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REVIEW ARTICLE Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 4  |  Page : 461-472
Foregut cystic developmental malformation: New taxonomy and classification - Unifying embryopathological concepts

1 Methodist University Hospital, Memphis 38103, USA
2 Department of Transplant Surgery, Memphis 38104, USA
3 Section of Hepatology, Division of Gastroenterology, Department of Medicine, Room S1008, 10 Thomas, Methodist University Hospital, Memphis, TN 38104, USA
4 Department of Transplant Surgery, 1265 Union Avenue, 10 Thomas, S1003, Memphis, TN 38104, Nosratollah Nezakatgoo, M.D., F.A.C.S, USA
5 Transplant and Hepatobiliary Services, Nazih Zudhi Transplant Institute, Integris Baptist Medical Center, Oklahoma City, Oklahoma 73112, USA

Click here for correspondence address and email

Date of Web Publication1-Oct-2009


Foregut cystic developmental malformations are rare developmental anomalies. The problems inherent to these malformations are their presentation across specialties that include embryology, anatomy, pathology, thoracic foregut surgery, pediatric surgery and general abdominal surgery. The direct consequence of this variation has resulted in diverse terminology, classification and a failure to identify the correlation. The article aims to summarize and unify the embryological concepts of foregut cystic malformation, to suggest a generic title to the various groups of these interrelated disorders and a uniform use of nomenclature on the basis of unifying concepts of embryopathogeneis.

Keywords: Bronchogenic, bronchopulmonary, cyst, duplication, enteric, foregut

How to cite this article:
Sharma S, Nezakatgoo N, Sreenivasan P, Vanatta J, Jabbour N. Foregut cystic developmental malformation: New taxonomy and classification - Unifying embryopathological concepts. Indian J Pathol Microbiol 2009;52:461-72

How to cite this URL:
Sharma S, Nezakatgoo N, Sreenivasan P, Vanatta J, Jabbour N. Foregut cystic developmental malformation: New taxonomy and classification - Unifying embryopathological concepts. Indian J Pathol Microbiol [serial online] 2009 [cited 2022 Dec 9];52:461-72. Available from:

   Introduction Top

Foregut cystic developmental malformations are rare developmental anomalies which are being increasingly reported in the recent years. We recently encountered a case of the ciliated hepatic foregut cyst and during our review of the literature found a lot of variations in terminology, classification and the suggested etiology for these entities. A problem inherent to these malformations is their presentation across specialties that include embryology, anatomy, pathology, thoracic foregut surgery, pediatric surgery and general abdominal surgery. The direct consequence of this variation is that during the ensuing years all publications dealing with these apparently dissociated malformations resulted in diverse terminologies, classifications and failed to identify the correlation.

The purpose of this article is to summarize and unify the embryological concepts of foregut cystic malformation, suggest a generic title to the various groups of these interrelated disorders and a uniform use of nomenclature on the basis of unifying concepts of embryopathogeneis.

Data sources : A Medline, Pubmed database search was performed to identify the articles using the keywords "foregut cyst", "duplication cyst", "enteric cysts", "bronchogenic cyst", "neurentric cyst", "esophageal cyst," "gastrointestinal cyst", "foregut duplication" and "bronchopulmonary foregut malformation". All the relevant articles were retrieved and additional cross references further searched and identified manually. Articles published before 1966 were searched and procured using the INTEGRIS health library in Oklahoma City.

   Discussion Top

After reviewing close to 200 original articles and reviews on foregut cyst, bronchogenic cysts, duplication cysts, enteric cysts, esophageal cysts and bronchopulmonary malformation, we found considerable controversy in the nomenclature, classification, and proposed embryogenesis of these foregut developmental errors. Various terms like gastric cysts, epithelial cysts, esophageal cysts, enteric cysts, bronchogenic cysts, foregut cysts, esophageal duplication cysts, cyst of foregut origins, bronchopulmonary foregut cystic malformations, cyst with intestinal epithelium, biliary cysts, congenital duplication cysts, cystic duplications, choriostoms, and enterocytoma have been used interchangeably adding confusion to the available literature. [1],[2],[3],[4],[5],[6],[7] We propose to introduce classification and nomenclature for these rare cystic disorders.

