Digestive disorders

IV./2.4.: Acute abdominal syndrome, as pathology of the perforating ulcer’s seriouscomplication

IV./2.4.1.: Definition, general characteristics

The acute abdominal syndrome is an umbrella term for diseases with different etiopathology but similar, typical abdominal and general symptoms, which usually occur abruptly and develop within a few hours.

Typical symptoms are severe abdominal pain that worsens with pressure, nausea with or without vomiting, abdominal guarding with rebound pain, sensitivity of the Douglas-area during a rectal digital examination, hypotension and tachycardia, weak pulse, pale skin with cold sweating, stopping of bowel movements and urinary excretion (paralysis), dehydration (exsiccosis), increased sedimentation rate, in summary, a state leading to shock. Most of the causes require surgical intervention (e.g. acute appendicitis, gastric perforation, ectopic pregnancy etc).

In rare cases no surgical intervention is needed (e.g. acute inflammation of pelvic organs; gall stones; kidney stones; colic pain caused by intestinal cramps; mesenterial lymphadenitis (toxoplasmosis); acute pancreatitis), but conservative treatment or a minimal invasive method is chosen. Some diseases outside the abdominal cavity can also mimic the symptoms of acute abdomen (e.g. heart infarct, pneumonia and pleuritis). Imaging techniques (abdominal X-ray) have an important role in the diagnosis (free intra-abdominal air [perforation], or nivaeus [gas-fluid levels] in distended intestinal stretches [ileus]). In case acute abdomen is suspected, using an oral contrast agent is considered as malpractice!

IV./2.4.2.: Main symptoms of acute abdominal syndrome

IV./2.4.2.1.: Shock

A state showing the signs of severe disease, with paleness, so-called „cold” sweating, sunken face (called facies hippocratica), hypotension, tachycardia, fainting (collapsus). Typically it has a biphasic pathogenesis: an initial, reflectory shock phase occurs at the onset of the abdominal event, followed by a temporary remission and then evolving into a prolonged, life-threatening shock phase.

IV./2.4.2.2.: Pain

Usually this is the first symptom. Pain can radiate to different directions and body parts: testicles, thighs, shoulders and back. The nature (stabbing, cramping, piercing, pulling, squeezing, etc) may have diagnostic significance.

IV./2.4.2.3.: Nausea (nausea) with or without vomiting (vomitus)

It occurs relatively early. At the beginning it is caused by the reflectoric hypermotility of the intestines, later the long-existing ileus. Ulcer perforation is usually not accompanied by vomiting.

IV./2.4.2.4.: Abdominal muscular defense (défense musculaire)

It is always a sign of peritoneal excitement. Stiff abdomen points to diffuse peritonitis, for example in the state after a perforation. It can appear in a circumscribed form, too: for example in the case of local peritonitis associated to acute appendicitis (appendicitis acuta cum periappendicitide).

IV./2.4.2.5.: Constipation of stool and bowel gas

It is also a sign of peritonitis. If it is present for longer periods, that usually points to ileus. Without peritonitis it can be a sign of a reflectory event, e.g. in the case of kidney colic.

IV./2.4.2.6.: Abdominal distension (meteorismus)

Usually a sign of ileus, but in severe cases it can be associated to peritonitis.

IV./2.4.3.: Etiology of acute abdomen syndrome

Among others, the following diseases are of etiological significance:

• - inflammations (peritonitis, pancreatitis, perforated ulcer, etc.);

• - complications of a trauma (liver-, spleen-, stomach-, urinary bladder rupture);

• - complications of hernias (strangulation – incarceratio);

• - haemorrhagic necrosis in any organ tissue (haemorrhagic necrosis of the intestines, ovaries, or testicles);

• - tumors and their complications (formation of fistulas, eruption onto the peritoneum, twining of a peduncle, spleen rupture in CML);

• - ectopic pregnancy;

• - aortic dissection (dissectio aortae);

• - oesophagus rupture (ruptura oesophagi).

IV./2.4.4.: Peritonitis

Peritonitises can be classified based on:

• - extent (diffuse/local);

• - nature of the exsudate (fibrinous /purulent /bilious/haemorrhagic etc.);

• - pathogenesis (e.g. perforation) or etiology (infectious or sterile);

• - time course (acute/subacute /chronic).

