US of the pediatric female pelvis: a clinical perspective.

When investigating pelvic pathologic conditions in female pediatric patients, one needs to be aware of the developmental changes that take place around puberty. The prepubertal uterus is thin, with a fundus equal in size to the cervix. Owing to the hormonal stimulation of puberty, the uterus enlarges and the fundus becomes prominent. The ovaries are demonstrated with ultrasonography (US) at all ages. Ovarian volume increases after 6 years of age. Microcystic follicles are normally seen throughout childhood. US is the modality of choice for imaging the pediatric female pelvis. The main indications for pelvic US in the pediatric age group are pubertal precocity or pubertal delay, pelvic pain or pelvic masses, and ambiguous genitalia. Vaginal bleeding in the prepubertal child can be due to a vaginal foreign body, vaginal rhabdomyosarcoma, or precocious puberty. Common causes of primary amenorrhea in teenagers include gonadal dysgenesis (Turner syndrome) and müllerian (uterovaginal) anomalies. Pelvic pain or pelvic masses in pediatric patients can be due to ovarian torsion, hemorrhagic ovarian cyst, pelvic inflammatory disease, or ectopic pregnancy.

Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience.

UNLABELLED: Congenital cystic adenomatoid malformation of the lung (CCAM) is diagnosed by prenatal ultrasonography with an increasing frequency but controversy persists as to its prognosis and prenatal management. METHOD: A multi-institutional study of cases of CCAM diagnosed antenatally identified by ultrasonographers and by a review of hospital charts. RESULTS: We obtained 48 cases from five centers. We estimate the incidence of CCAM at 1:25,000 to 1:35,000 pregnancies. The incidence of voluntary abortions was 15% (7/48), of spontaneous abortions 2% (1/41) and of postnatal death 10% (4/40). One of the postnatal deaths was from trisomy 18. Of the 7 aborted fetuses, 2 had multiple malformations and 1 had severe hydrops and oligohydramnios; the other 4 had a large mass with mediastinal displacement but without hydrops. When pregnancy was allowed to continue, 56% of the lesions regressed spontaneously, even though one third of these had initial progression. In 17 cases (42%) the mediastinal shift corrected itself, sometimes by simple growth of the fetus but most often by a decrease in the size of the lung mass. In 1 fetus, repeated needle decompressions followed by double-pigtail catheter drainage of large cysts allowed regression of hydrops. Despite this, neonatal death occurred from pulmonary hypoplasia. CONCLUSION: CCAM can lead to fetal or neonatal demise from hydrops, lung hypoplasia, prematurity or severe associated malformations, but has a good prognosis in the majority of cases.

Preoperative manual detorsion of the spermatic cord with Doppler ultrasound monitoring in patients with intravaginal acute testicular torsion.

OBJECTIVE: To assess the effectiveness of preoperative manual detorsion in acute testicular torsion. MATERIALS AND METHODS: Between June 1998 and March 1999, seven patients presenting with testicular torsion underwent manual manipulation under US monitoring in order to restore the flow to the testis prior to surgery (orchidopexy). All detorsions were lateral in direction. The success of the manoeuvre was assessed both clinically and sonographically and confirmed at surgery. RESULTS: Six manoeuvres were successful in restoring flow to the testis. The failed attempt in the seventh patient was due to failure to manipulate beyond an initial 1 1/2 rotations (540 degrees). CONCLUSIONS: Preoperative detorsion is the fastest way to relieve testicular ischaemia. However, manual detorsion of the spermatic cord is not a substitute for surgical exploration and bilateral orchidopexy is still necessary.

Liver disease in children with cystic fibrosis: US-biochemical comparison in 195 patients.