Historically, cysts of foregut origin have been classified into enteric cyst, bronchogenic cyst, and esophageal duplication cyst (some defined it as enterogastric cyst). [8],[9],[10],[11],[12],[13]

This classification has number of drawbacks and does not include the place for the foregut cyst which is a distinct entity and can have varied presentation. The distinction between enteric cyst and esophageal duplication cyst is even more perplexing in this classification as both the entities have the same histological appearance; the only difference being the location and presence or absence of the duplication. [14] It is interesting to note that various authors described the same entity and gave it different names based on location of the cyst.

Furthermore, traditional nomenclature uses the term duplication cyst as a distinct histological entity irrespective of the location of the cyst or its relationship with the bowel wall, some authors even used it for enteric cysts irrespective of location. Various terms like mediastinal duplication cyst, completely isolated duplication cysts, retro peritoneal duplication cysts and even intrabdominal esophageal duplication cysts, used by various authors do not convey anything as far the histology or the embryology of these cysts. [6],[11],[15],[16],[17],[18] Terms like these have diluted the meaning of duplication as an entity and the true definition of the duplication cyst has been lost. [19] Terms like true duplication of the bowel and duplication cysts have been equated and used interchangeably despite the fact that these two are distinct entities with completely different macroscopic, microscopic picture, clinical feature, management and an entirely different embryogenesis. [18],[20],[21]

The traditional description of the term enteric cyst in literature suggests that they are invariably closely associated with the esophagus although they can be dissected readily, have a muscular layer and a lining which consists of the alimentary tract epithelium. [14] This definition is far from complete as these cysts are not unique to the esophagus and can be seen in relation to any portion of the gastrointestinal tract and rarely as a completely isolated entity in the abdomen.

Another fallacy of the present classification is lack of place for the complex cystic malformation containing both respiratory and enteric elements. These cystic malformations have been repeatedly labeled enteric, foregut, bronchogenic or duplication cysts by various authors. [18],[20],[22],[23],[24],[25],[26]

   Embryogenesis Top

During differentiation in the third week of fetal development the foregut undergoes dichotomy and by the fourth week a primitive diverticula arises from the ventral part of the cranial foregut. Two laryngotracheal ridges form between the diverticula and dorsal foregut and later fuse together to become laryngotracheal septum. This septum divides the primitive foregut into a dorsal esophagus and the ventral respiratory laryngotracheal tube. The dorsal tube goes on to develop esophagus, stomach, duodenum, liver, gall bladder and the pancreas. [33] The ventral tube forms a primitive lung bud at its caudal end which differentiates by the seventh week to form bronchopulmonary buds and later the lungs by repeated bifurcation and differentiation. [33],[34]

While the underlying basis of the foregut and bronchogenic cysts have been hypothesized to be abnormal pinching of the buds from the portion of the foregut destined for the respiratory differentiation, the source of the bronchopulmonary foregut malformation is suggested to be the accessory lung bud. [13],[35] The formation of enteric cysts, duplication cysts and true tubular duplications have been attributed to the various theories including neurentric canals, split notochord, persistent vacoulation theory, accessory lung bud theory, presence of the mucosal diverticula or the faulty separation of the endoderm and notochord, early in the development.

However, the main drawback with these theories is their inability to satisfactorily explain all the malformations seen. Bentley and Smith [36] in their land mark article described the concept of the split notochord and found a spectrum of disorders that share a common embryogenesis and gave the term the posterior enteric remnant. [36] They identified a common embryogenesis for the posterior enteric fistula, posterior enteric diverticula, posterior enteric cyst and sinus and differentiated them on the basis of the severity of the malformation. [36] The underlying basis of this theory was frequent observation of the association of vertebral anomalies in the form of bifid or fused spines and thoracoabdominal duplications. [36],[37],[38],[39],[40] In some cases duplications were found attached to the vertebral column, further supporting this theory.