IV./2.4.4.1.: Diffuse purulent bacterial peritonitis

This is the most common form of peritonitides (57% of peritonitis cases in autopsies). It is caused by Gram-negative bacteria: E. coli, Pseudomonas, Proteus, Meningococci; Gram(+)bacteria: Streptococci, Pneumococci, Staphylococci ; rarely: Gonococci.

The following etiologies are common:

• - perforation e.g. in the case of perforated appendicitis, cholecystitis, gastric- or duodenal ulcer, diverticulitis, colitis ulcerosa, Crohn-disease, tumor, infected urachus cyst;

• - permeating peritonitis in case a pleural empyema spreads through the diaphragm;

• - ascending peritonitis from the inflammation of the fallopian tubes, e.g. in the case of salpingitis gonorrhoeica;

• - direct opening of the abdominal cavity e.g. due to a trauma or iatrogenic cause; the latter is the so-called postoperative peritonitis, which is usually benign and local;

• - spontaneous or diffusion peritonitis, typical in cirrhosis when the chronic portal stasis causes a permanent relative hypoxia of the intestines, thus increasing the permeability of the intestinal wall layers, and the passing-through of intestinal bacteria causes a peritonitis of varying severity, often rather latent and disposed to becoming chronic.

Complications:

• - paralytic ileus (ileus paralyticus), which can occur with any type of peritonitis, and when opening the abdominal cavity (operative, traumatic, perforative);

• - circulatory shock develops in almost all cases of diffuse peritonitis due to the released bacterial endotoxines;

• - turning into chronic-adhesive peritonitis can lead to further complications: with the organization of the inflammatory exsudate, due to the filamentous peritoneal adhesions (adhesiones peritoneales filamentosae), strangulation ileus of the intestinal loops can occur. The prognosis of the disease: mortality is high even today, especially in childhood, old age and gastrointestinal perforation: 10-60%.

IV./2.4.4.2.: Local purulent bacterial peritonitis (empyema)

The five most important locations of the local peritonitis (the so-called „storm centers” of the abdominal cavity)

1. - the periappendicular region;

2. - the subphrenium;

3. - the pericholecystic region;

4. - the perisplenium;

5. - the Douglas-pouch (excavatio rectouterina) / excavatio rectovesicalis.

The most common location (44%) is the right hypogastrium (the topographic place of appendititises and salpingitises).

IV./2.4.4.2.1.: Empyemas of the right hypogastric (appendicular) region

They are most commonly (94%) caused by acute, ulcero-phlegmonous/gangraenous appendicitis, which leads to periappendicular inflammation though perforation. This can develop into a perityphlitic/periappendicular abscess, especially if the appendix is located retrocoecally, and shows the clinical picture of the so-called inflammatory conglomerate tumor. Furthermore, purulent right hypogastric empyema often originates from purulent salpingitis/adnexitis (called pyosalpinx), or purulent peridiverticulitis as a complication of diverticulosis. Its pathogenic agents are E. coli, anaerob Streptococci. Mortality (lethality) rate of the disease is 2%.

IV./2.4.4.2.2.: Subphrenic abscess/empyema

Subregional localizations in the area are:

• - the right suprahepatic;

• - the right subhepatic;

• - the left perihepatic; and

• - the left rear subphrenic (bursa omentalis) subregions. Their purulent convergence, union and communication are possible and not uncommon.

The causes can be peptic gastric and duodenal ulcers perforating (around 25-30%) and forging towards this direction; surgeries on the organs of the area (stomach, intestine, bile ducts) with or without suture failure; traumas; spreading of periappendicitis to this direction (which means the union of regions); overspreading diseases from the thoracic cavity (bronchopneumonia, purulent pleuritis, pleuraempyema).

Its pathogenic agents are mostly intestinal bacteria (e.g. E. coli, Proteus, Streptococcus faecalis), Streptococcus pyogenes, Staphylococci). Some subphrenic empyemas can contain more than 1 liter of pus, with severe involvement of the surrounding organs (liver, lungs). It is possible to spread to the thoracic cavity through a fistula. Mortality (lethality) rate of the disease is 25-30%.

