Panacinar emphysema: CT and pathologic findings.

OBJECTIVE: To determine the ability to assess the presence and extent of panacinar emphysema with CT, two chest radiologists independently reviewed CT of 10 patients with pathologically proven panacinar emphysema and five normal controls. MATERIALS AND METHODS: Six of 15 cases had conventional 10 mm collimation CT, eight cases had conventional CT and high-resolution CT (HRCT), and one case had HRCT only. In each of the 15 cases a single CT image was compared with the inflated pathologic specimen cut in the transverse plane at the same level as the CT scan. Comparison was made between visual assessment of the severity of panacinar emphysema on CT and pathologic assessment of severity using the modified Thurlbeck panel score. RESULTS: The correlation between the assessment of extent of panacinar emphysema on CT and the pathologic grade was r = 0.90, p < 0.01 for conventional CT and r = 0.96, p < 0.01 for HRCT. There was less interobserver variation in the grading of emphysema on HRCT (mean 0.6, SD 3.9) than with conventional CT (mean 3.9, SD 11.0). Conventional CT correctly identified or excluded panacinar emphysema in 10 of 14 cases, whereas HRCT was correct in 6 of 9 cases. CONCLUSION: We conclude that HRCT allows improved correlation with the pathologic score and decreased interobserver variation than conventional CT in panacinar emphysema. However, emphysema may be missed on both techniques.

Imperforate anus in 700,000 consecutive liveborn infants.

We have studied the incidence of imperforate anus (anorectal atresia and stenosis) occurring in almost 700,000 consecutive liveborn infants in British Columbia (B.C.) from 1964-1982 using the records of a health surveillance registry which uses multiple sources of ascertainment. The estimated incidence rate was 1 in 2,524 live births (273 cases out of 689,118 consecutive liveborn infants). Data pertaining to sex ratio, additional anomalies, recurrence, and mortality were also analyzed over the period 1952-1983. Additional anomalies were common in infants with anorectal atresia and stenosis; approximately 6 out of 10 cases had anomalies outside the anorectal canal. Skeletal and limb anomalies were frequent, occurring in almost one-third of patients. Also common were genitourinary anomalies, occurring in one-third of patients. Details of associated anomalies occurring in these infants are given, and clinical implications arising from the study regarding the neonatal assessment of infants with imperforate anus are discussed.

Hirschsprung disease in a large birth cohort.

The incidence of Hirschsprung disease was studied in a series of almost 700,000 consecutive livebirths in British Columbia from 1964-1982, by means of the records of a health surveillance registry that uses multiple sources of ascertainment. The estimated liveborn incidence rate for Hirschsprung disease was 1 in 4,417 livebirths (156 cases out of 689,118 livebirths). Data pertaining to sex ratio, additional anomalies, recurrence, and mortality were also analyzed over the caseload period 1952 to 1983. A total of 29.8% of cases had some additional anomaly--the majority being nonregional anomalies in other systems or more distantly in the gastrointestinal tract. Cardiovascular and gastrointestinal anomalies not a direct consequence of Hirschsprung disease were the most frequent additional anomalies found, occurring in 10 and 12 of 178 cases, respectively. Sensorineural anomalies were also frequent, occurring in 12 of 178 cases. Clinical implications arising from the study regarding the neonatal assessment of infants with these anomalies are discussed.