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Biomedical subjects

D A Gillis

Publications and source records attributed to D A Gillis.

At least 37 records · Page 2Linked to original sources

Management of foreign-body bronchial obstruction using endoscopic laser therapy.

A case is presented in which the Nd:YAG laser obviated the need for a major thoracic procedure. A 7-year-old boy presented with an 18-month history of persistent nonproductive cough, preceded by a choking spell while eating pistachio nuts. High-grade left mainstem bronchial obstruction was caused by friable granulation tissue secondary to an underlying foreign body. By advancing a laser fiber through the working channel of a 5-mm Storz ventilating bronchoscope, the granulation tissue was vaporized, showing an irregular piece of pistachio nut shell embedded within the bronchial wall. After retrieval of the foreign body, bronchial patency was restored. Recovery was complete and uneventful. The bronchoscopic laser permits the delivery of precise and effective treatment to lesions within confined areas, and has a useful role in the management of some central airway lesions in children.

Airway Obstruction↗

A 40-year multinational retrospective study of 880 Swenson procedures.

This report reviews the experience of pediatric surgeons in seven cities in North American and Western Europe where the Swenson procedure was performed on 880 patients. Information on the diagnosis, treatment, complications, and long-term results was collected by reviewing the hospital records, the treating physicians' office records, and by interviewing the patients in person or by telephone. A follow-up evaluation was obtained on 814 patients. The patients' ages at the time of the resection ranged from four days to 50 years. The length of follow-up averaged 10.3 years, while the longest follow-up was 39.5 years. The overall postoperative mortality was 2.4% during the entire 40 years of the study. The postoperative mortality has decreased to 1.25% for the last 20 years. Significant factors influencing postoperative mortality included Down's syndrome, the patient's age at the time of the operation, and leak of the distal colonic anastomosis. Most of the patients followed for over 5 years have normal bowel habits, report one to three bowel movements per day, and have no soiling. No patient has urinary incontinence or impotence.

Adolescent↗

Secondary massive ovarian edema with Meig's syndrome.

Primary massive ovarian edema is uncommon, particularly when it involves both ovaries before puberty. Ovarian edema secondary to a preexisting ovarian lesion is one of the postulated mechanisms. A prepubertal girl presented with bilateral massive ovarian edema with stromal hyperthecosis, which may have predisposed both ovaries to undergo partial torsion. The associations with Meig's syndrome, greatly elevated hormone levels and presence of omental and retroperitoneal nodules, raised a suspicion of malignancy.

Child↗

Sinus arrhythmia in children with atrial septal defect: an analysis of heart rate variability before and after surgical repair.

The expression of sinus arrhythmia depends on separation of the systemic and pulmonary venous return to the heart as well as on normal autonomic control mechanisms. Patients with atrial septal defect provide a naturally occurring experiment of communication between the two venous systems. In adults with atrial septal defect sinus arrhythmia is minimal or absent. But children with atrial septal defect retain appreciable sinus arrhythmia, although this is not recognised in published reports. To understand why this is so, continuous electrocardiograms were recorded before and after operation in 10 children (aged 4-16 years, mean 6.3) with atrial septal defects and in 10 normal children (aged 5-7 years, mean 6.1). Mean RR intervals were calculated for periods of one minute, and the standard deviation was used as an index of heart rate variability (that is sinus arrhythmia). Frequency analysis (spectral analysis) was also performed on a continuous beat to beat record of heart rate to describe the frequency components that may reflect autonomic activity. The results confirmed the presence of considerable sinus arrhythmia in children with unoperated atrial septal defect. None the less, the standard deviation of RR intervals in the children with unoperated atrial septal defect was significantly less than that for the normal children, and variation increased after closure of the defect. Power spectral analysis of instantaneous heart rate indicated that the high frequency (0.15-0.45 Hz) vagally mediated component of variability was lower for patients than for controls which may indicate abnormalities of autonomic control of heart rate in these children. The comparative retention of sinus arrhythmia in children with atrial septal defect may relate to the small size of the right atrium or differences in myocardial compliance compared with adults.

Adolescent↗

Malignant mixed germ-cell-sex cord-stromal tumors of the ovary associated with isosexual precocious puberty.

Two cases of hormonally active, metastasizing malignant mixed germ cell-sex cord-stromal tumors are described in otherwise normal prepubertal girls. Isosexual precocity was noted 5 months and 1 month before surgery. One child died 1 year after presentation, and the other was alive, with no apparent tumor, 1 year after diagnosis. These two cases represent the first recorded instance of a unique tumor in which metastases of several cell types were encountered.

Child↗

Neurenteric cysts--a spectrum.