The split notochord theory has also been conclusively proven and has experimentally reproduced in the amphibian embryo. [36] The underlying inciting event for the attachment or herniation of the endoderm through the split notochord is debatable and various hypotheses including the accessory neurenteric canal theory. primary adhesion theory, duplicated notochord theory have identified various initiating events for the process. [37],[38],[41],[42]

An amphibian embryo model suggested that a dorsal midline fistula, induced experimentally, can induce partial separation of the notochord and subsequent herniation of the gut anlage through the split elements of the notochord. [43] The authors suggested that the fistula provides an open passage between the amniotic cavity and yolk sac and bears resemblance to the neurentric canal. [43] It is now widely accepted that the failure of the obliteration of this pathological neurenteric fistula results in formation of the foregut cystic malformations associated with vertebral anomalies and are located posteriorly, anywhere between the plane of the bowel and the vertebral column. [36],[37],[44] The factors responsible for the formation of ectopic neurentric canal are not clear yet but it is unrelated to any hereditary, chromosomal or pathological process.

The main drawback of this theory is its inability to explain the presence of the heterotrophic mucosa in true duplication and the absence of the vertebral and spinal cord in 20-60% of these cases. Some of the cysts were located distinctly remote from the spinal column and anterior to the bowel wall or on the antimesentric border further confirming the fact that more than one theory is at work in these complex malformations.

Split notochord theory can explain only those forms of posterior enteric cyst, duplication cysts located on the mesenteric border or when the duplications are associated with vertebral anomaly and is called split notochord syndrome [36] . However, it cannot explain complete duplication of the esophagus or stomach especially or when duplication does not have any relationship with the vertebral column (either close proximity or attachment). [45]

The unifying embryological concepts were first put forward by Heithoff et al[27] in which the authors tried to explain all the foregut cystic malformation on the basis of the "supernumerary lung bud theory". This theory was based on sold concepts laid down after observation of various intermediate embryologic stages displaying the involutionary stages of the supernumerary lung bud.

Accessory lung bud theory was also observed in the experimental Adriamycin rat model by two authors. They described their pathogenesis to the tracheo esophageal fistula. However, it was not substantiated by other authors. The chief drawback of this concept is that it is not uniformly reproducible in experimental animals and fails to explain neurenteric cysts and various spinal malformations commonly associated with these disorders.

The only way to prove a theory of embryogenesis is to induce the anomaly on the laboratory animal and observe the proposed mechanism. Short of an animal model, such a theory cannot be proven in totality but its validity can be ascertained by comparing as to what can be predicted and what is actually seen in these rare cases.

We propose to include all these developmental anomalies under the heading of the foregut cystic malformation as they all share a common origin from foregut and differ with each other in the site of origin, extent, migration and the degree of differentiation. [27],[28],[29],[30],[31],[32]

The underlying embryogenesis being the abnormal budding, can arise from one of the three important sites which include enteric portion of the foregut (dorsal), respiratory portion of the foregut (ventral) or from accessory lung bud (which usually occurs caudal to the normal lung bud) [Figure 1].

   Enteric Cysts Top

Pinching of the abnormal bud from the portion of the foregut destined for enteral differentiation will result in the formation of the enteral cyst. Posterior wall of the dorsal foregut is initially fused with the notochord primordium to separate later. [38] Failure of the notochord to separate from the dorsal foregut or abnormal herniation of the endoderm through the split notochord may result in the neurenteric cyst. [36],[38],[43] These cysts, though uniquely named, are embro pathologically enteric cysts with additional features of either vertebral anomalies and spinal cord malformation or their persistent attachment to the vertebrae. These associated anomalies are present in about 40-70 %of the cases. [46],[47],[48] The presence of the neural tissue within the cyst wall is not required for making their diagnosis [Figure 2].