IV./2.4.4.3.: Bilious peritonitis (gall bladder region)

About 5% of all peritonitides are bilious, caused by a breach in the biliary duct wall. This can develop postoperatively, due to the accidental total or partial transcision, or strangulation and consequential contusion and necrosis of the biliary duct. Rarely a perforation can be associated with multiple gall stones, when due to the gall bladder peristaltics, the stones scratch each other into multiple-sided shapes (facets), sides meeting in edges and points. If the gall bladder is full of these, the stones pressure its wall in specific locations, which means to decubital ulceration, wall necrosis and perforation.

Another possible cause of biliary duct perforation is acute gangrenous cholecystitis, and less often the distension, ischaemia and rupture of the small biliary ducts. In the abacterial form of bilious peritonitis, sterile bile gets into the abdominal cavity through the breach in the wall, causing peritoneal excitement due to its local chemical effect. Its importance is often overrated, as sterile intraperitoneal bile can be tolerated for weeks without symptoms. In the bacterial form, pathogenic agents coming from the duodenum can get into the abdominal cavity through the lesions of the biliary duct or gall bladder. Bile acids block the granulocytes’ phagocytosis, while bilirubin blocks their chemotaxis, subserving bacterial growth and aggravating the peritoneal process.

IV./2.4.4.4.: Other peritonitides

IV./2.4.4.4.1.: Peritonitis of the spleen area

It mainly occurs due to gastric perforation or when a subphrenic abscess spreads towards the spleen (left perihepaic localization). It can turn into a chronic form, the so-called „sugar-icing” spleen (perisplenitis hyalinea).

IV./2.4.4.4.2.: Peritonitis of the excavatio rectouterina (Douglas-pouch) and excavatio rectovesicalis

Due to topoanatomical reasons (this is the lowest point of the abdominal cavity), almost all exsudate coming from peritoneal excitement dribbles down here (except in the case of peritoneal concrescence and recidiving, isolated peritonitides)

IV./2.4.4.4.3.: Faeces-peritonitis (peritonitis stercorale seu faeculens)

It develops in case of large intestinal perforation, postoperative suture insufficiency, when the intraintestinal space and the abdominal cavity are open towards each other. Massive pathogenic agent load has to be expected especially at colon perforations, making the clinical prognosis unfavourable. Perforation can be caused by colon cancer, diverticulitis, colitis ulcerosa, Crohn-disease, etc. This type is responsible for 6% of dissected peritonitides.

IV./2.4.4.4.4.: Actinomycosis

Rare form, it can be associated to urogenital actinomycosis, sometimes mimics an intraabdominal tumor.

IV./2.4.4.4.5.: Parasitic peritonitis

Primer peritoneal colonisation can also occur in Echinococcosis,but it is more common that a liver echinococcus cyst’s rupture is followed by secondary colonization; the sudden disengagement of the pathogenic agents from their isolated place which was hidden from the immune system can lead to anaphylactic reaction! In Ascariasis (roundworm disease) parasites get out into the free abdominal cavity through the intestinal wall (appendix, Meckel-diverticulum), or between the sutures of surgical anastomoses, causing an abscessed, granulomatous, diffuse or local peritonitis.

IV./2.4.4.4.6.: Pancreatogenic peritonitis

The peritoneal excitement develops as a complication of acute pancreatitis, due to the disengaged pancreatic enzymes (similarly to the autodigestive lipid necrosis, also common in this disease).

IV./2.4.4.4.7.: Gonococcal  upper abdominal peritonitis (Fitz-Hugh-Curtis-syndrome)

This form mainly occurs in females, due to an infection ascending through the fallopian tubes. It can spread paracolically to the subphrenium as well. In male patients it is usually a result of haematogenous/lymphogenous dissemination.

IV./2.4.5.: Acute abdominal syndrome due to organ injuries (traumatic acute abdominal syndrome)

IV./2.4.5.1.: Hollow organs

(i) A stomach rupture may occur due to a blunt trauma to the abdomen, especially in case of tight full stomach (e.g. after a heavy meal). (ii) Urinary bladder rupture may occur in case large amounts of urine are held, also due to blunt abdominal trauma, (iii) Uterus rupture during pregnancy. Typical blunt abdominal injury can develop in all the above organs during car travel: the mentioned traumas may occur during a collision or even heavy braking, caused by the steering wheel or due to the pressure from the seat belt. Therefore it is advisable not only to eat moderately before traveling, but also to empty the bladder.