This review encompasses seven patients with clinically important cystic lesions of the gastrointestinal (GI) tract, exhibiting a wide range of vertebral anomalies and connections to the neural canal. Three patients had mediastinal masses connected to lower cervical and upper thoracic anomalous vertebrae with intraspinal extensions. In addition, one of these patients had a separate, juxtapancreatic intestinal duplication cyst. One infant with colonic duplication had a lumbar vertebral anomaly and an epithelial-lined tract between the two. Another patient had a presacral cystic mass which was the site of recurrent infections and meningitis until a connection with the rectum was divided. A newborn baby had a completely split notochord syndrome with a large dorsal enteric fistula. Finally, one patient had a dorsal enteric cyst with a direct intraspinal connection. Four of the seven patients had associated significant congenital anomalies, two of whom died early in the neonatal period. The rest of the patients did well. This broad range of enteric lesions with associated vertebral and intraspinal abnormalities suggests that the clinical spectrum of neurenteric cystic lesions is much wider than is generally appreciated.

Abnormalities, Multiple↗

Paraesophageal hernia after Nissen fundoplication: a real complication in pediatric patients.

Eighty-nine pediatric patients aged 6 weeks to 20 years (mean, 3.8 years) who underwent Nissen fundoplication were reviewed. Follow-up, including upper gastrointestinal (GI) series, was obtained in 55 patients (61.8%). Fifteen patients developed paraesophageal hernia (PEH) (16.8%). PEH was diagnosed between 4 and 36 months following fundoplication (mean, 17 months). Patients were divided into three groups: A, those with significant mental dysfunction (45); B, those with previous tracheoesophageal fistula (12); and C, others (32). Incidence of PEH is 20% for group A, 16.8% group B, and 12.5% group C. Combining groups B and C, 5 of 25 patients (20%) who underwent fundoplication at less than 1 year of age developed PEH, whereas one of 19 older patients (5.3%) developed PEH. One of 25 patients (4%) who had crural repair at fundoplication developed PEH, whereas 14 of 64 patients without crural repair (21.9%) developed PEH. At surgery, PEH occurred at the left posterolateral aspect of esophagus. We conclude that (1) follow-up after fundoplication should continue for 36 months and include upper GI series; (2) patients under one year of age undergoing fundoplication may be at a higher risk for PEH; and (3) technical refinement including crural repair may be required to prevent PEH.

Adolescent↗

Innovative palliative surgical procedure for hypoplastic left-heart syndrome.

A novel surgical procedure was performed on a 2-day-old infant with hypoplastic left-heart syndrome. It consisted of partitioning the pulmonary trunk thereby creating two channels, one leading to the pulmonary arterial system and the other to the aorta, and atrial septectomy to relieve left atrial pressure and facilitate mixing of blood. The technique avoids the use of conduits.

Aorta, Thoracic↗

Stenosis of individual pulmonary veins: radiologic findings.

Congenital stenosis of individual pulmonary veins is uncommon. Of the 49 cases reported, four were seen at the IWK Hospital for Children and are reported here. Plain radiographs show a shift of the heart toward the side of major involvement, Kerley B lines, fluid in the fissures, and interstitial edema of the affected lobes. These findings, while subtle, should nevertheless suggest stenosis. Technetium-99m macro-aggregate lung perfusion scans show absence of or diminished perfusion of the affected lobes. The diagnosis can usually be confirmed with angiography. Congenital stenosis of individual pulmonary veins should be considered in children with repeated pulmonary infections, dyspnea, failure to thrive, hemoptysis, or unexplained pulmonary hypertension.

Adult↗

Secretory dysfunction in parathyroid cells from a neonate with severe primary hyperparathyroidism.

We report a patient with neonatal severe primary hyperparathyroidism whose parathyroid cells were markedly refractory to regulation by calcium in vitro. He showed life-threatening hypercalcemia (4.8-5.2 mM vs. normal of 2.1-2.7 mM). A sibling had been treated previously for an identical disorder. At age 6.5 months, four hyperplastic parathyroid glands were removed, and portions of one were immediately grafted into the forearm. Serum calcium again became elevated post-operatively and then fall to the normal range after excision of grafted parathyroid tissue. Dispersed parathyroid cells from the first operation showed no suppression of PTH secretion by 2 mM calcium; however, there was normal maximal suppressibility at 4 mM calcium with half-maximal suppression at 2.53 mM (the calcium set point). This contrasts with much lower set points previously established for suppressible cells from normal (1.02 +/- 0.10 mM, mean +/- 1 SD), from primary hyperplastic (1.10 +/- 0.14 mM), or from adenomatous (1.26 +/- 0.14 mM) parathyroid glands. The strikingly high set point may not be unique because a small number of glands previously classified as nonsuppressible (by the criterion of failing to suppress below 50% maximum at calcium concentration up to 2-3 mM) might have shown similarly high set points if tested at higher calcium concentrations. We conclude that an unusual abnormality of PTH secretory control accounts, in large part, for both the marked hypercalcemia and for its refractoriness to surgical treatment in this patient.

Calcium↗

Megacystis-microcolon-intestinal hypoperistalsis syndrome: survival of a male infant.