The enteric cysts can be lined by ciliated columnar, squamous, gastric or intestinal epithelium alone or varying degrees of combination only when embryologically adjacent epithelium is present as the cyst lining.

Foregut cyst can be differentiated from the ciliated columnar epithelium lined enteric cyst (arise from the area of the foregut giving origin to the proximal esophagus) by the absence of the two distinct layers of smooth muscle layers in the former. [11] The presence of the two muscle layers in enteric cysts represent an embryologically futile attempt to differentiate into outer longitudinal and inner circular layer as in the gut. [11]

In addition, the foregut cyst shows positive reactivity to anti CC10 antibodies, which is a secretory product specific for Clara cells, which are unique to the respiratory and terminal bronchioles. [49]

The presence of the endocrinal cells in the wall of the foregut cysts similar to the respiratory epithelium is further confirmed by showing the positive immunoreactivity to the chromogranin, synaptophysin, bombesin and calcitonin, which again are unique to the respiratory epithelium. [49] A combined presence of the ciliated columnar epithelium and any other epithelium like gastric, intestinal or even pancreatic tissue in the cyst wall suggests bronchopulmonary foregut cystic malformation.

Cysts showing the combination of the ciliated columnar epithelium and squamous epithelium deserve special mention because these epithelial linings have an adjacent location in the esophagus and may represent an enteric cyst having its origin from the portion of the dorsal foregut destined for the esophageal differentiation. These cysts can be differentiated using immunohistochemical stains which will characterize the embryological origin of the ciliated columnar epithelium. When ciliated columnar epithelium belongs to the lung tissue these cysts should be called bronchopulmonary foregut malformations and if the columnar epithelium belongs to the esophagus these should be called enteric cysts.

The type of epithelium present in the cyst is dictated by the site of origin of the cyst on the foregut. In addition to the epithelium layer there is always presence of two distinct layers of smooth muscle layers in the wall of the enteric cyst.

The subsequent fate of the enteral cyst depends on whether it is incorporated - in the wall of organs of foregut including the esophagus, stomach, duodenum, liver pancreas, gall bladder (organs of close proximity) or remains detached.

Isolated enteral cysts are the detached variety and can migrate to different regions of the body. They are commonly located on the right side of the posterior mediastinum; in fact the presence of the right sided posterior mediastinal mass along with the vertebral anomaly suggests the diagnosis of enteric cyst unless proven otherwise. [50] Their isolated intrabdominal location is extremely rare and only four such cases have been described in literature. [15],[16],[17],[51] The enteric cysts located in the posterior mediastinum or in plane posterior to the bowel suggest action of split notochord embryogenesis (pinching of the buds occurs from the posterior wall of the foregut) while those lying in the anterior mediastinum or in a plane anterior to the bowel suggest spontaneous pinching of the buds from the portion of the foregut destined for enteric differentiation (in the absence of notochord defects).

Its essential histological features include the presence of entral foregut epithelium and two distinct layers of smooth muscle layer. These detached enteric cysts should be called isolated (name of the site) enteric cyst with type of differentiation as dictated by the presence of the epithelium as the suffix. Often these enteric cysts gets incorporated in the wall of the hollow viscous (the more common fate) especially the part of the gastrointestinal tract developing from the foregut when these should be called as enteric duplication cyst. The lining of the enteric duplication cyst may or more often may not match with the site of the cyst in the GIT. [52] The presence of the heterotrophic mucosal epithelium, like the presence of enteric cysts located in the mediastinum and lined by pancreatic or/and gastric tissue, can be very well explained by our unifying theory of embryopathogenesis. [53],[54],[55],[56],[57]

   Duplication Cysts Top

Reginald Filtz [58] first used the term intestinal duplication in 1844 and since then literature mainly consisted of a few case reports and small case series. [58]

