Pâncreas curto congênito, poliesplenia e trombose da veia porta: associação ou achado casual / Congenital short pancreas, polysplenia, and portal vein thrombosis: an association or a chance finding

A síndrome de poliesplenia e pâncreas curto é considerada uma anomalia congênita rara, freqüentementeassociada a malformações cardiovasculares e abdominais. Na literatura consultada não foram descritos casos desta síndrome associada à trombose da veia porta com transformação cavernomatosa da mesma. Neste estudo é apresentado o caso de um paciente com síndrome depoliesplenia, pâncreas curto, trombose da veia porta, sendo também observada má-rotação intestinal e interrupção da veia cava inferior com fluxo colateral para a veia ázigos. Enfatiza-se o papeldos métodos de imagem ù trânsito intestinal, tomografia computadorizada e ecografia com Doppler em cores ù no diagnóstico destas anomalias.

Polysplenia syndrome with short pancreas is a rare congenital anomaly usually associated with cardiovascular and abdominal malformations. We describe a patient with this syndrome associated with portal vein thrombosis and cavernous transformation, anassociation that has not been reported before. This patient also had intestinal malrotation and interruption of the inferior vena cava with azygous continuation. Special attention is drawn to the role of imaging studies ù bowel transit, computed tomography andcolor Doppler sonography ù in the diagnosis of these anomalies.

Beckwith-Wiedemann syndrome and bilateral adrenal pheochromocytoma: sonography and MRI findings.

Beckwith-Wiedemann syndrome is characterized by a group of clinical abnormalities, the most frequent of which are omphalocele, macroglossia, gigantism, neonatal hypoglycemia and umbilical hernia. The association of this syndrome with malignant tumors is well documented. We report a child with this syndrome associated with bilateral adrenal pheochromocytoma.

Color Doppler sonography of normal and torsed testicular appendages in children.

OBJECTIVE: The purpose of our study was to determine the size and appearance of normal and torsed testicular appendages on color Doppler sonography. SUBJECTS AND METHODS: Thirty-three patients with acute scrotal pain underwent gray-scale and color Doppler sonography and subsequent surgery. Twenty-two patients had torsion of the appendix testis, six had epididymitis, three had torsion of the testis, and two had torsion of the appendix epididymidis. The testicular appendages of 30 patients and the testes of three were excised and sent to the laboratory for histopathologic examination. RESULTS: A testicular appendage was identified on color Doppler sonography in 23 patients (21 patients with torsed appendages and two patients with epididymitis). The torsed appendixes testis measured from 4.1 to 16.3 mm, and the normal appendixes in the two patients with epididymitis measured 4.1 and 5.6 mm. With the visualization of an appendix larger than 5.6 mm as the sonographic diagnostic criterion for torsion of a testicular appendage, sensitivity was 68.2% (95% confidence interval (CI), 45.1-85.3%), and specificity was 100% (CI, 74.7-100%). CONCLUSION: The identification of a testicular appendage larger than 5.6 mm is suggestive of torsion. Therefore, depending on the patients' clinical conditions, these cases can be treated conservatively when an appendage larger than 5.6 mm is identified.

Omental infarction in children: color Doppler sonography correlated with surgery and pathology findings.

OBJECTIVE: The objective of our report is to describe color Doppler sonography findings of omental infarction and correlate them with surgical and pathology findings. Ten children underwent preoperative gray-scale and color Doppler sonographic examinations; omental infarction was confirmed at surgery. CONCLUSION: Color Doppler sonography findings were reviewed and correlated with surgical and pathology findings. Color Doppler sonography features of idiopathic omental infarction differ from those of infarction secondary to omental torsion.

Sonographic findings of Meckel's diverticulitis in children.

OBJECTIVE: We know of only one previous study that has described the sonographic appearances of the inflamed Meckel's diverticulum in several cases (nine patients). Our study reviews sonographic, clinical, and pathologic findings in 10 patients with an inflamed Meckel's diverticulum. We also describe unique findings on color Doppler sonography for two of our patients. CONCLUSION: Sonographic findings of an inflamed Meckel's diverticulum may mimic findings for acute appendicitis or intestinal duplication. In patients with rectal bleeding due to diverticulitis, the visualization of a tubular hyperechoic structure on sonography is suggestive of Meckel's diverticulum. The inflamed Meckel's diverticulum may present as a cyst, but its mucosal layer is more irregular than that found in an intestinal duplication. We found that routine color Doppler sonography revealed anomalous vessels and signs of inflammation on the wall of the Meckel's diverticulum.