Visceroptosis of the bowel in the hypermobility type of Ehlers-Danlos syndrome: presentation of a rare manifestation and review of the literature.

Gastrointestinal complications are common in patients with Ehlers-Danlos syndrome, affecting up to 50% of individuals depending on the subtype. The spectrum of gastrointestinal manifestations is broad and ranges from life threatening spontaneous perforation of the visceral organs to a more benign functional symptoms. Here we describe the clinical and radiographic manifestations of visceroptosis of the bowel, a rare complication of Ehlers-Danlos syndrome that is characterized by prolapse of abdominal organs below their natural position. We further review the literature on gastrointestinal complications in the different forms of Ehlers-Danlos syndrome.

Helical CT of blunt diaphragmatic rupture.

OBJECTIVE: This study evaluated CT findings for signs of blunt diaphragmatic rupture. MATERIALS AND METHODS: CT examinations of 179 blunt trauma patients, including 11 with left-sided and five with right-sided blunt diaphragmatic rupture, were reviewed by two staff radiologists who first decided by consensus on the presence or absence of 11 published signs of blunt diaphragmatic rupture and then formulated the diagnosis in terms of absence of, presence of, or suggestion of blunt diaphragmatic rupture. The significance of the findings was assessed by multivariate logistic regression. Four other reviewers interpreted the CT findings independently. They were asked first to formulate a diagnosis in terms of absence of, presence of, or suggestion of blunt diaphragmatic rupture and then to enumerate the findings supporting a diagnosis or suggestion of blunt diaphragmatic rupture. These findings were compared with those of the staff radiologists. RESULTS: Diaphragmatic discontinuity, diaphragmatic thickening, segmental nonrecognition of the diaphragm, intrathoracic herniation of abdominal viscera, elevation of the diaphragm, and both hemothorax and hemoperitoneum were strong predictors of blunt diaphragmatic rupture (p < 0.001). The combination of the first three findings was 100% sensitive (16/16). The staff radiologists' sensitivity for diagnosing blunt diaphragmatic rupture was 100% (16/16). The four reviewers' sensitivities were 56.2% (9/16), 81.2% (13/16), 62.5% (10/16), and 87.5% (14/16). CONCLUSION: Six of 11 signs were good predictors of blunt diaphragmatic rupture. Despite diaphragmatic thickening, focal defect and segmental nonrecognition had 100% cumulative sensitivity; the reviewers formulating the diagnosis before analyzing CT signs overlooked blunt diaphragmatic rupture on CT in 12.5-43.8% of the patients.

The "dependent viscera" sign in CT diagnosis of blunt traumatic diaphragmatic rupture.

OBJECTIVE: The objective of our study was to describe the "dependent viscera" sign and determine its usefulness at CT in the diagnosis of diaphragmatic rupture after blunt abdominal trauma. MATERIALS AND METHODS: The study sample consisted of 28 consecutive patients (19 men, nine women) between 17 and 74 years old (mean age, 31 years) who had undergone abdominal CT and subsequent emergency laparotomy after a blunt trauma. Ten patients had a diaphragmatic rupture (six, right-sided; four, left-sided) at laparotomy. An experienced radiologist unaware of the surgical findings retrospectively reviewed the CT scans, and then a second radiologist reviewed the scans to provide interobserver agreement. Note was made of discontinuity of the diaphragm, intrathoracic herniation of abdominal contents, and waistlike constriction of bowel (the collar sign). Also noted was whether the upper one third of the liver abutted the posterior right ribs or whether the bowel or stomach lay in contact with the posterior left ribs. Either of these findings was termed the "dependent viscera" sign. The radiologists' detection rate of diaphragmatic rupture on the CT scans via observance of the dependent viscera sign was determined. Interobserver agreement was assessed using Cohen's kappa statistic. RESULTS: The dependent viscera sign was observed on the CT scans of 100% of the patients with a left-sided diaphragmatic rupture and of 83% of the patients with right-sided diaphragmatic rupture. Both observers missed one case of right-sided diaphragmatic rupture. The radiologists' overall rate of detecting diaphragmatic rupture was 90% using the dependent viscera sign. We found excellent interobserver agreement (kappa = 1) for detection of the dependent viscera sign and for the diagnosis of diaphragmatic tear on CT scans. CONCLUSION: The dependent viscera sign increases the detection at CT of acute diaphragmatic rupture after blunt trauma.

Splenoptosis (wandering spleen).

Splenoptosis which is a congenital fusion anomaly of dorsal mesogastrium in children is a very rare entity. In the literature cases are usually diagnosed at operation and it is noted that in former years splenectomy had a special place among various methods of treatment. In this report, a 7-year-old case of splenoptosis diagnosed on ultrasonography and isotope scintigraphic methods preoperatively and treated by splenopexy is presented. In cases with splenoptosis the clinical and radiologic diagnostic criteria are given and the importance of splenopexy in treatment is emphasized.

[Hypertension and nephroptosis]. / Hypertonie und Nephroptose.

Renal diseases have been recognized as both a cause and a consequence of hypertension. Orthostatic renal hypertension is caused by ischemia resulting from elongation, twisting and angulation of renal vessels when a hypermobile kidney is present. Recent observation of a 22-year-old woman in whom orthostatic renal hypertension was cured by nephropexy prompted this report. Clinical manifestations, diagnosis and therapy of this condition are also discussed.

Hepatocolonic vagrancy: wandering liver with colonic abnormalities.

Abnormalities of hepatic fixation resulting in excessive mobility in a transverse plane are uncommonly encountered. The unusual incidental finding of a freely mobile liver and spleen in a patient presenting with sigmoid volvulus is reported. At laparotomy, the inferior aspect of the right hemidiaphragm was smoothly peritonealized, without evidence of coronary or triangular ligaments. It is postulated that this abnormal hepatic mobility reflects persistence of the primitive ventral mesogastrium. To the authors' knowledge, this unusual condition has not previously been recognized. The literature relating to wandering liver is reviewed and four other cases are presented. An invariable association of persisting ventral mesogastrium with abnormalities in colonic anatomy (hepatocolonic vagrancy) is described.

Torsion of a visceroptosed spleen.

Torsion of the pedicle of a visceroptosed spleen, a rare condition, was diagnosed preoperatively in a 4-year-old girl, with the aid of history, physical examination, blood smear findings, splenic scans with technetium Tc 99m sulfur colloid, and selective angiography. A splenectomy was performed, and the child made an uneventful recovery. This case report illustrates some of the diagnostic and therapeutic considerations pertaining to torsion of the spleen.