Pregnant mothers – especially by the end of the pregnancy when their belly claims a remarkable space – should only travel by car only if it is very important, and even in those cases they should avoid driving. Use of the seat belt means an otherwise unusual risk: the tight uterus may rupture even due to the seat belt pressure from a heavy braking, even the whole or parts of the foetus can get out to the abdominal cavity with abdominal bleeding, and in rapidly progressing cases this can be life-threatening for both the mother and the fetus; in milder cases with slower progression, acute abdominal syndrome can develop.

In the lack of a special seat belt (which compasses the thighs and does not constrains directly the belly) – as a lesser evil – in this temporary period the mother should be advised to use the seat belt the following way: the lower, transverse part which would otherwise compress the belly should be placed behind her body (under the bottom), and only the part angularly crossing in front of the chest should be in the front. This arrangement prevents the injuries from abdominal compression and also head injuries from leaning forward, however, unfortunately it allows slipping downwards out of the belt, thus bigger collisions will have such risk (of course bigger collisions – due to the effect of secondary smashes – are always more dangerous for pregnant mothers, even if they are not in the focus of the acting forces). In times past, when horses were generally used, the common causes of blunt abdominal traumas included horse kick and falling off a horse, today this is mainly occurs in life situations related to livestock raising.

IV./2.4.5.2.: Parenchymal organs

(i) Rupture of parenchymal organs (especially the liver, spleen and kidney) may occur due to blunt abdominal trauma. If as a direct result of the trauma, the capsule does not break, only the parenchyma crushes underneath, a biphasic rupture occurs: at first, subcapsular haematoma develops with transient abdominal symptoms and transient improvement, then hours later the pressure of the congestive blood causes capsule rupture and life-threatening intra-abdominal haemorrhage. In this state, there is a great risk of internal exsanguination (exsanguinatio), but the patient may show the symptoms of acute abdomen before the shock develops. (ii) Organs can partially or completely split off their hilum, e.g. due to falling from a height. Both types of injuries are facilitated when the organ’s original size is multiplied (e.g. extreme splenomegalia associated to CML; septic splenomegalia and softening of the spleen tissue; enlargement of kidneys due to a tumor, etc.)

IV./2.4.5.3.: Type of the acting force (trauma)

Such mechanisms include sports injuries, traffic accidents (car: steering wheel or seat belt-caused internal injuries [see above]; motorcycle: falling with or falling off the vehicle, the human body’s unprotectedness makes these injuries especially dangerous), injuries from brawls, etc.

IV./2.4.6.: Hernias and their complications 

IV./2.4.6.1.: Types of hernias

Based on their locations, hernias can be:

• - external  (appear under the skin through the lesion in the abdominal wall) and

• - internal (e.g. protrusion of abdominal organs into the thoracic cavity [diaphragmatic hernia]  or into retroperitoneal peritoneum pouches [retroperitoneal hernias])

IV./2.4.6.2.: External hernias

• - IV./2.4.6.2.1.: Hernia inguinalis lateralis (indirect inguinal hernia) et medialis (direct inguinal hernia) The lateral ones can be congenital (open processus vaginalis peritonei) or acquired; while the medial ones are always acquired.

• - IV./2.4.6.2.2.: Hernia femoralis (femoral hernia) This protrusion point develops in case of weak septum femorale (medially under the ligamentum inguinale, near the lacuna vasorum) which thus serves as locus minoris resistentiae.

• - 2.4.6.2.3.: Hernia umbilicalis (umbilical hernia) Its congenital variant usually spontaneously ceases after a few years. The acquired type, found in adults, can reach large sizes.

• - 2.4.6.2.4.: Herniae abdominales (abdominal hernias) These occur in various locations on the basis of the abdominal wall’s mechanical weakness. Overstretching (e.g. pregnant abdomen), and surgical scars play a role in it. Herniae lineae albae (so-called rectus diastasis) and herniae lumbales also belong here.

IV./2.4.6.3.: Internal hernias

IV./2.4.6.3.1.: Herniae diaphragmaticae These have two types:

1. - hiatushernias and

2. - extrahiatal hernias.

