occurring more commonly in adults than in children. They have observed males and females being equally affected,  whereas few other authors have reported female preponderance.  Shankar et al.  and Nordin et al., Quillin and McAlister, Chatterjee et al. ,, have presented isolated cases of neonates and infants of CSNCL detected antenatally and managed successfully in the postnatal period. Rygl et al. have reported review of six children (four girls and two boys) with congenital solitary liver cysts. The age range in their series was from the neonatal period to 14 years. 
In our case, there was a unilocular, thin-walled, transparent cyst of 15 cm × 12 cm × 12 cm in size. It originated between the right and left lobes of the liver and was overhanging both the lobes. Growing extrahepatically, it was displacing them on either side and had completely replaced the falciform ligament and the ligamentum teres. The absence of the falciform ligament and remnants of the ligamentum teres also assert its congenital origin. In this neonate, the working diagnosis was that of bowel duplication cyst or a lymphangioma. Hence, the decision was taken to explore the child. In most of the reports presented, the cases were diagnosed and confirmed on ultrasonography. Rygl et al. have described ultrasonography to be conclusive for diagnosis of CSNCL in children.  In our case, though cyst in relation to the liver was detected on ultrasonography, the diagnosis of CSNCL was totally unsuspected. CSNCL was diagnosed intraoperatively.
Rygl et al. have reported the presence of columnar epithelial lining in newborns; whereas atrophic changes were found in the lining epithelium in older children. They also found the presence of metaplastic squamous epithelium with dysplastic foci in the lining epithelium in a 13-year-old girl.  Furthermore, there is the possibility of adenocarcinoma or adenosquamous carcinoma.  Hence, surgical therapy should be undertaken, even though, the child is asymptomatic.
The differential diagnosis of the abdominal cystic lesion in neonates includes mesenteric cyst, duplication cyst, hepatoblastoma, teratoma, lymphangioma, choledochal cyst, and renal cyst. Though rare, CSNCLs should also be considered as differential diagnosis, especially in cases of cystic masses in the proximity of the liver. Acute surgical crisis in neonatal period occurs because of diaphragmatic splinting and respiratory embarrassment caused by giant CSNCL as in our's, and early relief of the same might be life-saving. If widely known, simple ultrasonography-guided aspiration of these cysts in unstable neonates may prevent unnecessary laparotomy in unprepared emergency situations and salvage the neonate.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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[Figure 1], [Figure 2]