Gastrointestinal duplications are rare congenital malformations occurring anywhere from the mouth to anus and have varied clinical manifestations depending on the site of occurrence. [39],[59] Histologically they are defined according to the strict criteria laid down by Ladd and Grossa [14] and later reinforced by Parker et al.[60] These include: their close proximity to the gastrointestinal tract, lining which resemble some part of the gastrointestinal tract, an outer smooth muscle layer which either shares the muscle wall with the gut or is intermingled with the muscular coat of the bowel. [14],[60]

The incidence of gastrointestinal duplication has been reported to be 1:4500, [61] out of which foregut duplication constitutes 33%. [62],[63] These cysts vary widely in size and can be spherical or tubular in macroscopic appearance. [64] Spherical forms are the commonest accounting for 80-90% of the cases, located on the mesenteric border of the bowel wall and do not communicate with the bowel as opposed to the tubular cysts. [65],[66] We believe there is an inherent difference between the two entities. Spherical duplication cysts are the product of the foregut cystic malformation and basically enteric cysts which after their abnormal pinch off from the foregut get incorporated in the wall of the developing gastrointestinal tract. Rarely can they be histologically foregut, bronchogenic, or BPFM in nature. [67] This theory not only explains the presence of the heterotrophic mucosa but also any combination of the epithelial lining within the cyst. Mesenteric surface of the bowel is earliest to develop and exposed for the longest time period to these pinched off migrating buds; subsequent incorporation in the wall of the gut results in duplication cysts. They are embryologically distinct structures and hence can be excised or shelled out surgically without resorting to bowel resection if size is not extensive. [68]

Tubular duplication on the other hand is true duplication of the gut and should not be called a cyst. In fact it is a blind loop of the gut as it tends to resemble the bowel both macro and microscopically, lie along the antimesentric border and tend to have communication with the bowel lumen. Their excision usually entails resection of the affected bowel as they share a common blood supply with the gut. We believe that no single theory can explain the occurrence of the true duplication of the foregut derivatives, rather a combination of the theories are in play.

Tubular duplications of the foregut associated with widespread intestinal duplication and other anomalies suggest the concept of abortive twinning as it applies to the hindgut and midgut, while at the same time the foregut tubular duplication possessing attachment to the spine or the associated presence of the vertebral or spinal cord anomaly signifies the work of split notochord theory. [68],[69] True duplications occurring in relation to the midgut and hindgut are more likely to be a consequence of abortive twinning as these are frequently associated with genitourinary duplication and lack vertebral anomaliesl [64],[69] [Figure 3], [Figure 4]

Over the years this definition of duplication cysts got diluted and various authors started using the term in different context. Some reports classified these duplication cysts into foregut, mid gut and hind gut depending on the site of the attachment of malformations on the gastrointestinal tract. [70],[71],[72] .Others used the term for any cystic lesion showing enteric differentiation irrespective of the location and proximity or attachment to the bowel. Some authors even used the term for the any lesion showing a mixture of respiratory and enteric epithelium.

We believe that the term duplication can be used for any cyst including foregut, bronchogenic or BPFCM provided the standard definition of the Ladd and Gross [19] is adhered to. The term duplication cyst should be used strictly to the structures (only cysts not for true duplications which are blind loops) lying on the wall of the gastrointestinal tract usually along the mesenteric border, sharing a common musculature, blood supply and an absence of any communication with the lumen of the bowel. [19] .

The nomenclature for these duplicated cysts should include the site as the prefix and the type of differentiation of the cyst as dictated by the epithelium as the suffix and duplication in the middle. On the basis of differentiation of the epithelium; the duplication cyst can be foregut in nature when the epithelium is PSCE or enteric in nature (which is the commonest) when the epithelium displays squamous or gastric, intestinal or pancreatic tissue differentiation or bronchogenic in nature when the characteristic histological feature of the later are present [Figure 5].

