Long-term outcomes of children with umbilical vein varix diagnosed prenatally.

OBJECTIVES: The current study aims were to assess the long-term outcomes of children who were diagnosed with umbilical vein varix (UVV) prenatally. METHODS: The study included fetuses with UVV diagnosed in the community between the years 2005 and 2011. They all have been refereed to our Ultrasound Unit for diagnosis' confirmation. This has been conducted locally by a single operator. After delivery, they were matched by gestational age at birth with a set of newborns from a random list. Developmental delay was assessed by telephone interview using a questionnaire based on Ages and Stages Questionnaire. If the child's score (both in the study and control group) was below the cut-off in one or more domain(s), the families were offered an examination by a child developmental health care team. RESULTS: There was no perinatal mortality in both groups. A low-development score was found in 41.7% (15/36) and 3.7% (4/108) in UVV and control group, respectively (P < 0.05). In 10 out of 15 (67%) children in the UVV group with low scores, formal developmental assessment was performed. Four (40%) were diagnosed having developmental delay. Among the four controls with low score, two families refused additional assessment and one child died. The remaining child was found to have normal development. CONCLUSIONS: A possible association between UVV diagnosed prenatally and child developmental delay was found. However, the clinical implications of these findings are still premature; thus, additional studies are needed.

Rotation of the vermis as a cause of enlarged cisterna magna on prenatal imaging.

OBJECTIVES: Dandy-Walker complex is a continuum of developmental anomalies of the posterior fossa which includes vermian rotation. However, vermian rotation alone may be benign. The aim of this study was to describe our experience with sagittal-plane prenatal ultrasound in the diagnosis of rotation of the vermis in cases of suspected enlarged cisterna magna on routine antenatal imaging, and to describe the follow-up of these patients. METHODS: Seven women, who were referred to our ultrasound unit for evaluation of an enlarged fetal cisterna magna and suspected agenesis of the vermis on axial-plane imaging, underwent further multiplanar studies of the posterior fossa and measurements of the vermis. RESULTS: The mean maternal age was 27 (range, 20-33) years and the mean gestational age at diagnosis was 19.5 (range, 18-31) weeks. The standard axial plane image showed a 'direct communication' between the cisterna magna and the fourth ventricle. In the mid-sagittal plane, the vermis was clearly delineated, with posterosuperior rotation. Vermis size was within normal limits for gestational age in all cases. Findings were confirmed by prenatal magnetic resonance imaging (MRI) in two cases and postnatal MRI and/or sonography in five. During a mean follow-up of 4.5 (range, 1-7.5) years, all children developed normally, with no neurological complications. CONCLUSION: The finding of an enlarged cisterna magna on standard- (axial-)plane ultrasound should be evaluated further in the sagittal plane to determine whether the cause is rotation of a normal vermis. This may spare patients unnecessary tests, anxiety and, in some cases, pregnancy termination.

Small bowel obstruction related to left-side paraduodenal hernia: CT findings.

The role of computed tomography in the evaluation of patients with small bowel obstruction has been extensively described in the current literature. We report a rare case of small bowel obstruction related to a surgically proved left-side paraduodenal hernia detected on abdominal computed tomography.

Echocardiogram in sternal fracture.

We reviewed the records of 50 consecutive patients presenting with sternal fracture after blunt chest trauma. The relationships between electrocardiogram, creatine kinase MB isoenzyme and echocardiogram (ECHO) were assessed in reference to myocardial contusion. Eleven patients (22%) had 1 or more abnormal cardiac tests but only 3 (6%) had clinically symptomatic myocardial contusion. Seven (22.5%) out of 31 ECHO studies revealed pericardial effusion which was not associated with the severity of all other associated injuries. The incidence of an abnormal ECHO study in patients with isolated sternal fracture was similar to that of patients who suffered multiple injuries. In 5 patients with pericardial effusion, this finding was not associated with any other abnormal tests and was regarded as of no clinical cardiac significance. On the other hand, a myocardial contusion was shown by ECHO examination only while echocardiogram and creatine kinase MB tests remained normal in that patient. We suggest that the ECHO should be used as a diagnostic and triage tool in every patient with sternal fracture associated with other injuries. However, patients with "benign" pericardial effusion can be discharged as soon as their medical condition improves regardless of other associated injuries.

Nuchal translucency measurement and pregnancy outcome after assisted conception versus spontaneously conceived twins.

