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Biomedical subjects

D A Gillis

Publications and source records attributed to D A Gillis.

At least 19 recordsLinked to original sources

Roux-en-Y jejunostomy in the pediatric population.

Surgical access for nutrition is required in a variety of pediatric disorders. In some, the presence of gastroesophageal reflux, poor gastric emptying, and risks for fundoplication favor the use of a jejunostomy. The significant problems associated with the simple loop jejunostomy can be avoided by using the Roux-en-Y configuration. The stoma can be fashioned either Brook-style (intubatable) or Stamm-style (modified Maydl, permanently intubated). Both types are used at the authors' institution and are compared in this retrospective review. During a 27-month period, 22 Roux-en-Y jejunostomies were performed; nine of them had the Brook-style stoma and 13 had the modified Maydl stoma. Significant complications requiring reoperation occurred in three (33%) patients with a Brook-style jejunostomy: prolapse, leakage, and perforation of the stoma. None of the patients with modified Maydl jejunostomies required reoperation; problems were encountered more with the care of the permanently intubated stoma. Therefore, our preferred choice for a feeding jejunostomy is the modified Maydl approach.

Adolescent

Survival in neonatal congenital hernia without extracorporeal membrane oxygenation support.

The experience with high-risk congenital diaphragmatic hernia (CDH) at an institution that does not offer extracorporeal membrane oxygenation (ECMO) was reviewed. Between January 1, 1983 and December 31, 1993, 38 children presented with Bochdalek-type CDH. Excluded were two infants with lethal cardiac anomalies and four presenting after 4 hours of age. Thus, the authors identified 32 high-risk patients. All had early respiratory distress and were intubated within 5 hours of birth. Sixteen were inborn; 16 were referred to the Izaak Walton Killam Children's Hospital (IWK) within 24 hours of birth. There were 19 males and 13 females. Three died before surgery could be attempted. Twenty-two survived, giving an overall survival rate of 69% (22 of 32). For 28 of the 32, the best preoperative postductal Pao2 (BPDPao2) was recorded. Fifteen of the 28 children had a BPDPao2 of greater than 100 mm Hg. Survival in this group was 14 of 15 (93%). Thirteen of the 28 patients had a BPDPao2 of less than 100 mm Hg. Survival in this group was 5 of 13 (38%). These survival rates are comparable to those of centers offering ECMO. BPDPao2 appears to be a useful discriminating variable.

Cause of Death

Omental-mesenteric inflammatory pseudotumor. Cytogenetic demonstration of genetic changes and monoclonality in one tumor.

BACKGROUND: Extrapulmonary inflammatory pseudotumor (plasma cell granuloma) is an uncommon lesion in adults and children, and little is known either of its etiology or clinical characteristics. However, it remains a significant source of morbidity to patients and confusion to clinicians. METHODS: Case reports are presented of three patients with intraabdominal inflammatory pseudotumor who recently underwent surgery. A review of the recent world literature is also presented. Clinical and laboratory characteristics of omental-mesenteric inflammatory pseudotumor are reviewed along with a discussion of its etiology. RESULTS: Cytogenetic data from cells of one patient show a derivative chromosome evolved from a translocation between the long arm of chromosome 2 and the short arm of chromosome 9 [(2;9)(q1,3;p2,2)]. CONCLUSIONS: The lesion is monoclonal, and genetic changes may play a crucial role in the development of this neoplasm. Omental-mesenteric inflammatory pseudotumor appears to represent a distinct clinicopathologic entity as a benign neoplasm in children.

Child, Preschool

Repair of pectus excavatum using a Dacron vascular graft strut.

Long-term follow-up was obtained for 161 patients who underwent a modified Ravitch repair of pectus excavatum. In all cases, the substernal strut was fashioned from a Dacron vascular graft. Follow-up was done by telephone (most cases) and also by office visit (43 cases). The follow-up period was 12 months to 21.5 years. One hundred thirty-three patients (83%) had satisfactory chest contour; 17 had a fair result, and 11 had frank recurrence. Risk factors for poor contour were an asymmetrical defect, a severe defect, and associated congenital anomalies. Of the three patients with Marfan's syndrome, all had recurrence. One hundred thirty (81%) had a satisfactory scar, and 31 had hypertrophic scars. Risk factors for hypertrophic scar formation were increasing age and weight, and tripod internal fixation used early in the series. Factors not contributing to clinical outcome include the sex of the patient, positive family history, perioperative complications, year of surgery, surgeon, and type of incision. The use of a Dacron strut is relatively easy and complication-free. It can be left permanently in situ. Long-term results have been good, except in patients with an extensive or asymmetrical deformity, and in those with other congenital anomalies, especially Marfan's syndrome.