The combination of megacystis with microcolon and severe intestinal hypoperistalsis constitutes a rarely encountered syndrome in neonates. Of 16 previously reported cases, 14 have occurred in females and all but one have been fatal. Various forms of intestinal diversions have been unsuccessful. Aganglionosis has been excluded in every case. A method of management involving prolonged total parenteral nutrition, followed by graduated jejunostomy and gastrostomy feedings, has resulted in the survival of a male infant with this syndrome. No previous male survivors have been reported.

Abnormalities, Multiple↗

Gastric damage from ingested acid in children.

Acid ingestion occurs relatively rarely and produces a spectrum of injury that is markedly different from the more commonly encountered alkaline burns of the oropharynx and esophagus. Gastric damage results from pylorospasm with pooling of the ingested caustic in a dependent location. Symptoms may be delayed for days or weeks. Perforation and/or strictures may require extensive gastric surgery. Early fiberoptic endoscopy is essential.

Acids↗

Klippel-Trenaunay syndrome: a review.

The Klippel-Trenaunay syndrome is a triad of congenital anomalies characterized by a vascular nevus, varicose veins and bony and soft-tissue hypertrophy. Although the syndrome is uncommon, initially the condition appears as a skin blemish or varicose veins. To avoid errors in management, it is important to recognize the syndrome. In this paper the authors outline the presentation, investigation and management of Klippel-Trenaunay syndrome and describe four cases of their own. One form of Klippel-Trenaunay syndrome, in which all the anomalies of the triad are important, in which direct signs of a large arteriovenous shunt are present and for which the surgical approach is difficult, is Parkes-Weber syndrome. Lack of reports in the world literature on long-term follow-up leaves many unanswered questions regarding prognosis and management.

Angiomatosis↗

Should prophylactic antibiotics be given perioperatively in acute appendicitis without perforation?

The authors question the need to give prophylactic antibiotics perioperatively in all cases of acute appendicitis without perforation to reduce the rate of wound infection. A retrospective study of 82 patients showed a wound infection rate of 4.8%; 3 of 53 patients who did not receive antibiotics had a wound infection compared with 1 of 29 patients who received antibiotics. A prospective double-blind clinical trial was performed comparing the results of perioperative administration of cefamandole and placebo. One of 21 patients who received the placebo had a wound infection; none of the 21 patients given cefamandole had infection. Thus, the authors conclude that, although reports in the literature suggest that antibiotics given perioperatively decrease the rate of wound infection in acute appendicitis without perforation, the incidence of such infection is too low to warrant routine administration. They suggest that such antibiotic use be reserved for those suspected of having more serious disease.

Acute Disease↗

Polytetrafluoroethylene shunts in congenital heart disease.

Over a 2-year period the authors inserted 22 systemic to pulmonary artery shunts using a polytetrafluoroethylene (PTFE) graft in 19 children. A modified Blalock-Taussig shunt was established in 18 instances and an aorta to pulmonary artery shunt in 3: one patient had an aorta to right ventricle infundibulum shunt. Three children had obstructed grafts and in two others the grafts became stenosed; on of the latter group had successful removal of thrombus. The remaining grafts functioned well. Three other patients are presented. In two an interrupted aortic arch was repaired using a PTFE graft; one of these patients died at later closure of an aortopulmonary window, the other did well following repair of a ventricular septal defect and aortic valvotomy. The third child had a right ventricle to pulmonary artery shunt established for tetralogy of Fallot with pulmonary atresia; this resulted in some growth of the pulmonary arteries. The modified Blalock-Taussig shunt, using a PTFE graft, can be inserted without opening the pericardium, normal flow through the subclavian artery is not interrupted, the PTFE is easy to handle and the shunt can be inserted and taken down more easily than traditional shunts. For these reasons, the authors believe that this procedure, using PTFE, has an important role to play in the surgical management of children with congenital heart disease.

Aorta, Thoracic↗

Intraventricular repair of double-outlet left ventricle.

A 3 1/2-year-old child with double-outlet left ventricle and subaortic ventricular septal defect underwent successful surgical intervention with an intraventricular repair and a boomerang-shaped piece of Dacron patch. While an intraventricular repair for double-outlet left ventricle has been reported, we discuss here its application to this particular type of double-outlet left ventricle.

Angiography↗

Surgical correction of pulmonary atresia with multiple large systemic-pulmonary collaterals.

A young patient in whom the pulmonary blood flow was supplied completely by five systemic-pulmonary collaterals underwent reconstruction of the pulmonary outflow tract by a new technique. This consisted of the insertion of a valve-bearing conduit between the right ventricle and an isolated segment of the descending aorta, which gave rise to three of the collaterals. Although the patient had a hypoplastic pulmonary artery confluence, other factors mitigated against its use as the sole conduit for right ventricular output.

Aorta, Thoracic↗