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A. Kolek, V. Kral, J. Klein, L. Horak, V. Prochazka, D. Houserkova, M. Kocher, M. Herman, T. Tichy

Faculty Hospital and Medical Faculty Palacky University Olomouc, CZ

Surgical Clinic of the 3rd Medical Faculty Charles´ University and Faculty Hospital Kralovske Vinohrady, Prague, CZ

 

Abstract

Authors of this case study describe treatment of a female patient (born 1982) who had been repeatedly treated since three years of age for enterorrhagias on the basis of arteriovenous malformations of rectosigmoideum. Simultaneously, prehepatal portal hypertension had been proven in the patient. Photocoagulation of arteriovenous malformations of rectosigmoideum with a Nd:YAG laser, followed by a surgery, were selected as definitive approach.

 

Case Study

Birth weight of the patient was 1300 grams, she was hypotrophic, and that was why umbilical vein was cannulated. Enterorrhagia had been followed intermitently since the first year of age. Endoscopic examinations proved varicoses on the walls of rectum, and these were repeatedly sclerotized. No vascular anomalies were indicated in the rectum area by an angiographic examination in 7 years of age. USG examination of liver and portal liverbed indicated no pathologic findings. The patient developed physiologicaly. She took ferrous preparations due to hypochromic anemia as a result of chronic intestinal blood loss. When she was 17 her height was 167 cm and weight 52 kg. At this age her enterorrhagia intensified and became more frequent, and  the scope and volume of varicosities increased as well, the varicosities being knotty and congestive. They affected distal 30 centimeters of the colon as far as to the anus. Transabdominal USG in lengthwise projection showed a tubular structure corresponding to colon (in rectosigmoideal area) in the central hypogastrium. The wall was widened to 16 mm. Hyperechogenous stripes in the intestinal wall corresponded to coiled vascular structures detected with a color doppler mapping.

Further examinations revealed pre-jecoral portal hypertension PHP). Gastroscopy verified a sedate varix 1st grade above the cardia. USG examination gave a picture of cavernous transformation of portal vein, unwidened in lengthwise projection (7.6 mm) and trackable to the liver hilus, with hepatopetal blood flow at normal speed (0.11 m/s). The portal vein passes to extensive coiled anechogenous formations, corresponding to venous structures in doppler examination. It is a case of cavernous transformation of the portal vein.

Direct splenoportography (6/2000) showed an increased pressure 27 cm of water column in the lienal vein, and seezofageal veins were filled through veni coronariaventriculi. The main phylum of the porta was found adequate, its branches narrowing quickly and their filling ending approximately after 4 cm. The obstruction is evident on the branches of the lower order. Intrahepatic filling of the portal flow was supplied through collaterals. A thorough hepatological examination including a biopsy did not show an affection of liver, neither a thrombophylous condition was detected (standard values of AT III, S-protein, C-protein, homocysteine in the serum, APC resistence). Selective angiography (a. mesenterica superior, a. mesenterica inferiora of both inner ilical arterias) did not show neither typical  nidus, nor early venous phasis, both being characteristical for a possible AV malformation in the rectum area. In this early phasis it was not evident whether varicoses of the walls of rectum are manifestations of the lower type of collaterization of PHP, or of venous malformations. A consulting surgeon had rejected primary resection of the intestine due to a fear of  unstoppable bleeding of above mentioned varicoses. That was why a non-surgical treatment was chosen - photocoagulation of venous malformations of the rectum with an Nd:YAG laser (wavelength 1064 nm, applied power 13 to 35 Watts) in three sessions (12/1999, 3/2000, 5/2000). A thermic coagulation necrosis of mucose and submucous ligament, followed by a trombosis were expected results of the therapy. During the second endoscopic treatment a massive bleeding of one of the varices appeared due to an inversion of the apparatus in the rectal ampula. During five minutes, necessary to change the device in order to ensure suction and view of the operation field, the blood loss was 1700 ml. Later on, the bleeding field was successfully treated by a laser coagulation. Decrease of the number of the varicoses, as well as of their volume, were benefits of this kind of treatment, however at the expense of fibrosis of the intestine wall. Nevertheless, massive and hemodynamicaly significant enterorrhagia, with decrease of KO values and with a need of blood transfers, continued in the course of the treatment.

