Figure 11.1 Clinical photograph of 5-week-old infant with abdominal distension due to bile ascites. Note the bile staining of his hydroceles and small umbilical hernia. (First published in black and white in Howard ER, et al., Archives of Disease in Children 1976, 51: 883–886.)

Endoscopic retrograde cholangiopancreatography (ERCP) can be diagnostic, although few centres possess the relevant endoscopes and expertise to perform it in the infants usually affected. Magnetic resonance cholangiopancreatography (MRCP) may become a more useful diagnostic tool, particularly with respect to defining loculations and the periperforation inflammatory mass due to technology advancements. Similarly, there is an expectation that newer imaging agents, such as Gd-BOPTA (gadoliniumbenzyloxypropionictetraacetate), when administered in conjunction with magnetic resonance imaging (MRI), can demonstrate dynamic liver function and the site of bile leak directly [17].

11.2.3  MANAGEMENT

In our opinion, laparotomy is always required to facilitate bile drainage and provide definitive treatment; however, there are reports of ‘simple’ external bile drainage alone [1,15,18,19].

Intraoperative cholangiogram via the gallbladder will show the site of the leak, the nature of the bile ducts and any distal obstruction. If this is the case with inspissated bile, for instance, then flushing with saline can also be achieved via the cholangiogram catheter. If the site of perforation is in the gallbladder or cystic duct, then simple cholecystectomy will be curative.

Not uncommonly, in the acute phase there can be significant inflammation around the porta hepatis, with ‘pseudocyst’ formation making definitive surgery challenging. This thin-walled pseudocyst must not be confused with the true wall of a choledochal malformation. Rather than cause further biliary injury, it may be advisable to simply place peritoneal drains, bring out the fundus of the gallbladder as a cholecystostomy (for latter cholangiography) and refer on [2,3,18,19].

If the perforation can be identified, then this could be sutured or patched directly as long as there is no distal obstruction. Alternatively, a small ‘T-tube’ can be placed (6 FG is usually the smallest available [2]. This should be left to drain freely as a controlled external biliary fistula for 2–3 weeks and then simply removed.

Sometimes, there is little structure left to the bile duct at the site of perforation, in which case a definitive biliary reconstruction needs to be considered, such as a Roux-en-Y hepaticojejunostomy [10].

There are advocates of a much more conservative approach, following the practice of the eminent American paediatric surgery John Lilly [1]. However, most reports show a protracted, sometimes stormy, clinical course of 6–8 weeks of ongoing external drainage, usually on parenteral nutrition, and even then it still fails to resolve satisfactorily [1,3,19]. Deaths [1,3], biliary strictures and portal vein thrombosis [3,18] have all been reported with this approach.

Some form of stenting has been described as an adjunct to this approach. Murphy et al. [19] described a case of a 14-week-old infant who had failed conservative management with an externally placed drain, and with the inability to achieve definitive reconstruction at the porta due to inflammation, the persistent leak was managed ultimately by a catheter placed externally via the intrahepatic bile ducts, traversing the perforation site and into the duodenum. This protracted course took 21 weeks from presentation to resolution.

In those children outside of the usual age range, ERCP and insertion of a transampullary biliary stent may be a more definitive therapeutic tool [20], as it is in adults. We have reported several successful examples where the bile duct injury was due to blunt abdominal trauma [21].

11.2.4  Complications

11.2.4.1  BILE DUCT STENOSIS

This is perhaps the most common complication [2,3] and is due to fibrosis and healing at the site of perforation. This may become complicated by biliary cirrhosis if ignored. Some infants might well present with obstructive jaundice initially after a relatively silent perforation. Indeed, this may be the mechanism for so-called acquired biliary atresia [22]. Such infants are usually older than those with typical biliary atresia at >100 days and often have had a more complicated neonatal history with necrotising enterocolitis (NEC), for instance. More proximal ducts, again, unlike those with more typical biliary atresia, will be dilated. Most infants will require formal biliary reconstruction with resection of the stricture or atresia and hepaticojejunostomy.

11.2.4.2  PORTAL VEIN THROMBOSIS

Portal vein thrombosis has been recently recognised as a possible sequel to bile duct perforation, and specifically in those arising from the posterior aspect of the duct [3,10,23]. Thrombosis is presumed to occur due to persisting direct exposure of the vein to bile, although its onset appears to be variable. This has been reported at the time of perforation, but it can often be delayed by several months. Portal hypertension and variceal formation may follow, although this is not invariable. It is therefore advisable to follow these children with serial US looking at portal venous flow and splenic size. It may also be beneficial to anticoagulate those few patients with a posterior perforation [10].

