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Biomedical subjects

J L Grosfeld

Publications and source records attributed to J L Grosfeld.

At least 145 records · Page 8Linked to original sources

Pancreatic disorders in infancy and childhood: experience with 92 cases.

Ninety-two children with pancreatic disorders were treated over a 10-year period. Thirty-three had blunt trauma, while 69 had medical, metabolic, or neoplastic diseases. Children with trauma had either duct disruption (3), gland fracture (4), or pseudocysts formation (26). Operation was required in 30. Pseudocysts were treated with observation alone in three cases, ultrasound-guided percutaneous aspiration in three, surgical external drainage in two, distal pancreatectomy in four, cyst gastrostomy in ten, and cyst-Roux-en-Y jejunostomy in six. Other disorders included pancreatitis (44), neoplasms (10), nesidioblastosis (4), and pancreaticosplenic abscess (2). Treatment for neoplasms included surgical excision in nine and biopsy in one (adenocarcinoma). Patients with nesidioblastosis underwent 95% (near total) pancreatic resection (two after previous unsuccessful 80% resection). Pancreatitis was familial in two cases, necrotizing in two, idiopathic in 11, and secondary to medications in six cases (steroids, 2; L-asparaginase, 4), gallstones in 17, and choledochal cysts in 6. Pancreatitis resolved after observation and conservative therapy in ten idiopathic cases, 4/6 medication-related cases, and following correction of biliary tract disease (15/17) or choledochal cysts (6). Pancreatic resection or drainage was required in the remaining cases. Pancreatic disorders can be accurately detected with computed tomography (CT) scan in most cases (excluding insulinoma). Ultrasound (US) is useful in cases of biliary tract disease and pseudocyst formation. Traumatic pseudocysts can resolve spontaneously or with US-guided percutaneous drainage (in the presence of normal ducts). Children with neoplasms, abnormal pancreatic ducts, or recurrent pancreatitis require resection or appropriate drainage procedures. Overall survival was 95%.

Child, Preschool↗

Pneumatosis intestinalis in children beyond the neonatal period.

Pneumatosis intestinalis (PI) is a well-recognized manifestation of necrotizing enterocolitis (NEC) in the newborn--a condition that often requires surgical intervention for infarcted bowel. However, little information is available concerning PI in older children or its management. Sixteen older infants and children (greater than 2 months) had x-ray findings of PI (intramural air). There were eight girls and eight boys ranging in age from 2 months to 8 years. Associated conditions included short bowel syndrome (SBS) (8), congenital heart disease (2), iron ingestion (1), nesidioblastosis (1), hemolytic anemia (1), rheumatoid arthritis (1), bronchopulmonary dysplasia (BPD) (1), and malrotation (1). Clinical presentation included abdominal distension (13), bloody diarrhea (12), bilious emesis (5), and lethargy (5). Two patients on steroids had unsuspected PI identified as an incidental operative finding during pancreatectomy for nesidioblastosis (1) and splenectomy for hemolytic anemia (1), respectively. Only four other children (iron toxicity, postcardiac catheterization, rheumatoid arthritis, and BPD required surgical intervention. Each manifested peritioneal irritation, acidosis, and hypotension or had pneumoperitoneum on abdominal x-ray. In ten of 14 patients, PI was managed nonoperatively with nasogastric suction, fluid resuscitation, intravenous (IV) antibiotics (seven to ten days), and repeated abdominal x-ray and physical examinations. Children with SBS comprised 50% of the total number of patients and eight of ten treated by observation. All had associated viral syndromes (rotavirus) or rhotozyme-positive stools and developed bloody diarrhea. There were two deaths (12.5%) in patients with iron toxicity and congenital heart disease who required resection of gangrenous bowel. All of the other patients survived.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗

Splenic and liver trauma in children.

These data indicate that most children with blunt injury to the liver and spleen can be treated conservatively. The fact that nonoperative management is a successful method of therapy limits the usefulness of DPL in most stable pediatric patients with abdominal trauma. Recovery of blood by DPL will neither influence the decision to operate nor yield information concerning which organ is injured. The abdominal CT scan is the most sensitive diagnostic method. In patients with associated head trauma, the head CT and abdominal CT both can be obtained during the same visit to the radiology suite. DPL may be useful in the unstable patient to be sure the abdomen is the site of bleeding before starting an emergency laparotomy and occasionally, in more stable patients with ongoing abdominal pain, to rule out an associated bowel injury with perforation (e.g., recovery of bilious or feculant material). Insight into the contemporary management of splenic and liver injuries in children is important. In the 1980s, a laparotomy safely can be avoided in many cases with reduction of patient morbidity, length of hospital stay and cost of hospitalization.

Abdominal Injuries↗

The efficacy of hepatoportoenterostomy in biliary atresia.

