J. Laurance Hill

The diagnosis and management of pyriform sinus fistulae in infants and young children

Acute suppurative thyroiditis is rare in childhood. It presented in two clinically euthyroid children over a 2-year period. Repeated drainage for recurrent abscesses was necessary in one child. Thyroiditis without abscess was present in the second. After treatment and resolution of the infection, contrast studies with barium demonstrated an internal fistula extending from the left pyriform sinus to the ipsilateral thyroid lobe. Thyroid scans showed decreased uptake in the left lobe. Thyroid-function tests were normal. A third infant developed acute respiratory distress at 2 weeks of age from an enlarging left neck mass without evidence of infection. Exploration revealed an intrathyroid cyst with fistulous communication to the left pyriform sinus. Fistulas arise from the third pharyngeal pouch as a branchial remnant which is exclusively left-sided. Administration of antibiotics, which are effective against oral flora, combined with initial drainage of suppuration control infection. Only then can the diagnosis be made by contrast study of the hypopharynx and upper esophagus. Operative excision of the entire epithelial tract and adjacent thyroid tissue is essential to prevent recurrent thyroiditis and abscess.

Characteristics of pediatric firearm fatalities

An increase in the awareness of the problem of gunshot fatalities in children has occurred nationwide over the last year. Unintentional firearm deaths are more common among children and young adults. These deaths may be addressed by preventive measures beyond those available for pediatric firearm suicide and homicide. This study focuses on the incidence of fatal gunshot injuries in children under 16 years of age during the years 1979 through 1987 in our state. Over the past 9 years 132 children (0 to 16 years) were shot and killed as a result of firearm injuries (M:F ratio, 3:1). No difference in absolute number in racial distribution existed. Deaths were classified as homicide, 61 (46%); accidental, 33 (25%); suicide, 29 (22%); undetermined, 7 (5%); and other, 2 (1.5%). Over 70% of these fatal injuries occurred in the home environment. Types of weapons involved included handguns (48%), shotguns (22%), rifles (17%), unspecified weapon (12%), and air rifle (1%). In a large number of cases, guns were found in the home unsecured. The perpetrator was known by the victim in 64 instances, while unknown in 27. Thirty-nine self-inflicted wounds and two shotgun blasts in the line of fire accounted for the remaining deaths. The most common anatomical injury and ultimate cause of death was cranial central nervous system (CNS) (62%), followed by chest/mediastinum (20%), abdomen (10%), and other (8%). A child who has sustained a firearm injury is more likely to know the perpetrator, be killed in the home by a readily available unsecured firearm, and die from severe head injury.

Translocation (11;15;19): a Highly Specific Chromosome Rearrangement Associated With Poorly Differentiated Thymic Carcinoma in Young Patients

Thymic carcinoma is a rare epithelial neoplasm of the thymus. The presence of a specific chromosomal abnormality may augment diagnosis and therapeutic stratification. We report a 15-year-old boy diagnosed with thymic carcinoma who presented with a large anterior mediastinal mass, pleural effusion, and bone metastasis. The pleural fluid, cytology, bony lesions, and bone marrow were examined and chromosomal studies were performed. Histologic and immunohistochemical studies confirmed a poorly differentiated squamous cell type of thymic carcinoma. The karyotype of the pleural fluid at the time of diagnosis revealed a complex three-way translocation t(11;15;19)(p15;q12;p13.3). The constitutional karyotype was 46,XY. Five months after diagnosis, a bone marrow aspirate demonstrated tetraploidy with all translocation chromosomes in duplicate, as well as an unbalanced rearrangement involving chromosome 1: 92,XXYY,t(11;15;19)(p15;q12;p13.3)×2[15]/92,XXYY,idem,add(1)(qter)[5]. Despite aggressive multiagent chemotherapy, the patient’s condition progressed with bone marrow disease and he died 6 months after diagnosis. Several case reports of a similar chromosomal abnormality have been reported for thymic carcinoma in young patients with poor outcome. This karyotypic abnormality appears to mark a cohort of patients with thymic carcinoma who have a poor prognosis despite aggressive chemotherapy.

Clinical technique and success of the esophageal stent to prevent corrosive strictures

The Reyes technique for the management of severe, acute corrosive injuries of the esophagus has been modified and successfully applied to two children and one adult whose cases are reported. None developed stricture and two were uncomplicated. Esophagomegaly, motility dysfunction, upper gastrointestinal bleeding, and pneumonitis complicated the hospitalization of one child whose subsequent course has been asymptomatic. Until more experience can be recorded, widespread use is not yet recommended.

Motility abnormality in intestinal atresia

This study was designed to investigate the motility of the small bowel of the lamb under the conditions of experimental intestinal atresia. Of 26 fetal lambs operated upon (50 to 90 days gestation), 13 came to term. All term lambs exhibited a type 3a atresia. Six were successfully repaired and had intestinal myoelectric activity monitored for periods from 2 to 27 hours. A slow wave pattern (12 to 13/min) was present in the bowel of control lambs and in the proximal dilated bowel and the microbowel of atretic lambs, confirming the presence of functional smooth muscle. Spike potentials, which indicated circular muscular contractions, occurred 10% of the time in the control intestine, 5% in the proximal dilated gut (P less than .2), and 0% in the microbowel (P less than .001). A previously undescribed myoelectric transient of variable amplitude and frequency (6 to 24/min) was identified in all lambs studied. Histologic evaluation demonstrated villous hyperplasia in the microbowel and mucosal flattening in the distended bowel. The data suggest that the quiescent microbowel distal to the atresia contributes significantly to the functional obstruction.

