Retroperitoneal ganglioneuroma. Report of a case

OBJECTIVE: To report an additional case of retroperitoneal ganglioneuroma. METHODS/RESULTS: A case of retroperitoneal ganglioneuroma that had been incidentally discovered in a 27-year-old male during abdominal US evaluation is described. The diagnosis was based on the histopathological findings after US-guided biopsy. The clinical features and the findings of the complementary tests, which included radiological assessment, intravenous urography, CT and cavography are presented, as well as the pathology findings. Treatment was by complete surgical excision of the tumor. CONCLUSION: Treatment of ganglioneuroma is by surgery, since the diagnosis is generally based on the histopathological analysis of the surgical specimen. For those cases with a preoperative diagnosis, some authors advocate surgery for patients with clinical evidence of neuroblastoma or another pathology arising from this disease. Although infrequent, ganglioneuroma coexisting with neuroblastoma can occur. For this reason, complete excision of the tumor is preferred.     

Unusual case of tracheal agenesis

We describe the clinical presentation and pathological features of an unusual case of tracheal agenesis. The axial derivatives of the primitive foregut between the larynx and stomach were represented by a single structure featuring sequential segmentation into regions showing exclusively tracheal or esophageal differentiation in a pattern that is not easily classified by existing nosologic systems nor explained by the conventional hypothesis of dysontogenesis.     

A case of bladder agenesis

A plasmid (5.4 kbp) from Salmonella Typhi D4 has been identified as encoding a restriction and modification (R-M) system. DNA fragments (2537 bp) that carried the genes for restriction endonuclease and methyltransferase encoded on the plasmid were sequenced. Two divergently arranged open reading frames of 957 bp for the restriction endonuclease consisting of 318 aa (amino acids) and 1140 bp for the DNA methyltransferase consisting of 379 aa were identified. These sequences were similar to the sequences of the SsoII R-M system, including the interspace between the two genes.     

Renal endometriosis. Report of a case

In the light of a personal case and a review of the literature, the authors recall the specific features of renal endometriosis. This rare lesion, with a favourable prognosis, is difficult to diagnose and is rarely diagnosed preoperatively. Medical treatment by LHRH agonists is discussed and has been used exceptionally.     

Late displacement of a fracture dislocation at the lumbosacral junction. A case study

STUDY DESIGN: This is a retrospective single-patient case report with a literature review. OBJECTIVE: To report on an uncommon injury: traumatic fracture-dislocation of the lumbosacral junction with delayed displacement. SUMMARY OF BACKGROUND DATA: Only 31 cases of traumatic fracture-dislocation of the lumbosacral junction were found in the English literature. Only three previous reports refer to this injury occurring with a posterior displacement. This is the first report of this type of injury displacing in a delayed manner. METHODS: Chart review. RESULTS: Open reduction, Arbeitsgemeinschaft fur Osteosynthesefargen instrumentation, and interbody fusion resulted in a satisfactory clinical result with no neurologic impairment. CONCLUSIONS: The present study reports a very rare injury consisting of traumatic dislocation of the lumbosacral joint, presenting with displacement 2 months after injury. The patient was treated successfully with surgery even though the injury displaced in a delayed fashion.     

Thoracic epidural angiolipoma. Report of a case

Angiolipomas of the spine are rare (40 cases in the literature). When they are intraspinal, these masses are epidural in more than 90% of the cases. Angiolipomas are benign tumors containing vascular and mature adipose elements. The clinical symptomatology is non specific, but computed tomography and mostly MRI provide a precise diagnosis. With one additional case and a review of the literature, we define the main characteristics of these tumors.     

Rendu-Osler-Weber disease. Report of a clinical case

Rendu-Osler-Weber disease is an hereditary disorder characterized by cutaneo-mucous telangiectasis and vascular abnormalities in several organs. Bleeding, especially epistaxis, represents the most important clinical feature. Pulmonary arteriovenous fistulae can cause hypoxaemia, haemoptysis, polycythaemia and clubbing. Diagnosis is based on family and personal history, teleangiectasis, laboratory (haemochrome, fibrinogen, PT, PTT) and instrumental findings (endoscopy and/or roentgen). Therapy depends on symptoms. Embolization of pulmonary arteriovenous fistulae and laser treatment of intestinal vascular abnormalities have been successful. Danazol treatment yielded controversial results. We report the case of a patient admitted for arterial hypertension and recurrent epistaxis. Rendu-Osler-Weber disease diagnosis was made based on positivity at family and personal history, clinical examination, laboratory and instrumental findings. In conclusion we underline the pivotal role of anamnesis and clinical examination in the differential diagnosis of hereditary bleeding disorders and emphasize the importance of early diagnosis for the correct therapeutic approach.     

