The dimensions of the triangle of Koch in children.

We measured the dimensions of Koch's triangle in children with normal intracardiac anatomy to determine the relation between the size of the triangle of Koch and patient age, weight, height, and body surface area. We found that the dimensions of Koch's triangle varies significantly and directly with patient age and body habitus in this pediatric population.

Collagen content in normal, pressure, and pressure-volume overloaded developing human hearts.

Increased myocardial collagen accompanies pressure overload of the adult left ventricle. This phenomenon is poorly understood in infants. This study compares the myocardial volume fraction of collagen in infants who did not have primary heart disease with infants with isolated pressure overload of the right ventricle (tetralogy of Fallot [ToF]), and with infants with combined volume and pressure overload (aortic valve atresia [AVA]). The distribution of collagen in the neonatal myocardium was also determined. We measured the volume fraction of collagen from right ventricular biopsy specimens of cadaver hearts in normal infants (1 to 9 months old; n = 7), infants with ToF (1 day to 9 months old; n = 9), newborns with AVA (AVA-NB) (1 to 4 days old; n = 5), and older patients with AVA (AVA-I) (5 to 8 months old; n = 5). Myocardium from 3 patients undergoing repair of ToF (6 to 8 months old) was also analyzed. Specimens were stained with Masson's trichrome and myocardial volume fraction of collagen determined by point counting. Myocardial volume fraction of collagen was significantly higher (p = 0.02) in AVA-I patients (8.0 +/- 3.5%) versus normal (3.3 +/- 2.7%), ToF (3.2 +/- 1.8%), and AVA-NB (3.5 +/- 2.3%) patients. There was a tendency for increased collagen in the subendocardium, especially in AVA-I patients (p > 0.05). We conclude that patients with AVA-I have increased collagen relative to normal subjects, patients with ToF, and patients with AVA-NB, and that this increase is greatest in the subendocardium.

Craniomicromelic syndrome: a newly recognized lethal condition with craniosynostosis, distinct facial anomalies, short limbs, and intrauterine growth retardation.

We report on two sisters with an unusual form of craniosynostosis, protruding nasal spine, micrognathia, short limbs, lung hypoplasia, absent or hypoplastic gallbladder, short intestine with ileal distention, hypoplastic uterus, and intrauterine growth retardation. This combination of defects appears to be a newly recognized and probably autosomal recessive disorder.

Intracytoplasmic inclusion bodies in the chondrocytes of type I lethal achondrogenesis.

Lethal achondrogenesis in the past has been frequently confused with achondroplasia. Clinical and radiologic advances in the last decade have led to clear differentiation of this condition from other types of bone dysplasia. It is further separated into two types on the basis of radiographic and pathologic findings. Re-evaluation of the histologic features has led to the recognition of heretofore unrecognized intracytoplasmic inclusion bodies in the chondrocytes of type 1 achondrogenesis. The finding of inclusions strengthens the differentiation of the two types and may have implications for the pathogenesis of the form of chondrodystrophy.

Liver fibrosis (cardiac cirrhosis) five years after modified Fontan operation for tricuspid atresia.

A 15-year-old girl was found to have severe liver fibrosis on liver biopsy at the time of cholecystectomy, 5 1/2 years following a modified Fontan procedure (right atrial-right ventricular conduit) for tricuspid atresia. Postoperative right atrial pressures were consistently elevated above 13 mm Hg and this, in part, may have been due to progressive mild conduit stenosis. Because of increasing symptoms, the patient underwent successful revision of the conduit at the age of 15 years. It is suggested that sustained systemic venous hypertension caused the striking morphologic changes in the liver and that this serious complication may significantly affect the long-term prognosis of patients surviving the Fontan procedure.

Upper cervical myelopathy in achondroplasia.

Two achondroplastic infants had small foramina magna with lethal compression of the corresponding upper cervical spinal cords. The damage was histologically comparable to the hyperextension type of spinal cord injury. An anterior displacement of the foramen magnum was demonstrated in one of the two cases. The displacement is considered worthy of future verification as it may be an additional factor in susceptibility to hyperextension injury to the spinal cord. The second patient survived 6 months, demonstrating that not all upper cervical spinal cord injuries are immediately fatal. Patients who have sublethal lesions are potentially salvageable by surgery. It is also speculated that there might be more cases of nonlethal upper cervical spinal-cord damage in achondroplasia, especially in early infancy when infants are unable to hold their heads erect. The true incidence of slight upper cervical spinal cord damage is not known, although it is considered rare at present. As a result of the findings in these two cases, it is suggested that hyperextension of the head be avoided, when possible.

Perinatal infection with Torulopsis glabrata: a case associated with maternal sickle cell anemia.