This report describes the treatment and outcome of 66 infants with biliary atresia. Mean age was 79.8 +/- 33.2 days. Diagnosis was achieved by 99mTc DISIDA scanning. Hepatoportoenterostomy (HPE) was performed in 48 cases and hepatoportocholecystostomy in four, with microscopic ducts at the porta hepatis. Fourteen infants without microscopic ducts did not undergo HPE. Patients were staged according to the postoperative result. HPE was successful in 25% of patients (group A), resulted in improvement in 19% (group B), failed in 43% (group C), and was short-term in 13% (group D). In patients less than 90 days of age, the HPE success rate was 31%; 23% improved, and 33% showed no improvement. Age (less than 90 days) and bile clearance were prognostic determinants of success. Reoperation was useful only in patients with a previously successful HPE. Ten of 20 patients referred for liver transplantation survived (50%) (7/11) survived after liver transplantation and 3/9 on the waiting list). Fourteen of 15 patients in group A remain anicteric and well without liver transplantation. Patients in group B have had extended survival (greater than 3 years) but eventually required transplantation. Patients in group C and children more than 90 days old at diagnosis require early liver transplantation. HPE is a useful procedure when performed in infants less than 90 days of age who have biliary atresia.

Anastomosis, Roux-en-Y↗

Obstructive jaundice. An unusual delayed presentation of congenital diaphragmatic hernia.

Most cases of congenital diaphragmatic hernia present as acute respiratory emergencies in the newborn period. Delayed presentation may be heralded by symptoms referred to the respiratory or gastrointestinal tract. An infant with unrecognized right-sided foramen of Bochdalek hernia presented with obstructive jaundice due to compression of the herniated common bile duct by the rim of the diaphragmatic defect. To our knowledge, this occurrence has not been previously observed in infancy or childhood.

Cholestasis↗

Bronchoscopy for aspirated foreign bodies in children. Experience in 131 cases.

One hundred thirty-one children underwent rigid bronchoscopy under general anesthesia for suspected aspirated foreign bodies. There were 79 boys and 52 girls, with a mean age of 2.1 years. Physical examination showed decreased breath sounds (n = 130) and wheezing (n = 119) over the affected site. Chest roentgenograms were diagnostic or suggestive of aspirated foreign bodies in 127 cases (97%). Radiopaque lesions were noted on roentgenograms in ten cases. Four infants had a preoperative hypoxic arrest. Two patients had negative results of bronchoscopy (1.5%). Extraction of the aspirated foreign body was carried out at laryngoscopy in two patients and by forceps under direct vision at bronchoscopy in 97 patients. A Fogarty catheter was used in 12 cases. Postendoscopic complications included fever (n = 27), pulmonary infiltrate (n = 11), ventilatory support (n = 4), and pneumothorax (n = 2). Rigid bronchoscopy for aspirated foreign body in children is a safe, effective, and sometimes life-saving procedure. Morbidity is low and mortality is zero.

Bronchi↗

The effect of increased intracranial pressure (ICP) on gastric motility.

This study evaluates the effect of increased intracranial pressure (ICP) on gastric motility. Nine male cats (weight, 4.84 +/- 1.16 kg) were anesthetized with ketamine and underwent laparotomy for placement of bipolar (silver-silver chloride) electrodes on the serosal surface of the gastroesophageal junction (GEJ), antrum, and prepyloric areas of the stomach. At 1 week frontoparietal burr holes were performed with placement of an epidural Fogarty catheter. Migrating myoelectric complexes (MMCs) were evaluated at the GEJ, antrum, and prepyloric areas at varying levels of ICP (baseline and 20, 40, and 60 mm Hg) using balloon inflation. MMCs at the GEJ were triphasic with a period of 4 sec (+/- 1 sec) at baseline levels. At ICP levels above baseline, periodicity and waveforms at the GEJ became irregular. Waveforms became multiphasic with 1- to 2-sec periods and variable amplitudes. In the antral and prepyloric areas, duration and amplitude of the triphasic MMCs was unchanged from baseline. At 60 mm Hg ICP periodicity was significantly altered at both 1 and 2 weeks. MMCs returned to baseline levels with balloon deflation. The data indicate that elevated ICP (to 60 mm Hg) results in consistent and reproducible alterations of MMC periodicity, suggesting that such alterations may influence gastric motility.

Animals↗

Impaired immune function in obstructive jaundice.