Chylous ascites has been reported, which is perhaps surprising as it does not usually follow similar portal dissections in biliary atresia or choledochal malformations [10]. This usually resolves with a period of drainage with either parenteral nutrition or medium-chain triglyceride therapy.

11.2.4.3  PERFORATION OF THE GALLBLADDER IN THE OLDER CHILD

There may be a number of causes for isolated or spontaneous acalculous perforation of the gallbladder outside infancy. Most seem to be associated with a more systemic viral infection, such as influenza, and are an unusual complication. Others are due to well-recognised complications but are unusual in this age group, such as typhoid fever^* [24].

11.3  MISCELLANEOUS ANOMALIES OF THE GALLBLADDER

11.3.1  Agenesis of the gallbladder

Gallbladder agenesis was first reported by Lemery in 1701. True isolated agenesis (i.e. not associated with biliary atresia) is surprisingly common, with an estimated incidence of 1 in 6300 [25].

It may make its first appearance antenatally, although what it implies can be quite complicated. Thus, Shen et al. [26] reported 21 cases of nonvisualisation of the fetal gall-bladder, of which 16 were isolated and 5 had additional, often severe malformations, such as trisomy 18. One fetus in the isolated group proved to have cystic fibrosis (CF), but the others appeared entirely normal to follow-up of 2 years. A larger series from Paris of just over 100 cases showed that about 25% were part of a malformation syndrome, 10% were associated with CF and 8% with biliary atresia [27].

Although most are asymptomatic and therefore diagnosed incidentally, some reports suggest that it can be a cause of right upper quadrant pain and describe the condition found during laparoscopic cholecystectomy following mistaken interpretation of a US [25]. Amazingly, authors report resolution of symptoms despite the lack of any actual intervention [25,28].

11.3.2  Left-sided gallbladder

This is rare and is defined as a gallbladder to the left of the falciform ligament. It was first described in three anatomical specimens by Hochstetter in 1886 [29]. In most examples, and certainly all the early ones, the cystic duct then passed below the ligamentum teres (round ligament) and still entered the CHD on the right side. Nagai et al. reviewed the literature (n = 105) and their own hospital experience, identifying 18 examples [30]. Sometimes, this can be confused with the situation of a ‘right-sided round ligament’, that is, embryological persistence of the right vitelline^† vein, together with an abnormal intrahepatic portal venous system [31]. In this, the ‘left-sided’ gallbladder actually sits relatively normal on the right.

A left-sided gallbladder can be an isolated entity or seen in combination with situs inversus, preduodenal portal vein, interrupted inferior vena cava and anomalous intrahepatic branching of the portal vein. The cystic artery usually crosses in front of the CBD from right to left. The cystic duct may join the left or right side of the CHD or may enter the left hepatic duct in isolation.

11.3.3  Double gallbladder

Duplication of the gallbladder has an incidence of about 1 in 4000 and occurs because of bifurcation of the gallbladder primordium during the fifth week of gestation [32]. There is no real basis for most of the proposed classifications [33], other than to say that the cystic duct, neck and fundus can all be duplicated to varying degrees. Recognition at the time of surgery mandates precise delineation with cholangiography of the connection with CHD. It is likely that at least one of the gallbladders will have some functional impairment and therefore predispose to stone formation. Whether to remove both gallbladders at the time of surgery is a moot point, and might depend on a preoperative radioisotope scan showing functionality.

Isolated case reports exist of the even less common triplicated gallbladders [34] – the same principles apply.

The so-called ‘Phrygian cap’^* appearance, also described by Boyden [35], is a variation with kinking of the fundus, sometimes simulating a mass or stone within. It again is surprisingly common and estimated at 4%, but has no real pathological significance [36] (Figure 11.2).

Figure 11.2 Duplicated gallbladders, both with Phyrgian cap anomaly. (Taken from Kannan NS, et al., Journal of Clinical Diagnosis and Research 2014, 8: ND05–ND06. doi: 10.7860/JCDR/2014/9057.4724.)

11.3.4  Multiseptate gallbladder

A multiseptate gallbladder is divided into smaller compartments by thin-walled membranes lined with columnar epithelium and was first recognised as an entity comparatively recently in 1963 by Simon and Tandon [37]. There is communication with each septum via small perforating holes. A muscular layer within each septum joining the outer muscular layer of the gallbladder may or may not be present. It can be seen in association with other ductal anomalies: gallbladder ectopy, choledochal malformations [38] and anomalous pancreaticobiliary junction. Three aetiological factors have been postulated: failure of the solid gallbladder to vacuolise; disproportionate rate of growth of the solid gallbladder in comparison with the investing peritoneum, resulting in bending and kinking; and, finally, simple variation in kinking and clefting of the gallbladder as it develops. Their external appearance varies from normal to bosselated, and typically they feel spongy, which when cut open shows a honeycomb of multiple cysts. Most reports, of course, have been in symptomatic adults or children with right upper quadrant pain. Increasing use of US will identify the incidental ones. Removal is probably unnecessary in the latter and will be curative in the former [39].

