Duplicated odontoid process: plain radiographic and CT appearance of a rare congenital anomaly of the cervical spine.

We describe a duplication of the odontoid process in a 6-year-old patient that included a partially fused midline ossicle on the anterior arch of C-1, fusion of the anterior lip of the foramen magnum and the arch of C-1, and an incomplete bony posterior arch of C-1.     

Utility of MR urography in children suspected of having ectopic ureter

Background

Conventional imaging modalities are limited in the assessment of complex lower urinary tract anomalies including ectopic insertion of ureters. MR urography can be useful in these situations.

Objective

To share our experience with MR urography in assessing lower urinary tract anomalies and to determine its accuracy in depicting ectopic ureters.

Materials and methods

We conducted a retrospective review of all MR urography examinations done between November 2007 and March 2013 to note the presence or absence of duplex kidneys and insertion of ureters. We reviewed patient charts, surgical findings and results of other investigations including cystoscopy with retrograde ureterogram in order to establish presence or absence of ectopic ureter. This served as a reference standard against which we compared MR urography results.

Results

Of 22 MR urography examinations (3 boys, 19 girls; age range 3–16 years, mean 9.2 years) performed during the study period, 19 were performed to rule out ectopic ureters, two to assess complex anatomy and one to rule out crossing vessel in ureteropelvic junction obstruction. MR urography showed ectopic ureter in 9/19 children; one proved to be a false-positive. MR urography correctly showed normal insertion in 7/19 children. In the remaining 3/19 children distal ureter could not be seen, hence insertion was indeterminate on MR urography. One of these children had an ectopic ureter on cystoscopy and surgery. Statistical analysis showed MR urography’s sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) to be 88.8–100%, 70–90%, 75–88.8% and 90–100% for the detection of ectopic ureter.

Conclusion

MR urography is highly accurate in the assessment of ectopic ureters. In incontinent girls, MR urography should be the method of choice for depicting or ruling out ectopic ureter.     

Magnetic resonance imaging of congenital,inflammatory, and infectious soft-tissue lesions in children

Magnetic resonance imaging has the advantages of multiplanar capability and high degree of tissue differentiation. It is useful for assessing the extent of soft-tissue abnormalities, such as vascular malformations, inflammatory and infectious processes, muscle disorders, and limb hypertrophy. Magnetic resonance imaging is sensitive to the presence of water and edema and is a good indicator for early diagnosis of inflammation and its level of activity. Fat-saturation techniques, including T2-weighted sequences and inversion recovery imaging, optimize diagnostic accuracy. T1-weighted images are good at defining the distribution and proportion of fat in the body, so they are useful in evaluating syndromes of the limbs, including vascular malformations, as well as lipoatrophy-lipodystrophy conditions. Magnetic resonance imaging provides guidance for efficient tissue biopsy. It allows comprehensive pretherapeutic assessment of soft-tissue vascular anomalies. It constitutes a good modality for following up the natural history of soft-tissue disorders during childhood.     

Do Systemic Corticosteroids Effectively Treat Obstructive Sleep Apnea Secondary to Adenotonsillar Hypertrophy?

To determine if pediatric obstructive sleep apnea syndrome (OSAS) caused by adenotonsillar hypertrophy (ATH) could be treated by a short course of systemic corticosteroids, we conducted an openlabel pilot study in which standardized assessments of symptomatology, OSAS severity, and adenotonsillar size were performed before and after a 5-day course of oral prednisone, 1.1 ± 0.1(± SE) mg/kg per day. Outcome measures included symptom severity, adenotonsillar size, and polysomnographic measures of OSAS. Selection criteria included age from 1 to 12 years, ATH, symptomatology suggesting OSAS, an apnea/hypopnea index (AHI) ≥ 3/hour, and intent to perform adenotonsillectomy. Only one of nine children showed enough improvement to avoid adenotonsillectomy. Symptomatology did not improve after corticosteroid treatment but did after removal of tonsils and adenoids. Polysomnographic indices of OSAS severity did not improve after corticosteroid treatment. After corticosteroids, tonsillar size decreased in only two patients, adenoidal size was only marginally reduced, and the size of the nasopharyngeal airway was not significantly increased. These results suggest that a short course of prednisone is ineffective in treating pediatric OSAS caused by ATH.     

Choanal and ileal atresia: a new syndrome or association?

Choanal atresia is a relatively common congenital malformation which is often associated with other anomalies. On the other hand, ileal atresia is very rare, mostly nonsyndromic and occasionally associated with other anomalies. The association of choanal and ileal atresia is unknown. Here we report the first instance of bilateral choanal atresia and ileal atresia in a full term male infant and describe the subsequent surgical treatment of both conditions. The association is unique and may represent a syndrome.     

WILMS TUMOR AND MULTICYSTIC DYSPLASTIC KIDNEY DISEASE

Purpose

There is ongoing controversy concerning the management of multicystic dysplastic kidney disease, particularly with regard to the potential for malignant transformation. Our report fuels the debate by adding the 2 youngest patients in whom malignancy was present from birth or developed subsequently.

Materials and Methods

Two well documented cases of malignancy associated with multicystic dysplastic kidney disease are presented in 2 female infants (5 and 3 months old). The 5-month-old female infant was followed for multicystic dysplastic kidney disease and had no evidence of tumor either antenatally or at birth. The 3-month-old presented with hypertension and interventricular septal defect. A renal tumor was present on initial ultrasound.

Results

Even though malignant degeneration is rare in multicystic dysplastic kidney disease, 9 cases have been reported in the literature so far. Of these cases 3 were Wilms tumor, 5 were renal cell carcinomas and 1 mesothelioma.

Conclusions

Our 2 cases lend support to the surgical management of multicystic dysplastic kidney disease, particularly as nephrectomy can now be performed in a day surgery setting with minimal morbidity. Only the risks of coexisting malignancy and possible malignant degeneration transformation are specifically addressed in this article. Other complications of multicystic dysplastic kidney disease such as hypertension, infection, abdominal pain, hematuria and persistent dysplastic renal tissue despite ultrasonographic resolution of multicystic dysplastic kidney disease are additional risk factors to be considered. A recommendation for nephrectomy in all cases of multicystic dysplastic kidney disease cannot be based only on these 2 cases. Several other factors must be weighed before making that decision.     

Spastic paraplegia,optic atrophy,microcephaly with normal intelligence,and XY sex reversal: a new autosomal recessive syndrome?

Two female sibs of first cousin Iranian parents were found to have the syndrome of spastic paraplegia, optic atrophy with poor vision, microcephaly, and normal cognitive development. Karyotype analysis showed a normal female constitution in one and a male constitution (46,XY) in the other. The XY female showed normal female external genitalia, normal uterus and tubes, and streak gonads. SRY gene sequencing was normal. We conclude that the present family probably represents a new autosomal recessive trait of pleiotropic effects including XY sex reversal and adds further evidence for the heterogeneity of spastic paraplegia syndromes as well as sex reversal syndromes.     

Occult fractures in preschool children

Five hundred consecutive radiographic examinations of acutely limping infants and toddlers were analyzed retrospectively. One hundred of the 500 (20%) had a fracture as the underlying etiology. Although the most common sites of involvement were the tibia/fibula (56 cases) and femur (30 cases), fractures in the pelvis and feet, notably the metatarsals (11 cases), also were seen. We therefore recommend obtaining radiographs of the pelvis and both lower extremities including the feet, when occult trauma is suspected and the exact area of injury cannot be pinpointed clinically.