Adolescent

Thrombotic complications of saphenous central venous lines.

Authors of recent publications advocate central venous access by saphenous vein cutdown in the thigh. Even relatively inert SILASTIC catheters are recognized to convey a risk of large vein thrombosis when maintained for long periods. Thrombosis of the inferior vena cava (IVC) and tributaries place the patient at risk for a spectrum of problems not associated with superior vena cava (SVC) cannulation. The authors describe 46 neonatal intensive care patients with central venous cannulation of more than 2 weeks' duration. Prospective monitoring by ultrasound yielded a thrombosis rate of 16.6% for SVC cannulation and 28.5% for IVC cannulation. Complete occlusion of the IVC was clinically apparent and confirmed radiographically in four patients. In another patient, bilateral renal vein thrombosis developed, which contributed to her death. The authors compare their data for IVC and SVC cannulation and question whether the saphenous vein should be a primary route.

Catheterization, Central Venous

Routine inguinal hernia repair in the pediatric population: is office follow-up necessary?

Routine inguinal hernia repair in the pediatric population has a low complication rate. Very few complications are identified at follow-up, which brings into question the necessity of the traditional postoperative visit. A retrospective review of patients who had undergone a routine inguinal hernia repair in 1991 at our institution was done in order to determine our current follow-up practices and complication rate. To determine the perceived necessity for the follow-up visit, parents were given a short telephone questionnaire. Of 175 eligible patients, questionnaires were completed on 145. Of these 145 patients, 77 were seen in follow-up by the pediatric surgeon only, 43 by the family doctor only, 12 were seen by both, and 13 patients had no physician follow-up. The sole complication was a stitch abscess (complication rate 0.7%). Results of the questionnaire showed that 90% of parents felt the follow-up visit was "helpful," 80% felt it was "necessary," and 35% would have been satisfied with telephone follow-up. The main purpose of the postoperative visit appears to be parental reassurance. Careful preoperative and postoperative instruction and reassurance may be sufficient in a significant number of cases.

Child, Preschool

Follow-up after patch aortoplasty for coarctation of aorta.

BACKGROUND: Patch aortoplasty (PA) for coarctation of aorta (COA) can lead to aneurysm formation at the repair site. X-ray, echocardiogram and computed tomography are unreliable for diagnosis of this complication. OBJECTIVE: To evaluate prospectively patients with PA for COA by magnetic resonance imaging (MRI) to detect presence of aneurysm at the repair site. DESIGN: All patients who underwent PA at the authors' institution were identified. MRI was performed in transverse and long axis oblique views on all patients except those who had, or were going to have, aortic angiography for other reasons. Details of the surgical procedure were obtained from the hospital records. RESULTS: Of the 18 patients studied, 15 had MRI and three had aortic angiography. Age at PA ranged from one week to 13.3 years (mean 6.3 years). The interval from PA to MRI or angiography was 9.5 years (range four to 12.5). No aneurysm was detected in any patient. Recoarctation was diagnosed in two patients not previously suspected but discovered on MRI. At PA the intimal shelf causing coarctation was either not excised or only minimally trimmed in 14 of 15 instances. CONCLUSIONS: The incidence of late aneurysm formation following PA for COA is low in the authors' patients, possibly due to minimal intimal damage at repair, although these patients should be followed longer. MRI was useful for assessment of aneurysm and restenosis.

Adolescent

Blunt traumatic disruption of the thoracic aorta: a rare injury in children.

Rupture of the thoracic aorta secondary to blunt chest trauma is an exceedingly uncommon injury in pediatric patients. We present a case of blunt traumatic aortic disruption in a 10-year-old child who was successfully managed by primary aortic repair using partial cardiopulmonary bypass. The epidemiology and pathophysiology of this injury, with particular reference to children, is reviewed. The ongoing controversies regarding the diagnosis and operative management of this injury are summarized.

Aorta, Thoracic

The use of indium 111 leukocyte scans in children with inflammatory bowel disease.

Indium 111 leukocyte (WBC) scans have been used in the investigation of inflammatory bowel disease in recent years, but their use in children is not well described. We reviewed our experience with 281 indium 111 scans over an 8-year period in a total of 109 children with inflammatory bowel disease (Crohn's disease 70, ulcerative colitis 39). One hundred twenty-eight indium 111 scans done within 2 weeks of a radiographic contrast study, endoscopic examination, or surgical procedure were evaluated for diagnostic accuracy. Analysis showed that indium 111 scans were accurate when compared with conventional diagnostic tests and operative findings. This noninvasive imaging technique can play a useful role in the management of children with inflammatory bowel disease.