11.3.5  Porcelain^† gallbladder

This condition is characterised by abnormal calcification in the gallbladder wall. Its incidence in a large series of excised gallbladders is quoted at 1%–2%, but it is rarely reported in children [40]. Calcium is deposited within the muscular wall of the gallbladder and forms a rim of calcification or multiple scattered areas in the mucosa. Gallstones may be associated with the condition (up to 90%), and in the adult literature, incidence of malignancy up to 60% was once quoted, and this relationship gave it its clinical importance. More recent reviews downgrade this association, and the usual advice to remove the asymptomatic porcelain gall-bladder has been modified [41,42].

Paediatric examples are rare; Casteel et al. reported a 10-year-old girl with an incidental finding of a porcelain gallbladder on intravenous urogram. She was asymptomatic but still underwent an uncomplicated cholecystectomy [40].

11.3.6  Duct of Luschka and cholecystohepatic ducts

There is a whole range of peculiar variations in bile duct drainage related to the gallbladder. Figure 11.3 illustrates some of the possibilities.

Abnormal connecting ducts draining liver parenchyma and traversing the fossa directly into the lumen of the gall-bladder (cholecystohepatic ducts) or cystic duct (cystohepatic) can be defined [43]. The degree of liver drained is usually segmental, but it can be more with, in some cases, the CHD (or right and left hepatic ducts) draining into the gallbladder [43]. Exceptionally, the gallbladder acts then as the only bile conduit (because of an absent distal CBD), and drainage is via the relatively small and inefficient cystic duct directly into duodenum. We have previously described such a cholecystohepatic duct in two children (one male and one female) who presented with obstructive jaundice at 3 and 4 years of age, respectively. Interestingly, both had previously undergone successful correction of oesophageal atresia as neonates [44].

Figure 11.3 Hubert von Luschka (1820–1875) and schematic diagram illustrating possible abnormal ductular connections in the gallbladder fossa: (a) normal arrangement, (b) duct of Luschka, (c) cholecystohepatic duct and (d) aberrant insertion of right posterior duct into gallbladder (or cystic duct).

Hubert von Luschka ^* described an aberrant biliary duct found in the gallbladder fossa in 1863 [45]. This is a small bile duct running along the fossa between the gallbladder and liver parenchyma, unaccompanied by an artery or vein, which does not enter the lumen or connect the two [46].

The major clinical importance of these type of anomalies is that they may be damaged by cholecystectomy and be a cause of a postoperative bile leak. Identification of any aberrant bile leak from the bed is important and should be clarified by intraoperative cholangiography. If recognised and sufficiently small, then it may simply be oversewn [46,47].

11.4  BILE DUCT STRICTURES AND STENOSIS

Table 11.1 illustrates the main causes of biliary obstruction in children. Malign strictures (e.g. rhabdomyosarcoma and cholangiocarcinoma) are considered in Chapter 18. Mirizzi’s syndrome^† (i.e. external compression of the CHD) is considered in Chapter 17 as the most common cause and is the impaction of a gallstone in the cystic duct or Hartmann’s pouch^‡

Table 11.1 Causes of extrahepatic biliary tract obstruction

Note: LCH, Langerhans cell histiocytosis.

Strictures in the extrahepatic biliary tree may occur at any anatomical point, but are most commonly seen in the CBD. Children will present with a gradual onset of conjugated hyperbilirubinaemia, pale stools and intermittent abdominal pain and are at risk of cholangitis. Diagnostic modalities include US scan (USS) (may demonstrate dilated proximal intra- and extrahepatic bile ducts). For more definitive outlining of the anatomy, a cholangiogram should be performed. MRCP is possible, but in neonates and infants lacks the anatomical definition required for correct diagnosis and surgical planning. A percutaneous transhepatic cholangiogram (PTC) or ERCP can be performed. These modalities, if successful, have an added benefit of being therapeutic if a percutaneous transhepatic biliary catheter (following PTC) or a transampullary stent (following ERCP) can be left in situ. Limiting factors for ERCP are the size of infant or child and indeed the position of the stricture, because if it is very distal, then cannulation of CBD may not be possible. Management is via either stenting and dilatation or resection and biliary reconstruction typically using a Roux-en-Y hepaticojejunostomy loop.

11.4.1  Primary and autoimmune sclerosing cholangitis

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