Essential iris atrophy. Report of a case

The clinicopathologic findings in an eye from a 40-year-old man with essential iris atrophy are reported. Noteworthy is the proliferation of corneal endothelium and a laying down of a new Descemet (cuticular) membrane over the trabecular meshwork and in areas of anterior peripheral synechiae formation over the pseudoangle. The histologic characteristics favor a vascular cause for the condition, but the evidence is circumstantial.     

Giant cell myocarditis. Report of a case

The authors report a case of giant cell myocarditis leading to rapidly progressive cardiac failure despite immuno-suppressor treatment in a 20 year old woman. The cardiac failure was successfully managed by implantation of a left ventricular assist device and then cardiac transplantation. The problems encountered underline the importance of accurate diagnosis by endomyocardial biopsy before undertaking treatment and the difficulties in the choice of appropriate method of assistance in this indication. Giant cell myocarditis is a rare cause of cardiac failure and should be considered in the differential diagnosis in view of its clinical features and risk of progression. The literature and the therapeutic implications are discussed.     

Tropical pulmonary eosinophilia. Report of a case

Tropical pulmonary eosinophilia (TPE) is an unusual manifestation of filarial infection, most commonly found in South-East Asia and caused by immunologic hyperresponsiveness to Wuchereria bancrofti and Brugia malayi. This report concerns a case of TPE in a 25-year-old Indian male who had been living in Italy for two years and was admitted to hospital with chest pain. Diethylcarbamazine therapy proved effective in rapidly eliminating symptoms and pulmonary abnormalities, as well as normalizing of laboratory findings.     

Acute plasma-cell leukemia. Report of a case

A case of fatal, diffused plasmacytoma is presented. The picture also included leukaemia and is felt to be an example of plasma-cell leukaemia, as opposed to plasmacytoma in the light of the anatomical and clinical features of the onset and course: high and very pronounced plasma-cell leukocytosis, diffuse proliferation of the plasma cell series in the bone marrow, spleen and lymph nodes, infiltration of plasma cells in the parenchymal organs, extremely rapid progress with serious anaemia, haemorrhage, general organic involvement, high fever, and infection.     

Intra-orbital dacryops. Report of a case]

Cirrhotic liver is predisposed to bacterial infections. Different species of bacteria including Escherichia coli, Enterobacter and Bacteroides fragilis were found to colonize thioacetamide-induced cirrhotic rat liver. Zinc treatment of the cirrhotic rats significantly corrected the histological and histochemical changes in the liver. However, this reversal with zinc treatment was not accompanied by any change in the bacterial colonies in the liver. The study shows that cirrhosis predisposes liver to bacterial colonization and the process is not reversible despite the partial reversal of the cirrhotic changes.     

Right hematocolpos and hematometra with left renal agenesis. A rare association. Apropos a case

Although this anomaly is seen more frequently, the present case is unique in that the genital anomaly (imperforate vagina with the subsequent hematocolpos and hematometra) was associated with contralateral renal agenesis, whereas the cases reported in the literature have all been ipsilateral. Like most of the cases, the presenting symptom was acute urinary retention arising from extrinsic compression of the genital mass on the lower urinary tract. The embryological aspects of the genitourinary system concerning the present case are discussed, as well as some of the hypotheses that have been put forward to explain the etiology of these anomalies, although these have been developed on the basis of the associated ipsilateral anomalies observed.     

Fibroadenoma of the vulva. Report of a case

A case of fibroadenoma occurring in the vulva of a 24 year old woman is presented. This rare benign tumor appears as dermal or subcutaneous well circumscribed nodule. Histologically the fibroadenoma is characterized by epithelial and stromal proliferation. Two different concepts concerning histogenesis are cited; the authors believe that this tumor arises in ectopic mammary tissue.