PURPOSE: To determine if abnormal liver architecture at ultrasonography (US) is related to abnormal function in children with cystic fibrosis (CF). MATERIALS AND METHODS: For 1 year, all 195 children (112 boys, 83 girls; mean age, 8.5 years) attending a CF clinic underwent abdominal US and a standard set of liver function tests. Aspartate aminotransferase, alanine aminotransferase, and gamma-glutamyltransferase levels were analyzed. US signs were interpreted as follows: hypoechogenicity with prominent portal tracks as edema, hyperechogenicity as steatosis, and increased attenuation and nodules within or at the edge of the liver as cirrhosis. Signs of portal hypertension also were sought. US signs were compared with liver function test results. RESULTS: Liver sonograms were abnormal in 38 children (19%); of these, 24 (63%) had abnormal test results. The 157 children with normal liver architecture had a much lower prevalence of biochemical abnormality (33 patients [21%]; P < or = .001). All eight children with signs of portal hypertension had abnormal test results. Fourteen (82%) of 17 children with signs of cirrhosis had abnormal liver function. Eight (57%) of 14 patients with signs of steatosis had abnormal function. Diffuse hypoechogenicity of the liver with prominent portal tracks in 16 patients was associated with abnormal function in only five patients. CONCLUSION: The relation between abnormal liver architecture at US and results of three liver function tests in children with CF was significant. The most specific US abnormalities related to abnormal function are signs suggestive of portal hypertension and cirrhosis.

Soft-tissue hemangiomas in infants and children: diagnosis using Doppler sonography.

OBJECTIVE: We describe the sonographic appearance and vascularization of hemangiomas and determine if vessel density and peak systolic Doppler shifts distinguish hemangiomas from other superficial soft-tissue masses. SUBJECTS AND METHODS: Our pilot study included 20 infants and children with hemangiomas who were to undergo biopsy before treatment with interferon alpha-2b. We used Doppler sonography to determine the number of vessels per square centimeter, peak arterial Doppler shift, resistive index, and signs of arteriovenous shunting. All hemangiomas showed high vessel density (more than five per square centimeter) and high Doppler shifts (more than 2 kHz), and these two factors became our diagnostic criteria. A prospective study of 116 patients was then carried out. One hundred sixteen consecutive pediatric patients with superficial soft-tissue masses were examined using Doppler sonography; sonographic findings were compared with the final diagnoses that were established by biopsy, CT, or clinical follow-up. RESULTS: The final diagnoses included 70 hemangiomas, 20 venous malformations, three arteriovenous malformations, three arteriolocapillary malformations, and 20 other masses. Fifty-nine lesions showing high vessel density (more than five per square centimeter) and a peak arterial Doppler shift exceeding 2 kHz were correctly diagnosed as hemangiomas (sensitivity, 84%; specificity, 98%). One arteriovenous malformation showed high vessel density and high Doppler shifts, but none of the other masses that were not hemangiomas did. Eleven patients with hemangiomas who were being treated with interferon at the time of the study fulfilled only one of the two diagnostic criteria. CONCLUSION: High vessel density and high peak arterial Doppler shift can be used to distinguish hemangiomas from other soft-tissue masses.

The natural history of sacrococcygeal teratomas diagnosed through routine obstetric sonogram: a single institution experience.

BACKGROUND/PURPOSE: The antenatal diagnosis of sacrococcygeal teratoma (SCT) is increasingly being made with fetal sonography. However, the natural history of SCT detected on routine obstetric sonogram is not well defined. METHODS: A retrospective chart review of 21 fetal SCT diagnosed on routine sonography at Hospital Sainte-Justine between 1980 and 1997 were performed. The patients' clinical and sonographic characteristics, prenatal, and perinatal outcomes were examined. Prognostic criteria were identified by correlating patients' characteristics with outcome. RESULTS: In utero mortality rate was 19% and perinatal mortality rate was 14%. The incidence of premature labor was 50%. Of the various criteria examined as an independent variable, the presence of a solid tumor was found to be a important negative prognostic factor with a 67% incidence of death in utero and an overall mortality rate of 100%. Patients with new onset polyhydramnios were at significant risks for premature labor (100%). All of the perinatal deaths were attributable to tumor rupture. CONCLUSIONS: The course of sacrococcygeal teratoma diagnosed on routine sonograms is associated with a higher-than-expected incidence of prenatal and perinatal complications. Close antenatal follow-up for new onset polyhydramnios and the presence of a completely solid tumor will help optimize patient counseling and treatment.

Excess of congenital abnormalities in French-Canadian children with neuroblastoma: a case series study from Montréal.