As the following step a surgical approach was evaluated - either a classical resection of the rectum and rectosigmoideum, or a resection of mucose and submucose of the rectum after Longo. Repeated massive enterorrhagias speeded up indication for an operation (08/2000). A resection of rectosigmoideum and a simultaneous portocaval anastomosis were planned. The finding in the abdominal cavity did not indicate PH, vessels in the portal flow were not dilated, both mesentheric vessels were slender. The rectum and a part of the sigma were pathologically changed and apparently transformed on the basis of the vessel malformations. Furthermore, previous photocoagulations caused a fibrosis of the rectosigmoideum. Therefore it was decided to abandon the insertion of a portocaval bypass, and resection of rectosigmoideum was performed. The proximal resection line was chosen in the area of starting macroscopic changes and the distal line was 8 cm above the constrictor, with an end-to-end anastomosis. A pathologist evaluated the mount an arteriovenous vascular malformation (haemangioma), wide, coiled and mainly thick-wall veins passing to numerous thin-wall cavernous spaces were found in the submucosis, with only incomplete and reduplicated elastic threads being found in the walls of the veins.

The disease was closed as duplicated pre-hepathal portal hypertension caused by cavernomatous transformation of the portal vein and AV malformation of the wall of rectosigmoideum. The cavernomatous transformation of the portal vein might be caused by a cannulation of vena umbilicalis carried out in the infant age.

 

Discussion

Children´s enterorrhagia is usually initiated by intestinal infection, by unspecified intestinal inflammations, by food supersensitivity, by polyps. Representation of individual affections changes by aging, and its spectrum differs from this in adults (Roy). Bleeding into alimentary tract is usually caused by venous anomalies, such as haemangiomas, angiodysplasias, teleangiectasias (Mathus Vliegen), by cavernous haemengiomas (Corbally, Saito, Masterson). There have been described family venous vascular malformations in brothers and sisters (Bernardini, Atin).

In a part of the patients, bleeding into the lower part of the digestive tract can be tied in with overpressure in the portal vein. In cases of hepatic portal hypertension it was sometimes possible, especially in patients with hepatic cirrhosis, to find endoscopic symptoms of portal hypertensional colonopathy (PHC) characterized by a "colitislike" picture - oedema, vascular ectasias, nidal erythemas, granulated relief of the mucose (Bini, Misra), and/or polypous lesions (Huang). In adult patients suffering from hepatic cirrhosis and pressure gradient over 12 mm Hg vascular ectasias were identified in 26 per cent, irregularities of mucosal veins in 32 per cent and their dilatations in 30 per cent, solitaire red spots in 25 per cent, diffuse red spots in 10 per cent, and hemorrhoids in 25 per cent of cases (Yamakado). In another group of similarly affected patients anorectal varices were present in 40 per cent and colonopathias in 48 per cent of cases. In comparison with healthy people the ocurrence of hemorrhoids was not increased (Misra). In one third of patients with hepatal cirrhosis thickening of the wall of the ascending colon was detected. No similar endoscopic picture was found in our patient. Obliteration of cranial shunts (sclerotization, devascularization, transection and mesoportal thrombosis) can worsen PHC (Naef 98, Ohashi). After obliteration of upper shunts colonic varices were present in as much as 60 - 100 per cent of patients (Naef). In a group of 139 childresn patients colonopathy was detected only in 2 patients (1.4 per cent), colonic AVM and inner hemorrhoids always in 1 patient (0.7 per cent) (Yasha). Apart from our patient no enterorrhagia was present in 15 children patients with PH treated in our clinic, therefore no colonoscopic examination was indicated in them (data not stated). That is why we have to reserve our judgement about ocurrence of portal hypertensional colonopathia  and varicoses of the colon in other children suffering from PH.  Histologically it is possible to prove in the mucose of the colon of patients with PHC coiled veins with thickened wall and arteriolization of veins up to vascular anomalies (Lamps).

It is typical for prehepatal portal hypertension that varices of rectosigmoideum occur more frequently as a manifestation of distal type of shunt formations. Endoscopic picture of PHC is not given. We had at first been considering this possibility, but a histologic picture of the wall of a resection sample pointed to a primary AV haemangioma (malformation). However, we were not able to explain why a typical picture of an AV malfomation was not proven by an angiography.

Coincidences of hepatal/prehepatal portal hypertension and AV malformations of rectosigmoideum have been described by Yasha et al. Schulman et al. describe a patient whose AV malformation in the colon lead to increased inflow of blood into mesenterial flow and thus to overpressure. Similar haemodynamic manifestations can be caused by AV malformations localized in liver and spleen. In these cases the mechanism of development of portal hypertension is different than the condition found in our patient.

Treatment of venous affections is either endoscopic (electro-coagulation, sclerotization, photo-coagulation) or, in case of failure, surgical (Machicado, Carolo, Pratt,Morris, Mathus-Vliegen, Naef). Reduction of the number of varicoses by photo-coagulation in our patient was the initial step to make the following smooth surgical operation possible.

 

Conclusions

At present, our patient is after a surgery, with no complications, and control colonoscopy has shown an untroubled line of anastomosis on rectum with no signs of relapsing vascular malformations.