Adolescent

Unbalanced translocation of chromosome 3p in Wilms' tumor.

Genetic studies in Wilms' tumor have most commonly shown a deletion involving band 13 on the p arm of chromosome 11 in association with aniridia. Structural rearrangements of chromosome 3p have been found in carcinoma of renal cell and lung origin but have not been previously reported in Wilms' tumors. We present two phenotypically normal, unrelated patients with Wilms' tumors, one of which was bilateral, in which cytogenetic analysis of the tumors showed an unbalanced translocation of the p arm of chromosome 3. Two biopsies were done in the patient with bilateral Wilms' tumor. The first biopsy specimen showed a translocation between chromosome 3 and 13 with partial trisomy of 3p and loss of material from 13q. The second biopsy three and a half months later again showed trisomy of chromosome 3p. The unilateral Wilms' tumor showed trisomy of 3p with partial loss of 7p. Neither patient showed a constitutional chromosomal abnormality and neither tumor showed any cytogenetic abnormality involving chromosome 11p. Quantitative DNA analysis was performed in the tumors of both patients. The bilateral Wilms' tumor was nearly diploid with a DNA index of 1.284 (mean ploidy, 2.45; SD, 0.854) while the unilateral Wilms' tumor was aneuploid with a DNA index of 1.531 (mean ploidy, 3.35; SD, 0.976). DNA analysis results are discussed in relationship to the chromosome abnormality seen on the karyotype analysis. These cytogenetic findings suggest that genetic oncogenesis in Wilms' tumor is heterogenous.

Aniridia

Topical sucralfate: effective therapy for the management of resistant peristomal and perineal excoriation.

Peristomal and perineal excoriation commonly occurs despite preventive measures. Drainage from around gastrostomy tubes or ongoing perineal soilage after a pull-through procedure can lead to chemical irritation, cutaneous denudation, and chronic discomfort. A multitude of topical agents have been tried with variable results. We present our experience using topical sucralfate. Fifteen patients with stomal or perineal skin ulceration were treated with topical sucralfate only after other agents had failed. Clinical photographs were obtained first. Sucralfate, prepared as either a powder or an emollient, was liberally applied to the affected area during diaper changes or when the stomal appliance was emptied. For tube gastrostomy sites, sucralfate was applied every 4 to 6 hours as required to maintain a visible layer. In 13 patients, complete healing occurred. Recovery time was dependent on the severity and extent of skin denudation. Partial healing occurred in one patient. In another patient, the skin excoriation healed but a residual candidal rash required addition of an antifungal agent. General observations included: (1) a lag time of 2 to 3 days before visible healing was evident; (2) healing occurred from the perimeter; (3) sucralfate was soothing and reduced discomfort; (4) it was ineffective for fungal dermatitis; and (5) sucralfate did not appear to have toxic or systemic effects. Topically applied sucralfate is soothing, safe, and effective.

Abdomen

The absence of clinically significant tracheomalacia in patients having esophageal atresia without tracheoesophageal fistula.

The association of tracheomalacia (TM) with esophageal atresia (EA) has been well described. This study attempted to find a correlation between the severity of TM and the presence or absence of an associated tracheoesophageal fistula (TEF) in patients with EA. A review of all patients presenting at this institution with EA through the years 1970 to 1990 was carried out. Six patients with EA without fistula (type A) and 61 patients having EA with fistula (types B, C, D, and E) were analyzed. Five of the six type A patients required esophageal dilatations and antireflux procedures; none had clinically significant TM. Thirty-five of the 61 patients with fistula required esophageal dilatation, and 8 had an antireflux procedure; 11 (18%) required either long-tube tracheostomy or aortopexy for TM. This small study supports a recent hypothesis of different embryological events resulting in different types of esophageal and tracheal anomalies. When EA occurs without fistula, it appears that no significant alteration in tracheal development occurs.

Abnormalities, Multiple

Emergency separation of conjoined twins.

Female omphalopagus conjoined twins were successfully delivered vaginally and required emergency surgical separation shortly after birth for gastroschisis. Shared tissue included conjoined bowel; one twin also had a complex cloacal abnormality and patent urachus. A 2-year follow-up is presented. A review of the relevant literature confirms that this is the first example of gastroschisis conjoining omphalopagus twins.

Abdominal Muscles

Congenital pyloric atresia and junctional epidermolysis bullosa: a report of long-term survival and a review of the literature.