IV./2.4.6.3.2.: Hernia intraabdominalis (intraabdominal hernia)

Intraabdominal organ contents protrude or slide through into anatomically preformed, but pathologically dilated peritoneal pockets (recessus peritoneales). These include: paraduodenal hernias; mesenterial hernias (the hernial orifice is a breach on the mesenterium); hernia bursae omentalis (the orifice is the foramen epiploicum); paracoecal hernias; hernias of ligamentum latum uteri.

IV./2.4.6.4.: Hernia contents 

Any mobile organ of the abdominal cavity can become hernia content: intestinal loops, omentum, less often the ovaries, fallopian tubes, appendix, part of the urinary bladder, etc.

IV./2.4.6.5.: Complications of hernias

• - IV./2.4.6.5.1.: The hernia sac can adhere or accrete with the hernia content (hernia accreta), this is associated with decreased reducibility (reponability) and increased risk of incarceration, and it also increases the risk of pseudo-reposition (’reposition en bloc’) which means that at the attempted reposition the peritoneum of the wall is torn down from its base.

• - IV./2.4.6.5.2.: Incarceratio (incarceration). One type of incarcerations is the so-called flexible incarceration. In this case, with the elevation of the abdominal pressure, visceral parts protrude into the hernia sac . The immediate, reflective contraction (contractio) of the hernial orifice on the hernia content causes incarceration, and spontaneous reposition becomes impossible. The other type is the so-called faeces incarceration, when the approaching stem of the bowel loop that had slid into the hernia sac is increasedly filled with bowel contents, pressuring the departing stem. Thus the bowel content passage gets stuck and the swelling of the hernial content prohibits the reposition.

• - IV./2.4.6.5.3.: Consequences of incarceration. Incarceration means a real passage obstruction, mechanical ileus, in prolonged cases with iliac passion (miserere – vomiting of fluid with fecal odor and colour [faeculent]), haemorrhagic intestinal loop infarct, in time followed by total intestinal wall necrosis, which leads to perforation, development of fecal abscess and fistula, or diffuse fecal-purulent peritonitis (peritonitis faeco-purulenta).

IV./2.4.7.: Haemorrhagic organ necrosis

IV./2.4.7.1.: Haemorrhagic infarct of the intestinal wall (necrosis seu infarctushaemorrhagicus)

It occurs at both arterial and venal vascular lesions.

Causes of acute arterial occlusion:

• - thrombembolisation from left heart (ventricular origin: most commonly a post-infarct thrombotic wall endocarditis (so-called concomitant endocarditis), or infectious, or sterile valve-endocarditis; atrial origin: atrial dilatation associated to valve defect; auricular thrombosis [thrombosis auriculae]);

• - local mesenterial arterial thrombosis.

Causes of obstructed venal circulation:

• - outside compression or bending of the veins (e.g. incarcerated hernias, a part of the intestine’s sliding into another section of intestine [invagination, intussusception], clamping [strangulation]);

• - venal occlusion (e.g.. thrombosis);

• - increased blood thickness (e.g.. polycytaemia rubra vera or other thrombotic disposition [thrombophilia, hypercoagulabilitás])

IV./2.4.7.2.: Gallbladder torsion (torsio vesicae felleae)

It can develop if:

• - ligaments fixing the gall bladder are congenitally absent (wandering gallbladder), or

• - the gallbladder is fixed loosely to the liver surface (‘floating gallbladder’), or it can also happen in cases of

• - positional abnormality of the gallbladder (ptosis),

• - sudden body movements,

• - abdominal force (trauma).

IV./2.4.7.3.: Ovarian torsion

Torsion of the stem, most commonly in cases of pathologically enlarged ovaries (e.g. ovarian cysts, tumors). It can happen spontaneously or due to an abdominal trauma.

IV./2.4.7.4.: Testicular torsion (torsio testis)

The torsion of the stem occurs mostly to direct force (e.g. sports injuries, mostly soccer). It is more common in childhood. It can cause abdominal symptoms in cases when the pain radiates towards the abdomen, but examination of the testicle area usually shows the diagnosis.

IV./2.4.8.: Tumor complications

IV./2.4.8.1.: Gastrointestinal tumors

Perforation due to a tumor’s transmural degradation can be associated with the acute purulent inflammation of the peritoneal surface, but also even with stercoral inflammation.

IV./2.4.8.2.: Ovarian tumors

The tumor mass can cause the torsion of the organ stem as an archpoint, with consequential haemorrhagic necrosis (infarctus haemorrhagicus).