Neuroblastoma is one of the most common cancers of childhood. Some studies have shown an excess of congenital abnormalities in children who have been diagnosed with neuroblastoma. In this study we examined the medical records of all children with neuroblastoma seen at St. Justine Children's Hospital between the years 1977 and 1993. A total of 141 children (131 of French-Canadian ancestry) were included in this study. Twelve children (8.5%) had 21 defined congenital abnormalities (1,490 per 10,000 children). This compared with a rate of 444.3 children with abnormalities per 10,000 live births (4.44%) for all congenital abnormalities in the British Columbia Health Surveillance Registry, 1979-1988 (relative risk = 1.91, P = 0.03). Six of the 12 children had cardiovascular malformations. These and previous results suggest that there may be a common developmental origin to neuroblastoma and to some congenital malformations. Genes that control development may be worthy of further study in these children.

Liver transplantation: review of the literature. Part 2: Vascular and biliary complications.

The first attempted human orthotopic liver transplantation, in 1963, involved a child with biliary atresia, who died on the operating table as a result of uncontrollable coagulopathy. Improvements in immunosuppression, surgical technique, medical imaging and postoperative care, as well as more stringent patient selection, have allowed the development of liver transplantation and its universal acceptance as the treatment for a variety of liver diseases. The radiologist plays a major role in the multidisciplinary transplantation team and must be familiar with each stage of orthotopic liver transplantation and its associated complications. In the first article of this series (Can Assoc Radiol J 1997;48[3]:171-178), the authors reviewed the anatomic features and current concepts relevant to orthotopic topic liver transplantation. In this, the second article, they discuss the vascular and biliary complications of the operation, and the third article will cover the medical complications.

Liver transplantation: review of the literature. Part 1: Anatomic features and current concepts.

The first attempted human orthotopic liver transplantation, in 1963, involved a child with biliary atresia, who died on the operating table as a result of uncontrollable coagulopathy. Improvements in immunosuppression, surgical technique, medical imaging and postoperative care, as well as more stringent patient selection, have allowed the development of liver transplantation and its universal acceptance as the treatment for a variety of liver diseases. The radiologist plays a major role in the multidisciplinary transplantation team and must be familiar with each stage of orthotopic liver transplantation and its associated complications. In the first article of this series, the author reviews the anatomic features and current concepts relevant to orthotopic liver transplantation. Future articles will discuss the vascular, biliary and medical complications of the operation.

Liver transplantation: review of the literature. Part 3: Medical complications.

The first attempted human orthotopic liver transplantation, in 1963, involved a child with biliary atresia, who died on the operating table as a result of uncontrollable coagulopathy. Improvements in immunosuppression, surgical technique, medical imaging and postoperative care, as well as more stringent patient selection, have allowed the development of liver transplantation and its universal acceptance as the treatment for a variety of liver diseases. The radiologist plays a major role in the multidisciplinary transplantation team and must be familiar with each stage of orthotopic liver transplantation and its associated complications. In the first article of this series (Can Assoc Radiol J 1997;48[3]: 171-178), the authors reviewed the anatomic features and current concepts relevant to orthotopic liver transplantation. In the second article (Can Assoc Radiol J 1997;48[4]: 231-242), they discussed the vascular and biliary complications of the operation. This, the third and final article in the series, covers the medical complications.

Imaging features of type 1 hereditary tyrosinemia: a review of 30 patients.

Hereditary tyrosinemia type 1, a common genetic disorder in the province of Quebec, is characterized by a deficiency of fumarylacetoacetate hydrolase. In this autosomal recessive disorder of tyrosine metabolism, the accumulation of succinylacetone leads to neurologic crises, acute and chronic liver failure, complex renal tubulopathy, rickets and a hemorrhagic syndrome. Liver trans- plantation has dramatically modified the spontaneous course of this lethal disease. The present paper describes the imaging features of tyrosinemia in 30 patients followed from 1980 to 1995 at Hôpital Sainte-Justine, Montreal, Canada.

Fetal congenital diaphragmatic hernia: accuracy of sonography in the diagnosis and prediction of the outcome after birth.