It is possible to encounter duplication cysts showing the combined presence of enteric epithelium (one of the various types) and PCCE (pseudo stratified columnar epithelium) in the lining. [20],[24],[73] These cysts are the result of bronchopulmonary foregut malformation and should be named with site as prefix followed by BPF differentiation as the suffix.

   Bronchopulmonary Foregut Malformation Top

The term bronchopulmonary foregut malformation (BPFM) was first used in 1968 by Gerle et al[74] to describe 13 rare cases of pulmonary sequestration that had gastrointestinal communication. [74] The concept was subsequently refined by Heithoff et al[27] in the landmark article in 1976.

Both these authors proposed the underlying basis of BPFM as the presence of the accessory lung bud which occurs distal to the normal lung bud. The accessory bud from the ventral foregut grows and communicates with the regions of the dorsal foregut destined for enteral differentiation. The type and the severity of the BPFP is the result of various factors including the extent of differentiation in the accessory lung bud, stalk and the point of entry of the accessory tube in the dorsal foregut, its growth, degree of involution of the stalk connecting the dorsal and ventral foregut, presence or absence of the pinching of the entire complex and extent of migration of the complex. [27],[28],[29],[30],[31],[32]

The whole spectrum of cystic changes can be seen in both sub and supradiaphragmatic areas. The underlying factors for the all these variables had been explained by Clements and Warner in their unifying concept theory. [75]

Other subtypes of BPFM mainly located in the chest, which can be explained by the unifying theory, include the Pulmonary sequestration (intra and extralobar), Bronchogenic cysts, CCAM (congenital cystic adenomatoid malformation), CLE (congenital lobar emphysema), tracheal and esophageal divetricula, tracheal stenosis, tracheo esophageal fistula, pulmonary agenesis and hypoplasia. [27],[74],[75],[76],[77],[78],[79],[80],[81],[82]

In the past, various theories have been proposed to explain the embryological basis of foregut anomalies. These were broadly divided into theories of esophageal occlusion, tracheoesophageal septal deviation, and mechanical theories. However, after the development of the Adriamycin rat model for the esophageal atresia, and the TOF, most of the above theories have become obsolete [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10].

Since the introduction of the rat model in the 1978, more than 65 articles were published highlighting the various aspects of the Adriamycin induced foregut malformation. [83] The Adriamycin rat model studied by Crisera et al[84] showed, what they described as an "offspring "or a "third bronchus". This was in their opinion the fistula between the trachea and the esophagus, as they further showed the growth of this tract from the bronchial bifurcation and subsequent fusion with the dorsal foregut. These observations were further confirmed by the Spilde et al.[85] However, there were many subsequent studies which failed to demonstrate this kind of growth in the rat model. We believe the Adriamycin model is far from being complete, as the administration of this drug has a global effect on the proliferating cells especially large areas of intestinal atresia which are not part of the spectrum of the VACTER malformation associated with TOF.

In the subdiaphragmatic location these BPFM malformations should be called BPFCM (bronchopulmonary foregut cystic malformation). They may occur as isolated cysts when found alone or embedded in a solid organ and should be called isolated BPFCM may also present as duplication cyst when they lie in the wall of the hollow gastrointestinal tract. The underlying basis for their presentation as a duplication cyst is the abnormal pinching of the whole complex of accessory lung bud with a portion of dorsal foregut and subsequent incorporation in the wall of the developing gastrointestinal tract similar to foregut and bronchogenic duplication cysts.

Depending on the degree of the differentiation of the lung bud it is possible to see bronchogenic feature, foregut features or the presence of the lung tissue as the respiratory components of these cysts. [22],[32] The gastrointestinal elements may vary from esophageal, gastric, pancreatic tissue or intestinal lining, either alone in varying degree of combination depending on the site of the dorsal foregut where the accessory lung bud meets it.. [22]

Subsequent courses of these malformations depend on whether these complex cystic entities get pinched off or remain attached. A review of the cases in the literature suggests that more often than not these cysts get pinched off and may or may not migrate to different locations. [25] Their subphrenic location is explained by the course taken by these cysts which is exactly similar to the foregut or bronchogenic cyst.