Torulopsis glabrata is a yeast of low virulence and commensal within the female genitourinary tract. The first case of congenital infection with Torulopsis glabrata was reported in 1980. An additional example of perinatal Torulopsis infection which is associated with maternal sickle cell anemia is reported. The most common source of congenital fungus infection is Candida albicans. Differences in morphology between T glabrata and C albicans are apparent, particularly the pathologic response within the placenta. Pathogenicity in this case may be associated with increased susceptibility to infection in patients with sickle cell anemia.

Prolonged extracorporeal life support of pediatric and adolescent cardiac transplant patients.

Options for mechanical support of pediatric patients with severe heart failure who are awaiting transplantation or have undergone transplantation are limited. This report examines 3 patients placed on extracorporeal life support (ECLS) while awaiting transplantation and 3 patients who underwent transplantation and suffered subsequent heart failure due to rejection or postoperative myocardial dysfunction. The overall survival rate was 2 of 6. The 2 surviving patients had a failing transplanted heart. There were no survivors among the patients placed on ECLS as a bridge to transplantation. In each case a contraindication to transplantation developed before a donor heart could be obtained. The mean time of ECLS support was 147.5 hours (range, 70 to 370 hours). The ECLS circuit did not affect cyclosporin levels or antirejection therapy. Extracorporeal life support can be used to support pediatric cardiac transplant patients with biventricular failure due to acute rejection or postoperative dysfunction. Although the results have been discouraging, ECLS may still have a role as a bridge to transplantation. However, complications can develop during ECLS that may preclude transplantation.

Echocardiographic indexes of allograft rejection in pediatric cardiac transplant recipients.

To determine the usefulness of echocardiographic indexes of left ventricular (LV) function as possible predictors of cardiac rejection, 12 transplant recipients (ages 3 to 17 years) underwent a total of 52 serial echocardiographic examinations and cardiac biopsies. The results were compared to those of 12 normal children (ages 2 to 17 years). Biopsies were graded as no rejection (n = 23), mild rejection (cellular infiltrate, n = 13), and moderate rejection (myocyte necrosis, n = 16). LV dimensions, percent shortening fraction, indexed LV mass, and ejection fraction were measured from M-mode and two-dimensional echocardiography. From the mitral valve Doppler tracing, the following measurements were made: isovolumic relaxation time, peak E and peak A velocities, and the fraction of filling under the E and A waves as well as in the first third of diastole. Compared with normal subjects, transplant recipients with no rejection had higher heart rates (95 +/- 15 vs 80 +/- 17 beats/min), longer isovolumic relaxation time (68.8 +/- 11.2 vs 51.5 +/- 13.6 msec), decreased first third area fraction (0.48 +/- 0.10 vs 0.57 +/- 0.10), and similar shortening fraction, LV mass, and peak E and A velocities (p less than 0.03). Compared with transplant recipients with no rejection, patients in whom mild rejection developed also had decreased shortening fraction (31% +/- 10% vs 37% +/- 8%) and decreased peak E velocity (0.68 +/- 0.19 vs 0.88 +/- 0.15 m/s) (p less than 0.03). From mild to moderate rejection, no further changes were noted in any echocardiographic indexes measured.(ABSTRACT TRUNCATED AT 250 WORDS)

A simplified approach to diagnostic rectal biopsy in infants and children.

Ninety-four patients underwent rectal biopsy with a suction capsule during a two year period. This painless procedure was performed in seventy-one patients for diagnosis of aganglionosis (Hirschsprung's disease). In nine patients, no ganglion cells were encountered, and subsequent surgery confirmed the diagnosis of Hirschsprung's disease in each of these patients. In twenty-three patients, biopsies were taken to search for neuronal storage disease. Six specimens contained abnormal material in the cytoplasm of the ganglion cells.

Alpha-1-antitrypsin deficiency: a review; 1963-1975.

Alpha-1-antitrypsin (A1AT) deficiency originally was recognized as a biochemical abnormality in patients with pulmonary emphysema. Studies of the protein nature of A1AT during attempts to help explain the disease, led to the recognition of a protein polymorphism which expressed itself in several different phenotypes. As investigative work progressed, the spectrum of diseases associated with a deficiency of A1AT increased. Methods for determination of quantitative levels have been developed but these have proved lacking for the discrimination of the various phenotypes, for which specialized testing is necessary. Of the clinical states associated with deficiencies of A1AT, pulmonary emphysema and hepatic disease are the best defined. Pathogenetic implications, however, remain elusive. As a consequence, preventive aspects are debatable and the usefulness of the determination of A1AT levels as screening measures is controversial.

Diagnosis and management of congenital cystic disease of the lung in children.