Intestinal Aganglionosis: A Histologic And Acetylcholinesterase Histochemical Study

Varying results have been reported with the use of acetylcholinesterase (AchE) staining to diagnose Hirschsprungs disease in rectal suction biopsy. We analyzed the histology and AchE staining of rectal biopsies from 10 patients with documented intestinal aganglionosis and 57 patients with ganglionic bowel. The results show that histologic identification of submucosal ganglion cells is reliable in excluding Hirschsprungs disease and that the absence of ganglion cells in an adequate suction biopsy is highly suggestive of intestinal aganglionosis. Four AchE staining patterns were recognized; the staining patterns overlap in some patients who have and some who lack ganglion cells. The AchE staining pattern did not correlate with sex or age of the patients, or with the length of the aganglionic segment. The acetylcholinesterase stain is not a reliable method of making or excluding a diagnosis of intestinal aganglionosis except when AchE-positive fibers are increased in both the lamina propria and muscularis mucosae. This AchE staining pattern occurred in 6 of our 10 patients with Hirschsprungs disease. In addition, eight segments of aganglionic colon were studied that included 2 cases of total colonic aganglionosis in which hypertrophic, AchE-positive nerve fibers were absent in all layers of bowel wall. This last finding suggests that an abnormality in the preganglionic cholinergic fiber or extrinsic neuron is involved in the pathogenesis of this unusual form of total colonic aganglionosis.

Surgical intervention in children with human immunodeficiency virus infection

Twenty-one children with human immunodeficiency virus (HIV) infection required surgical intervention during the course of their disease. There were 11 females and 10 males (age range, 3 months to 6 years). The children underwent 54 operative procedures after diagnosis of their disease. These included placement of central venous catheter (23 patients), open lung biopsy (11), incision and drainage of perirectal abscess (4), incision and drainage of soft tissue abscess (5), myringotomy (2), diverting colostomy (3), Nissen fundoplication (1), and other (5). All 21 patients had clinical AIDS by the Centers for Disease Control CDC classification. To date, there have been 12 deaths in the 21 patients (57%) due to progressive deterioration with the patients disease. Most procedures were adjuncts for diagnostic and therapeutic intervention in a population of children with a uniformly fatal disease. The knowledge of various high risk groups for AIDS must heighten the surgeons awareness to the growing and significant pediatric segment of the HIV population, the complications of their disease, and the surgeons limited role in treating these problems.

Myoelectric activity differences in acute and chronic models of lamb intestinal atresia

Clinical and experimental investigations of intestinal atresia have reduced mortality and clarified etiology. However, the morbidity of this lesion remains excessive and its pathophysiology uncertain. The purpose of this project was to produce the first long-term model of intestinal atresia and thereby to study the motility of the adapting bowel. Intestinal atresia was induced in nine fetal lambs at midgestation by mesenteric avulsion. At term, this experimental group and eight control lambs underwent resection with anastomosis, decompression gastrostomy, and implantation of electrodes proximal and distal to the anastomosis. Vigorous resuscitation and constant intensive care were critical for survival. Slow wave activity (SWA) and spike potential activity (SPA) were recorded in the first 24 hours and every 48 hours thereafter for a 6-hour interval. Three of nine lambs with atresia and five of the eight control animals survived over 48 hours (chronic). Analysis of 220 hours of recording showed, in the first 24 hours of life, a statistically significantly different SWA of 16.4 cycles/min (+/- 1.15 SD) in the chronic atretic survivors compared with 13.1 cycles/min (+/- 2.6 SD) in the acute (died less than 48 hours) atretic group (P less than .05). The SWA of the chronic atretic survivors was similar to the SWA of the chronic nonatretic control animals, 15.1 cycles/min (+/- 1.4 SD). SPA was present on the first day of life in the microintestine of the chronic atresia group in contrast to the acute animals of this study and of Doolin and Hills previous study. Delayed function has been attributed to the absence of SPA in the microintestine of acute models.(ABSTRACT TRUNCATED AT 250 WORDS)

Does handgun legislation change firearm fatalities

When comparing firearm fatalities for children under 16 years of age before and after handgun legislation enactment in Maryland, firearm fatalities increased overall. A decrease in accidental deaths in the home perhaps reflects a response to public education and awareness. More aggressive handgun legislation is imperative to reverse this public health trend.

Hemolytic-uremic syndrome: initial presentation mimicking intestinal intussusception.

A case of hemolytic-uremic syndrome preceded by laparotomy for suspected intestinal intussusception is reported. The pathologic lesions in the colon are described; they are attributable to microangiopathic thrombosis. Their significance in the hemolytic uremic syndrome is discussed.