In the context of the sub or supradiaphragmatic cystic lesions presence of tissue showing varying degree of ventral foregut differentiation (PSCC epithelium as the lining or presence of the cartilage tissue / bronchial glands in the wall of the cyst, lung tissues showing varying degree of differentiation as part of the wall) along with any component of the dorsal differentiation (presence of gastric, intestinal, pancreatic epithelium in the lining) should be labeled BPFCM (bronchopulmonary foregut cystic malformation). [27],[30],[32],[74], [86],[87],[88],[89],[90],[91] Depending on the presence or absence of involution of the connecting stalk these malformation may have persistent communication with the enteric or respiratory tract or they may be found completely isolated if the stalk involutes on both the ends. [26],[27],[28],[29],[30],[31],[32],[75],[79],[90],[92],[93],[94],[95]

A review of the nomenclature in literature shows that that majority of the bronchopulmonary foregut cystic malformations are either labeled as foregut cyst, isolated duplicated cyst, enteric or bronchogenic cyst. [18],[20],[23],[24],[25]

   Bronchogenic Cysts and Foregut Cyst Top

These are again part of the spectrum of the foregut cystic malformation. The two types of cysts share considerable similarities in their origin, migration and entrapment in various organs of the body. The traditional embryological theory explains their origin to the abnormal pinching of the normal lung bud. [35] We propose their origin to either abnormal pinching of the caudal portion of the lung bud or detachment of the accessory lung bud. The basic difference between bronchogenic cysts and foregut cysts is the timing of abnormal pinching. [49] In bronchogenic cysts the pinching occurs at the time of bronchial differentiation when cartilage tissue is present while foregut cysts derive from pinching of the bud at a later time of bronchiolar differentiation and so, the absence of the cartilage. [49]

Histologically, wall of the foregut cysts consists of four layers; the innermost being the columnar or cuboidal epithelium containing ciliated and mucous cells, subepithelial connective tissue followed by smooth muscle layer and an outer fibrous layer. [35],[49],[96],[97],[98],[99] Bronchogenic cysts have the similar microscopic stricture as foregut cysts but, in addition, they contain cartilage tissue and glandular tissue in the wall of the cyst.

These cysts may or may not get pinch off from the developing lung this result in their presence or absence of communication with the tracheobronchial tree. Cases have been described where these cysts tend to show persistent communication with the lung and appear like cystic cavities with an air fluid level. [100],[101],[102]

Subsequent to their formation, the course depends on whether they remain isolated or more frequently get incorporated into various nearby developing organs. The proximity of these cysts during development to the foregut and pharyngeal arches may result in their extraordinary locations. [2],[3],[67],[103],[104],[105],[106],[107],[108],[109],[110],[111],[112],[113]

The location of the cyst in the thorax is dictated by the timing of the abnormal pinching of the bud. When abnormal pinching occurs in the early embryonic period the cyst is paratracheal in position otherwise they are more peripheral within the lung parenchyma. [102]

Their atypical positions in the neck can be explained by their trajectory through a plane parallel to the trachea that results in the deep bronchogenic cyst of the neck or a plane which travels through the ununited sternal bars before its fusion at ninth week, and results in their subcutaneous position in the neck.

Their intrabdominal (both intra and retroperitoneal) location had been explained by the presence of two patent pleuroperitoneal canals. [33],[49],[114] These canals are the defects present in the septum transversum whose function is to separate the pleural and the peritoneal cavities and is the precursor of the one of the components of the future diaphragm. [33]

The pleuroperitoneal canals close by the end of the eighth week, the path taken by these abnormal pinched off lung buds through these defects before the eighth week is proposed to be the track of migration for these cysts. [49] The lung bud differentiates into the bronchopulmonary segments by the seventh week. It appears that for the presence of intra abdominal cysts the critical events of budding and migration occurs between the seventh and eighth week.