IV./2.4.8.3.: Tumors of the blood and blood-forming organs

Hepato-splenomegaly is common in these tumors. Due to the softening and pathological enlargement of the tumor-infiltrated organs (in CML the spleen can reach the hip bone and may weight several kilograms) the risk of organ rupture is increased even spontaneously or to a normally non-dangerous trauma.

IV./2.4.8.4.: Lymphangioma cysticum

A rare childhood tumor. It can cause its own necrosis by compressing its own blood vessels, or it can occlude the haemoperfusion of the intestinal wall, thus leading to less or more severe acute abdominal syndrome.

IV./2.4.8.5.: Haemangioma cavernosum hepatis

Tumors located under the capsule can rupture due to blunt abdominal trauma, causing abdominal bleeding and acute abdomen syndrome.

IV./2.4.9.: Tubal pregnancy (graviditas tubaria)

Causes:

• - salpingitis chronica;

• - salpingitis isthmica nodosa, the nodular thickening of the tubal wall near the corpus uteri and consequential lumen stenosis due to the mucosal gemmations enfolding into the wall (a progress considered similar to endometriosis, called endosalpingiosis);

• - dysfunction of the tubal mucosal cilia. Its complications are tubal rupture and life-threatening abdominal bleeding, with the symptoms of shock and acute abdominal syndrome (Macropicture 6).

Macropicture 6: Graviditas tubaria: a few millimeter sized intact embryo can be seen in the fallopian tube which is haematosalpinx-like dilated, has attenuated walls, but had not yet ruptured. It is notable that at this size the fallopian tube normally ruptures with free abdominal bleeding. However, in this case a balloon-like tubal dilatation is present, which does not develop due to the relatively fast growth of the embryo, but due to prolonged pressure rise. Therefore in this case there had already been a hydrosalpinx present, and the stricture leading to that caused the tubal pregnancy, too. (Source: Picture Archives of Semmelweis University 2nd Department of Pathology, collected by Attila Kovacs and Ildiko Szirtes)

IV./2.4.10.: Aortic dissection (dissectio aortae)

Its symptoms may mimic an acute abdominal syndrome, and – as it is not primarily an abdominal process – sometimes it has to be differentiated from that. It is usually a consequence of severe aortic sclerosis (atherosclerosis aortae) or Erdheim-Gsell cystic medial degeneration (as part of the Marfan-syndrome).

Behind an initial endothelium rupture – usually downstream, less often in the opposite direction – the wall layers split up horizontally along the longitudinal axis of the blood vessel; blood enters into the generated pseudolumen, and it bloats the originally only virtual gap (virtual lumen) to an actual space, and simultaneously compresses the original inner space into a narrow gap. Thus the circulation stops, as the original lumen is compressed and not permeable, while the pseudolumen is only a blind sac downstream, until another fissure is produced on the lower end and the blood can leave here – in a favourable case, back into the aortic lumen.

However if the second fissure opens outwards of the blood vessel, a life-threatening retroperitoneal haematoma can occur. Further penetration of the haematoma towards the free abdominal cavity results in the so-called haemascos (haemoperitoneum). Its main symptom is the clapping or piercing, exterminating, abdomino-thoracic pain attack, which may radiate into the back or even the thighs.

It can cause collapsus and circulatory shock (most commonly without défense, as there is not enough time for it to develop). Orifices of the branches can narrow if the vascular wall disruption reaches them, moreover the dissection can spread into the branches, causing obstruction in them, too.

The evolving clinical picture is diverse: radial pulse can become irregular; involvement of the head arteries (aa. carotis) can cause brain dysfunction; stenosis of the mesenteric root may be associated with ischaemic pain of the intestines (colic) and intestinal necrosis; lesion of the kidney arteries may present as anuria; backward-spreading dissection can reduce coronary circulation, leading to angina pectoris or heart infarct, or it can disturb the function of the aortic valve (vitium valvulae aortae). Immediate complete rest and analgesic treatment is necessary in order to survive. In favourable cases (for example if the second fissure is directed inwards the aortic lumen), surgery with a vascular graft can be chosen in planned time. Emergency surgery is necessary in case of rapidly progressing symptoms and pathogenesis, however, mortality rates are still high.