Pulsatile anorectal varices and pulsatile hepatosplenomegaly secondary to traumatic tricuspid regurgitationH Ashrafian, C Salih, A Darzi, T Athanasiou
Department of Biosurgery and Surgical Technology, Faculty of Medicine, Imperial College London, United Kingdom
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0022-3859.48450
Source of Support: None, Conflict of Interest: None
A 16-year-old boy presented with an eight-month history of "persistent throbbing hemorrhoids" that intermittently bled and had been refractory to treatment following two sessions of banding therapy. He had no significant past medical history, although he had been involved in a road accident 12 months earlier. There was no history of discernable injury at that time, but he did complain of increased fatigue, weight gain and dyspnea following this episode.
Proctoscopy revealed pulsating anorectal varices and no hemorrhoids. There was also pulsatile hepatomegaly and pulsatile splenomegaly. There were no caput medusae. Cardiovascular examination revealed a holosystolic murmur at the left sternal edge accentuated with inspiration (Rivero-Carvallo sign). This was more pronounced with hepatic compression (Mueller sign). There was jugular venous distension at 8 cm with both sacral and pedal edema. Full blood count, coagulation and biochemistry were normal.
Transthoracic echocardiography [Figure 1] and trans-esophageal echocardiography (TOE) revealed tricuspid regurgitation (TR) resulting from anterior leaflet prolapse secondary to rupture of the chordae tendinae. Doppler sonography was consistent with portal hypertension (PHT) and upper gastrointestinal endoscopy revealed Grade-1 esophageal varices. Colonoscopy revealed bluish tortuous varices at the anorectal junction, but was otherwise normal and did not demonstrate any other cause for lower gastrointestinal bleeding.
The working diagnosis was of PHT with anorectal varices secondary to traumatic TR, and a decision was made for operative repair. This was performed in the presence of TOE through a median sternotomy, cardiopulmonary bypass and moderate systemic hypothermia. The tricuspid valve was exposed through the right atrium. Annuloplasty (De Vega technique  ) and repair of the prolapsed anterior cusp was carried out by inserting two artificial chordae of 5-0 GORE-TEX® sutured between the anterior leaflet and the corresponding papillary muscle.
Postoperative course was uneventful and follow-up at two years revealed a competent tricuspid valve on echocardiography. There was also complete remission of the anorectal varices and portal hypertension.
Anorectal varices and hemorrhoids are rare in adolescents and children, and when present, they are usually secondary to PHT. Hemorrhoids are defined as arteriovenous vascular cushions originating between the superior rectal and hemorrhoidal arteries. Anorectal varices are distended saccular veins at the site of the anorectal porto-systemic anastomosis.  In children suffering from PHT, 35% have anorectal varices, 33% have hemorrhoids and overall 73% have some abnormalities at their external hemorrhoidal plexuses.  To the best of our knowledge, we believe this case to be the first in the literature associating TR as a cause of PHT complicated by an anorectal disease.
TR can present with a wide spectrum of peripheral signs resulting from venous congestion along the path of the superior and inferior vena cavae.  In this case, hypertension at the porto-systemic junction of the superior rectal vein (portal system) with the middle and inferior rectal veins (caval system) ultimately led to the finding of pulsatile anorectal varices.
Furthermore, to the best of our knowledge, the finding of splenomegalic pulsatility associated with TR has not been previously described (although it is known in aortic regurgitation - Gerhardt sign), and presumably occurred by pulsatile transmission through the splenic vein which communicates directly with the portal vein.
Traumatic TR is a rare complication of blunt chest trauma, particularly in the adolescent and pediatric populations. It is most commonly associated with road traffic accidents, and can have a delayed presentation, which can lead to significant morbidity and mortality.  Although the valve repair resolved the symptoms in this instance, increased awareness of the multi-systemic involvement of TR is necessary to prevent its potential for morbidity and mortality.