Clinical features and surgical outcome of a suprarenal mass detected before birth.

PURPOSE: With the widespread use of the obstetrical ultrasound, identification of a fetal suprarenal mass has become more common. Most of these masses prove to be congenital neuroblastomas (CNB), but the diagnosis is often confused with other benign lesions and the postnatal management remains to be controversial. METHODS: The medical records of 18 patients that underwent primary surgical excision for an antenatally detected suprarenal mass, between January 1995 and April 2009, were reviewed. The clinical, radiological, surgical, and pathological data were collected. Staging evaluation was performed after histological confirmation of the CNB. RESULTS: There were 13 cases of CNB, 1 adrenal cyst, 2 adrenal hemorrhages, and 2 pulmonary sequestrations. The differential diagnosis was impossible before surgery. Most of the CNBs were stage I (N = 11), with 1 stage IV and 1 stage IV-S. Four patients (3 stage I and 1 stage IV-S) had more than one copy of N-myc gene. The stage I patients were cured by surgery alone, and stage IV patients underwent nine cycles of adjuvant chemotherapy and currently have no evidence of disease. The five benign lesions were cured with excision alone. There were no postoperative complications. CONCLUSION: For early diagnosis and treatment, surgical excision should be considered as the primary therapy for an antenatally detected suprarenal mass. The surgery can be safely performed during the neonatal period and provides a cure in most cases.

Prophylaxis of peripartum haemorrhage using recombinant factor VIIa (rfVIIa) in pregnant women with congenital factor VII deficiency: A case report and literature review.

Congenital factor VII deficiency is a rare autosomal recessive disorder associated to different haemorrhagic manifestations. Labour and delivery may cause bleeding risk in patients with this coagulation deficit, thus it is appropriate to clarify whether prophylaxis of peripartum haemorrhage is necessary. To date, there are very few cases in scientific literature which report the management of women with congenital factor VII deficiency during labour, and a consensus for prophylaxis does not exist. In this manuscript we present the management of a 35 years old woman with factor VII deficiency, treated with recombinant factor VIIa before delivery, without haemorrhagic complications either for the woman and for the infant. Therefore, we present a review of similar cases managed with a peripartum prophylaxis with recombinant factor VIIa, and discuss its usefulness and effectiveness, in view of the severity of the deficit and the doses used.

Inherited bleeding disorders: a 14-year retrospective study

Congenital bleeding disorders comprise a heterogeneous group of diseases that reflect abnormalities of blood vessels, coagulation proteins, and platelets. A 14-year retrospective study (1991-2005) was conducted for patients referred to the coagulation section of the Hematology Department (King Hussein Medical Center, Amman, Jordan), who had suffered from bleeding tendencies to assess the prevalence of bleeding disorders among Jordanians and to describe their clinical manifestations. Four hundred and three patients matched our criteria. All patients were screened with routine coagulation assays and a complete blood cell count; a factor assay was performed if indicated by the results of the screening assays. A total of 168 patients (41.6 per cent) were diagnosed with a bleeding disorder caused by a factor deficiency, of which 17.1 per cent were described as hemophilia A (n=69), 6.2 per cent were described as vWD (n=25), and 4.2 per cent were described as hemophilia B (n=17). A subset of the total patient population comprising 14.1 per cent of the patients were diagnosed with a Rare Inherited Coagulation Deficiency (RICD), where 4.0 per cent were FX deficient (n=16), 3.7 per cent were FVII deficient (n=15), 3.7 per cent were FV deficient (n=15), 2.5 per cent were FXI deficient (n=10), and 0.2 per cent were diagnosed with afibrinogenemia (n=1).PMID: 19174980 [PubMed - in process]