Pulmonary sequestration, congenital adenomatoid malformation (CAM), congenital lobar overinflation (CLO), and bronchogenic cysts are four congenital lesions that may present as abnormal cystic areas within the pleural cavity in early life. They share similar clinical and embryologic characteristics, are frequently difficult to diagnose, and all require surgical treatment. From December 1974 to January 1985, 22 patients were operated upon on the Pediatric Surgical Service for congenital cystic disease of the lung. There were eight females and 14 males, ranging in age from 1 day to 18 years; 12 were under 6 months of age, and 14 were under one year. There were 7 CAMs, 5 extralobar pulmonary sequestrations, 5 intralobar pulmonary sequestrations, 3 bronchogenic cysts, and 3 cystic lobes caused by CLO. One patient had bilateral pulmonary sequestrations, intralobar on one side and extralobar on the other, with both sharing a common systemic artery arising from the infradiaphragmatic aorta. All except two asymptomatic patients presented with either progressive respiratory distress or recurrent pulmonary infections, and had cystic changes noted on chest roentgenograms. The differentiation of these congenital lesions from resolvable inflammatory cysts is important. Diagnosis was aided by selective utilization of barium contrast studies, ultrasonography, computed tomography, and arteriography. Operation consisted of resection alone of the extralobar pulmonary sequestrations and bronchogenic cysts, and total lobectomy in CAM, intralobar sequestration, and CLO. One infant with CAM died 1 day postoperatively from bilateral hypoplastic lungs. The other 21 patients are alive and well with follow-up ranging from 1 month to 9 years, with a mean of 3.5 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Historical changes in the postoperative treatment of appendicitis in children: impact on medical outcome.

BACKGROUND/PURPOSE: The introduction of managed care in the 1980s caused increased pressure to reduce costs for hospitalized patients. The authors hypothesized that these market forces have resulted in a decreased hospital stay and utilization of sophisticated diagnostic testing in children treated for appendicitis. If true, the impact of this paradigm shift on patient outcome is unknown. METHODS: Hospital records for 913 pediatric patients treated for appendicitis from 1974 to 1998 were reviewed retrospectively. Patients were stratified into those with perforated appendicitis (PA) and nonperforated appendicitis (NPA). Demographics, perioperative hospital course, diagnostic testing, complications, and long-term outcomes were analyzed after stratification into time intervals. RESULTS: Over time, children with NPA were treated with shorter antibiotic courses (P<.05) and were placed on a regular diet earlier (P<.05). These changes in treatment resulted in an earlier discharge (P<.05). The amount of time to become afebrile with a normal white blood cell count (WBC) did not change over time. Children with PA exhibited similar results with shorter antibiotic courses (P<.05), earlier dietary intake (P<.05) and earlier hospital discharge (P<.05) over time. In all children with appendicitis there was no significant difference in the rate of wound infections, abscesses requiring drains, readmission, or reoperations overtime. The utilization of abdominal radiographs (83%) and ultrasonography (USN; 40%) was high and remained unchanged over time. Utilization of computed tomography (CT scan) was low (4.3%) in the early decades and was not used as a preoperative test from 1991 to 1994. Given the high diagnostic accuracy of a pediatric surgeon for this disease, Bayesian analysis indicates that USN utilization rates should be 15%. CONCLUSIONS: The market pressures of managed care have resulted in a new treatment paradigm with an earlier discharge of all children with appendicitis. There has been no concomitant increase in the complication rate in either group as a result of this paradigm shift. Bayesian analysis indicates that USN and abdominal radiographs are overutilized in our institution.

Enterocolitis associated with Hirschsprung's disease: a clinical histopathological correlative study.

Enterocolitis associated with Hirschsprung's disease (HEC) remains a major source of morbidity and even mortality, both before and after definitive surgical treatment. This study was undertaken to investigate whether histopathologic mucosal changes, in the absence of clinical manifestations of HEC, could predict the subsequent development of this complication. The clinical data and histopathology of 25 patients who eventually developed clinical HEC were compared with a control group of 25 age- and sex-matched patients with Hirschsprung's disease (HD) but with no clinical HEC either preoperatively or postoperatively. The histopathologic findings of tissue obtained by rectal biopsy or during laparotomy were graded from 0 to V according to severity and compared with the eventual clinical course. This study showed that (1) histopathologic findings of HEC appear to predict the clinical development, but not the severity, of future episodes of clinical HEC; (2) a histological grade of > or = II (> or = 2 crypt abscesses per high power field) should raise suspicion for subsequent occurrence of HEC, whereas a grade of > or = III (multiple crypt abscesses) places a child at high risk for development of clinical HEC; and (3) contrary to what is generally thought, histopathologic changes of HEC occur in both ganglionic and aganglionic segments. Based on these findings, it is recommended that histopathologic documentation of HEC and its grade should be an integral part of the tissue diagnosis of HD.

Gastric leiomyoblastoma in a child.

A gastric leiomyoblastoma can occur in any age group, but the prognosis and sex distribution are different in the child-adolescent group than in adults. In the adult, the lesion is generally considered to be benign, though in the child-adolescent group it may metastasize to regional lymph nodes. The possibility of a leiomyoblastoma is suggested by multicentric intramural gastric tumors discovered in a female child or adolescent who has had gastrointestinal hemorrhage.