The coexistence of congenital pyloric atresia (PA) and epidermolysis bullosa (EB) in newborns is a rare but distinct association. Mortality is high. In particular, a universally fatal outcome has been reported in neonates born with the junctional type of EB and PA. This has led some investigators to advocate that surgical correction of PA be withheld to obviate needless suffering. We treated five patients, including one set of siblings. Maternal hydramnios and nonbilious vomiting were constant features. Delayed passage of meconium was found in four. Plain x-rays demonstrated gastric dilatation in an otherwise gasless abdomen. Blistering skin lesions were noted at birth in four and developed soon after in the last patient. All lesions were determined to be junctional EB based on electronmicroscopy. The clinical course for these children has been far better than the literature predicts. Successful repair of PA was performed after appropriate stabilization. One infant died at 4 months of age of staphyloccal septicemia, malnutrition, and sepsis from chronic urinary tract obstruction. Another child, born with dysmorphic features to consanguineous parents, is 9 years old and has a seizure disorder. The remaining three are alive and well at 17 months, and 9 and 16 years. The oldest two are siblings. In all four surviving patients, the blistering nonscarring lesions were found to significantly improve in severity, duration, and occurrence with age. Presently, these lesions are mild and require little therapy. Their nails, initially normal at birth, have become discoloured, thickened, and dystrophic. The management of pitted, carious, and yellow teeth is currently the major problem.(ABSTRACT TRUNCATED AT 250 WORDS)

Epidermolysis Bullosa, Junctional

Atrioventricular canal defects: results of repair in the current era.

Between December 1986 and December 1990, 37 consecutive patients underwent repair of complete atrioventricular (AV) canal with the two-patch technique. Mean age at repair was 22 months and 51% were less than 1 year of age. Eighteen (48.6%) had undergone previous palliative operations. Two operative deaths (5.4%) occurred and another patient died in-hospital for an early mortality of 8.1%. One late death (2.9%) has occurred from a respiratory infection. Actuarial survival is 87.7% at 3 years. Small size (p less than 0.05), unbalanced ventricular size (p less than 0.05), New York Heart Association (NYHA) Class IV (p less than 0.05), and severe preoperative AV valve insufficiency (p less than 0.05) were significant preoperative risk factors for death. Five survivors (14.7%) required reoperation for severe AV valve insufficiency (two) or patch leaks (three). The risk for reoperation was increased in non-Down's patients (p less than 0.02). All survivors are in NYHA Class I (93%) or II (7%). The risk for early AV valve insufficiency was increased in patients who did not have the cleft sutured (p less than 0.05), and in those with unbalanced ventricles (p less than 0.01). Risk of late AV valve insufficiency was increased only by small size (p less than 0.02). Previous pulmonary artery banding did not increase the risk of repair or of postoperative AV valve insufficiency. Complete AV canal can be repaired with low mortality. Pulmonary artery banding may still have a role to play in the very small (4-5 kg) infant in refractory heart failure.

Aortic Valve Insufficiency

Idiopathic dilatation of the aorta with dissection in a family without Marfan syndrome.

Dissection of the aorta is very rare in children, but classically occurs in the presence of Marfan syndrome or other connective tissue disorder. We present a case of spontaneous dissection in a 12-year-old boy whose half brother has an idiopathic dilated aorta and whose mother has also required surgery for dissection of a dilated aorta. No features of connective tissue disorder were present in any family member.

Adult

Balloon catheter dilatation for hypertrophic pyloric stenosis.

Balloon dilating catheters (BDC) have provided a non-operative means of managing obstructive lesions within the gastrointestinal tract. Its potential utility in infants with hypertrophic pyloric stenosis (HPS) was studied. Six patients with HPS underwent balloon catheter dilatation of the pylorus under the direct observation of the surgeon. The pylorus was exposed using a standard right upper quadrant incision. The BDC was passed transorally into the stomach and manipulated into the pyloric canal by the surgeon. The balloon was inflated with saline to a maximum pressure of 50 psi for 2 minutes. Four patients were dilated with a 10-mm diameter balloon catheter, and in two patients, a 15-mm balloon was used. Success was defined as the complete and longitudinal disruption of the seromuscular ring without violation of mucosal integrity. Using this criterion, none had successful pyloric dilatation. No disruption occurred in three patients, partial disruption in two. These patients subsequently underwent a Ramstedt pyloromyotomy. Complete disruption was observed in one; however, a breach of the mucosa was evident. This was repaired without incident. All seromuscular breaks occurred at the point of vascular entry along the lesser curve, presumably the weakest point of the ring. Pyloric dilatation using BDC does not reliably disrupt the muscular ring. This preliminary report recognizes that major refinements must occur before this method will supplant the time-honored surgical pyloromyotomy for HPS.

Catheterization