OBJECTIVE: This study evaluated the diagnostic accuracy of prenatal sonography in congenital diaphragmatic hernia (CDH) and assessed sonographic predictors of postnatal outcome. MATERIALS AND METHODS: Sonograms and medical records of 43 fetuses with CDH were retrospectively reviewed. Sonographic features were correlated to clinical evolution and surgical and pathologic findings. RESULTS: CDH was diagnosed prenatally with sonography in 40 cases. Intrathoracic stomach and liver were routinely identified. Ipsilateral lung tissue could not be differentiated from herniated content. Contralateral lung was identified in all cases except two. The overall survival rate was 43% after excluding terminated pregnancies. Besides associated malformations and chromosomal anomalies, the only statistically significant predictor of survival was the quantification of the contralateral lung area at the level of an axial four-chamber view: The survival rate was 86% when the contralateral lung area was equal to or greater than one half the area of the hemithorax. CONCLUSION: Sonography is highly accurate for prenatal diagnosis od CDH. Sonography also assists the prognostication of postnatal outcome in isolated CDH by allowing quantification of the contralateral lung area on a four-chamber view.

Appendicitis in children and young adults: Doppler sonographic-pathologic correlation.

OBJECTIVE: The purpose of this study was to determine the following: whether the arterial supply of a normal appendix is visible with Doppler sonography; whether the physiologic vasodilatation that accompanies childhood appendicitis is visible; what Doppler patterns appear once necrosis of the appendix has occurred; what Doppler shifts are visible with chronic, recurrent appendicitis; and whether other conditions in the right lower quadrant can mimic the Doppler sonographic patterns of appendicitis. MATERIALS AND METHODS: Twenty-five fasting patients without abdominal pain or intestinal disease and 45 patients (1-25 years old; mean, 8 years old) with right lower quadrant pain and suspected appendicitis were examined sonographically, using 5- and 7-MHz linear, color, and pulsed Doppler transducers. Arterial signals were sought within the appendix and neighboring tissues, counted, and classified as sparse (1-2), moderate (3-4), or abundant (>4). The resistive index (RI) was measured. Thirty patients who underwent surgery were retained for surgical and pathologic correlation, and only they formed the appendicitis study. RESULTS: A normal appendix was found in 10 patients. Doppler shifts were sparse, and diastolic flow was low or absent (RI, 0.85-1). Acute uncomplicated appendicitis was found in 13 patients, who had abundant color Doppler signals throughout most of the appendix, with high diastolic flow (RI, 0.40-0.77; mean, 0.54). Acute necrotic appendicitis with perforation was found in 11 children, eight of whom showed no signals in the necrotic area at the tip. Few or no signals were seen in the remainder of the appendix (RI, 0.33-0.90; mean, 0.54). In two patients, signals were abundant in the tissues surrounding the appendix. Recurrent or chronic appendicitis was found in three patients, previously diagnosed as having Crohn's disease, psychosomatic illness, or nonspecific abdominal pain. The appendix had Doppler signals confined to the tip (RI, 0.63-0.83; mean, 0.75). Other diagnoses were found in three patients, in whom the appendix was not seen. There were abundant color signals in the intestinal wall and adjacent tissues in the right lower quadrant. Two patients had Crohn's disease, and the third had an unsuspected early pregnancy. The appendix was normal in all. CONCLUSION: Acute appendicitis is accompanied by inflammatory hypervascularity reflected as an increased number of color signals and higher diastolic Doppler shifts as compared with those found in normal persons. No Doppler shifts are identified in areas of appendiceal ischemia. Other acute inflammation in the right lower quadrant also produces numerous Doppler shifts with high diastolic flow, as does ovulation. Care must be taken to identify the source of these Doppler signals. The changing vascularity of healing, recurrent, and chronic appendicitis promises to further our understanding of the pathogenesis and evolution of this disease.

Renovascular hypertension in children: curability predicted with negative intrarenal Doppler US results.