Both the cysts have been reported to occur in the cutaneous, [115] subcutaneous, [116] neck, [117] tongue, [118] spine, [119] mediastinum, [120] lung, [102] intrabdominal and retroperitoneal regions [Figure 11]. [103],[114]

Bronchogenic cysts are most commonly located in the thorax, mainly, in the post mediastinum and intrapulmonary regions. Data on the most common site of occurrence of these cysts in the chest is conflicting at best. Some studies suggest mediastinum as the most common site while others suggest the lung parenchyma. [102],[121],[122] Maier [123] further classified bronchogenic cyst into typical which includes hilar, carinal, paratracheal, para esophageal and the atypical variety which included extra thoracic location.

Both bronchogenic or foregut cysts either persist as isolated cysts or get incorporated in other developing organs like esophagus, stomach, pancreas, gall bladder, liver or retroperitoneal region. When the organ of entrapment is the hollow structure of the gastrointestinal tract they should be called duplicated foregut or duplicated bronchogenic cysts provided the standard definition of duplication cyst is adhered to; otherwise they should be called as isolated foregut or isolated bronchogenic cysts.

The terminology to be used for these isolated cysts includes the use of location of the cyst as the prefix. The term to be used in the presence of their location as duplication cyst should be site or organ as the prefix and foregut or bronchogenic differentiation as the suffix.

Within the abdomen, the isolated foregut cysts have been described in relation to the liver, pancreas, gall bladder and retro peritoneum. The most common subdiaphragmatic location is liver. Friedreich [124] was the first to describe the hepatic foregut cyst, postulate its congenital nature and predict it as an embryological malformation. [124] Wheeler and Edmonton [35] in 1984 were the first to use the term CHFC (ciliated hepatic foregut cyst) in the context of these benign liver lesions. Since its first descriptions, in 1857, a total of 96 cases have been described in the literature.

According to our nomenclature, this entity should be called isolated hepatic cyst with foregut differentiation as the term ciliated does not describe anything.

The common location of the ciliated hepatic foregut cyst in segment 4 of the liver may be explained by the fact that the left lobe of the liver, [49] in particular, segment 4 of the liver, constitutes the bulk of the entire liver during the fourth to sixth weeks of development. [114] There is a period of continuous remodeling between the sixth to eighth weeks as the left lobe undergoes peripheral degeneration and the right lobe grows in size after the 9 th week. [114]

We reviewed 94 cases of ciliated hepatic foregut cysts. In almost all the citations that reported the site of the cyst, the cyst appeared within or close to segment 4.

Other intrabdominal solid organ locations of the foregut cysts included six cases associated with gall bladder [1],[49],[96],[125],[126],[127] and 15 within the retro peritoneum and pancreas. [5],[105],[106],[114],[128],[129],[130],[131],[132],[133],[134],[135],[136],[137],[138] A majority of the pancreatic foregut cysts were diagnosed as mucinous cystic neoplasm of the pancreas in the preoperatively workup. [130],[135]

An isolated bronchogenic cyst in the abdomen is usually located in the retro peritoneum as opposed to the more common intraperitoneal location of the isolated foregut cyst. A review of literature on sub diaphragmatic cyst by Liang et al[139] reported 37 citations for this condition in English literature In 82% of the patients the intrabdominal cyst appeared on the left side in a triangle bound by the splenic vein, midline and the diaphragm. [139],[140]

   Conclusion Top

The classification unifies the embryological concepts of foregut cystic malformation and suggest a generic title to the various groups of these interrelated disorders and a uniform use of nomenclature on the basis of unifying concepts of embryopathogeneis.

   References Top

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Sharad Sharma
Department of Transplant Surgery, Methodist University Hospital, Memphis, TN 38104
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DOI: 10.4103/0377-4929.56119

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]

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