BACKGROUND: Nuchal translucency (NT) measurement for Down's syndrome screening or detecting various fetal anomalies is a reliable sonographic marker. This study evaluates the contribution of NT screening in spontaneously conceived and assisted conception twin pregnancies. METHODS AND RESULTS: Maternal age at measurement, chorionicity, ultrasound features, karyotype results and pregnancy outcome were recorded prospectively and compared in 83 assisted reproduction treatment and 91 spontaneously conceived twins. Pregnancy outcome was evaluated according to maternal age, method of conception, NT data and chorionicity. NT measurements (> or =95 centiles of the normal range) were considered screen-positive and mid-pregnancy fetal karyotyping was advised. Complicated pregnancy outcome, which could be signalled by increased NT, was defined as either chromosomal abnormalities, severe structural defects or fetal demise. Based on NT measurements, 16 fetuses (4.6%) were found to be screen-positive. Five of them had chromosomal aneuploidy and selective termination was performed. The parents also opted for this procedure in another five fetuses because of major structural abnormality diagnosed during NT assessment. No other chromosomal or major fetal abnormality were found post-natally. Although no difference was found in NT, crown-rump length and maternal age between spontaneous and assisted reproduction technology twin pregnancies, the former group had a significantly higher rate of screen-positive results (7 versus 2%, P = 0.047), amniocentesis uptake (33 versus 22%, P = 0.014), monochorionic twining (32 versus 4%, P = 0.001) and complicated pregnancy outcome (11 versus 5%, P = 0.02). CONCLUSION: The present study confirms that first trimester target scanning can improve outcome by early detection and management of cases with an anomalous co-twin. It also identifies some differences between spontaneously and artificially conceived twin pregnancies in relation to this area of testing.

[Primary gastrointestinal stromal tumors].

UNLABELLED: Stromal tumors of the GI tract are rare. In the retrospective and prospective study we investigated the relationship between tumor symptomatology, tumor grade and prognostic factors. During the period May 1993-September 1999, 11 female and 13 male patients with a mean age of 62 (range-29-81) years were operated for primary gastrointestinal stomal tumors (GIST) in our department. Observed signs and symptoms were: GI bleeding (65%), abdominal pain (45%), abdominal mass (15%) and weakness (5%). In 4 patients tumor was an incidental finding during investigation or operation for another tumor. Tumor location (in decreasing order) was: stomach (15), small bowel (SB, 6), esophagus (1), duodenum (1) and colon (1). Preoperative biopsy or FNA were diagnostic in less than 50% of the cases. Operative procedures included wedge resection (8 patients), resection of segment of bowel (10) and extended resection (6), of diaphragm, SB, colon, bladder, kidney and liver. The mean tumor size was 7.8 (range-0.9-22) cm. Four tumors were graded as benign, 8 of indeterminate malignant potential and 12 malignant. CONCLUSION: The main presentation of GIST is acute GI bleeding. Endoscopy is most effective for studying proximal tumors, and CT should be used to identify distal GI tract tumors. Tumor size or malignancy were not necessarily predictive of GI bleeding. When invasive to adjacent organs is present, wide excision should be contemplated as long-term survival can be achieved.

Synchronous bleeding of liver adenomatosis and possible relation to acoustic trauma.

BACKGROUND: Solitary hepatic adenoma is a rare tumour but adenomatosis of the liver is even less common; it has been defined arbitrarily as more than four adenomas within otherwise normal hepatic parenchyma. Usually asymptomatic, the main clinical presentation is abdominal pain secondary to bleeding from the tumour. CASE OUTLINE: A 40-year-old woman admitted for abdominal pain and operated within a few hours due to circulatory instability was found to have synchronous bleeding from three out of four liver cell adenomas. The onset of pain followed shortly after exposure to particularly loud music. The three bleeding tumours were enucleated. The fourth lesion was left in situ; during outpatient follow-up it decreased in size and eventually disappeared. DISCUSSION: Although rupture with bleeding is a well-known presentation of liver cell adenomas, synchronous bleeding from more than one tumour is exceedingly uncommon and this may be the first reported case. Enucleation of the tumour is easy to perform even during active bleeding. Small asymptomatic tumours may be followed without surgical intervention.

Prognostic factors and early resumption of cyclosporin A in renal allograft recipients with thrombotic microangiopathy and hemolytic uremic syndrome.

Biopsy-proven thrombotic microangiopathy (TMA) was found in 22 of 436 (5%) renal transplant recipients, with similar incidence in recipients of cadaver or living related allografts. All patients with TMA presented different degrees of severity of the hemolytic uremic syndrome (HUS). Prognosis was poor when HUS occurred shortly after transplant in recipients of cadaveric kidneys (55% graft loss). It was more favorable when HUS occurred later in the post-transplant course or in recipients with allografts from living related donors, irrespective of time of occurrence. Other factors such as extent of TMA, degree of thrombocytopenia, hemolysis or renal dysfunction were not predictive of graft loss. Cyclosporine was resumed in 14 of 16 recipients shortly after clinical recovery without recurrence of HUS. In conclusion, HUS carries poor prognosis when occurring shortly after transplant in cadaver kidney recipients. Once the graft function improves, cyclosporine can be safely resumed.

Main bronchial rupture from blunt trauma in a 2-year-old child.

The authors report on a 2 year old who sustained a bronchial rupture after a motor vehicle accident and was operated on soon after admission. This injury is very unusual in young children.

[Gas gangrene].

A 66-year-old woman presented with left lower abdominal pain and paresthesias of the left thigh of 6 hours duration. 10 years previously she had undergone massive abdominal irradiation for retroperitoneal lymphoma. 3 days prior to admission she underwent rectal biopsy as part of a work-up for suspected inflammatory bowel disease. Within a few hours she developed fulminating gas gangrene of the left thigh and lower abdomen. In spite of aggressive surgical debridement she died 12 hours after admission. The incidence of gas gangrene is decreasing, but the mortality is still high. The diagnosis requires a high index of suspicion, especially in immunodepressed patients after invasive procedures or even minor trauma.