PURPOSE: To assess whether intrarenal Doppler ultrasound (US) enables prediction of the outcome of renovascular hypertension in a pediatric population. MATERIALS AND METHODS: A prospective study of 29 children with renin-mediated hypertension was performed. In these patients, intrarenal Doppler US was performed before angiography. Doppler US and angiographic findings were compared with patient outcome with respect to treatment required and blood pressure (BP) status after therapy. Cure was defined as a normal BP without medication. RESULTS: Intrarenal Doppler US was positive in 15 patients (52%), group 1, and negative in 14 patients (48%), group 2. In 10 patients (67%) in group 1, the severity of the vascular lesion precluded permanent cure. Cure was achieved in all patients in group 2, except for patients with neurofibromatosis. Thus, there was a statistically significant association (P < .05) between cure and a negative Doppler US result. CONCLUSION: In children with renovascular hypertension, a negative Doppler US result enables the prediction that a cure is more likely to be achieved with endovascular therapy or surgery.

New autosomal recessive form of amelia.

Amelia is a rare, usually sporadic malformation. We report on a family in which three fetuses had amelia of the upper limbs and variable deficiency of the lower limbs. The fetuses also had minor facial anomalies. Recurrence of the condition in sibs of both sexes suggests autosomal recessive inheritance. Recurrent amelia has been documented in only a few families most often associated with a different set of malformations. Possibly, mutations in more than one gene with different modes of transmission can lead to this severe limb deficiency. We speculate that the mutation found in our cases interferes with formation of the apical ectodermal ridge in the upper limbs and results in its premature degeneration in the lower limbs.

Improved survival for congenital diaphragmatic hernia, based on prenatal ultrasound diagnosis and referral to a combined obstetric-pediatric surgical center.

Between January 1990 and January 1993, 36 patients with antenatal ultrasound (US) diagnosis and/or postnatal diagnosis of congenital diaphragmatic hernia (CDH) were referred to the authors' high-risk obstetric and pediatric hospital. Among the 36, there were four spontaneous abortions (11%), five deaths after live births (14%), one false-positive US examination, and 26 patients who underwent surgery, 23 of whom survived (66% overall, 74% of live births, and 89% postoperatively). Only one survivor had extracorporeal membrane oxygenation (ECMO). Thirty-five ultrasound examinations were performed in 24 patients; there were 18 true-positives results (51%), one false-positive (3%), and 16 false-negatives (46%). In this series, there were 25 left-sided CDHs, eight right-sided, one bilateral, and one central. Of the four right-sided hernias having antenatal US, only one was diagnosed prenatally; 15 of the 17 left-sided CDHs were diagnosed correctly (88%). All 19 babies with the prenatal diagnosis were born at the authors' institution. US diagnosis before 25 weeks' gestation and polyhydramnios separately resulted in a mortality rate of only 50%. Patients born at this institution tend to be sicker than those transferred from elsewhere, as reflected by the lower 1- and 5-minute Apgar scores (3.7 v 6.9, P < .001, and 5.4 v 6.9, P < .16, respectively), lower gestational ages (37.0 v 39.2 weeks, P < .007), and lower birth weights (2,525 v 3,049 g, P < .02). Nevertheless, transferred patients had a mortality rate (3 of 15 patients, 20%) similar to that of nontransferred patients (5 of 20 patients, 25%).(ABSTRACT TRUNCATED AT 250 WORDS)

Renal vein thrombosis in children: evidence of early flow recovery with Doppler US.

PURPOSE: To characterize renal arterial and venous Doppler signals in pediatric renal vein thrombosis (RVT) and to study changes in the circulation in the months following the acute event. MATERIALS AND METHODS: Ten patients with proved acute RVT (eight infants and two boys with renal allografts) were studied with serial Doppler ultrasonography (US) of the inferior vena cava and the main renal and intrarenal veins and arteries. RESULTS: The resistance index in the affected vessel was increased by 10% compared with that of the normal side in patients with unilateral RVT. Only the allografts had reversed diastolic flow. Venous flow, which was detected around the thrombus and in intrarenal veins in all native kidneys, was absent during the 1st week in the transplanted kidneys but reappeared thereafter in one. Perirenal collateral vessels were demonstrated with color Doppler US in one baby. Seven of eight native kidneys atrophied, and the allografts failed. CONCLUSION: Classic Doppler signs of RVT--absent venous flow and reversed diastolic arterial flow within the kidney--were present in only the acute phase in transplanted kidneys. These signs are unreliable in RVT of native kidneys.