Sepsis is a common and occasionally lethal complication of obstructive jaundice. The reasons for this increased susceptibility to infection are unknown. This study examines lymphocyte and reticuloendothelial (RES) function in animals with obstructive jaundice. Twelve New Zealand white rabbits (3-4 kg) were studied. Lymphocyte function was evaluated in six rabbits by phytohemagglutinin (PHA), concanavalin A (Con A), and pokeweed mitogen (PWM) stimulation. In six animals, hepatic RES function was assessed by calculating the phagocytic index (PI) using the disappearance of 99Tc sulfacolloid (5 mg/kg) iv. After baseline studies, the common bile duct was divided and ligated. The above studies and serum bilirubin were repeated at 3 weeks. Obstruction was then relieved by cholecystojejunostomy (CJ) and RES studies repeated monthly x 6. Preobstructive lymphocyte function showed a stimulation index ratio (log) of 0.85 +/- 0.25 for PHA, 0.75 +/- 0.3 for Con A, and 0.71 +/- 0.25 for PWM. With biliary obstruction, the values fell to -0.23 +/- 15 (P less than 0.006), -0.31 +/- 0.12 (P less than 0.006), and -0.29 (P less than 0.006), demonstrating impaired lymphocyte function. When tested lymphocytes were mixed with control pooled rabbit serum, however, no lymphocyte impairment was noted. Baseline hepatic PI was 6.02 +/- 0.18 and fell to 3.79 +/- 0.33 with obstruction (P less than .01) and remained low at (3.20 +/- 0.14) 1 month (P less than 0.01) and (3.33 +/- 0.23) at 3 months (P less than .01), after CJ but returned to normal (8.04 +/- 0.97) at 6 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Late results following esophagomyotomy in children with achalasia.

Twenty one children with achalasia of the esophagus were treated from 1970 to 1986. There were 11 girls and ten boys (average age, 10.9 years; range, 6 months to 16 years). Diagnosis was established by barium swallow in 21 cases and confirmed by manometrics and motility studies in 14. Four children had unsuccessful dilatation (range, 1 to 16 dilatations/pt). All 21 children underwent modified anterior Heller esophagomyotomy (transabdominal in 15 and transthoracic in six). Concomitant Nissen fundoplication was performed in three. Follow-up from 1 to 14 years (mean, 6.3 years) showed complete relief of obstruction in 18 patients (86%), while three required additional procedures for persistent dysphagia. One child improved after a single dilatation, but two others eventually required a second esophagomyotomy. Three additional patients subsequently developed gastroesophageal reflux (GER), and two were managed with Nissen fundoplication; the third responded to medical management. The mortality for this series was zero. Postoperative complications occurred in nine children (42%) and was due to atelectasis and postoperative fever. Modified Heller esophagomyotomy is safe and effective in children with achalasia (mortality, 0%; relief of obstruction, 86%). Results were similar after a transabdominal or transthoracic approach. Esophageal dilatation was not an effective method of treatment. Although postsurgical barium swallow showed relief of obstruction, abnormal esophageal motility persisted, suggesting that long-term follow-up is important.

Adolescent↗

Vasoactive intestinal polypeptide-secreting ganglioneuromatosis affecting the entire colon and rectum.

This report describes a 7-year-old boy presenting with watery diarrhea, hypokalemia, and hypochlohydria associated with vasoactive intestinal polypeptide (VIP)-secreting ganglioneuromatosis involving the entire colon and rectum. The child's symptoms resolved following proctocolectomy, and the VIP levels returned to normal. Although 55 previous children have been reported with VIP-secreting tumors, this case is the first involving the entire colon and rectum.

Adenoma, Islet Cell↗

Chest wall resection and reconstruction for malignant conditions in childhood.

This report evaluates the efficacy of extensive chest wall resection and prosthetic reconstruction in 15 children with chest wall malignancies. There were nine boys and six girls, with a mean age of 9.6 years. Eleven patients had primary chest wall tumors including Ewing's sarcoma (ES), six; rhabdomyosarcoma (RH), two; chondrosarcoma (CS), one; Askin's malignant neuroectodermal tumor, one; and mesenchymal sarcoma, one. Four children had metastases to chest wall and lung from Wilms' tumor (WT), two; osteogenic sarcoma (OS), one; and neuroblastoma (NB), one. Chest wall resection of two to six ribs and reconstruction with Marlex mesh (seven), lattisimus flap (two), prolene mesh (one), and more recently, a Gortex patch (five), was performed. Eight of the patients required concomitant en-bloc pulmonary resection (wedge, five; lobectomy, two; pneumonectomy, one) and two required resection of diaphragm. Fourteen received adjunctive therapy (chemotherapy, 14; irradiation, eight [preoperative, five; postoperative, three]. Six patients had second-look resections after chemotherapy. There was no operative mortality. Early pulmonary function was normal; however, pulmonary restrictive disease and scoliosis occurred with growth. One ES patient developed a radiation-induced second malignant tumor at age 10 and one ES child died at age 6 (no evidence of disease) of meningitis. Average survival length for ES patients was 77 months (range, 18 to 132 months.) Currently, eight patients are alive and five are free of disease. Extensive chest wall resection and reconstruction is useful in the treatment of primary chest wall tumors, but is palliative in metastatic cases. The Gortex patch is the current prosthetic of choice.

Adolescent↗

Postoperative intussusception: experience with 36 cases in children.

Intestinal obstruction is a common postoperative complication and is usually related to peritoneal adhesion formation. A less well-recognized cause is postoperative intussusception (POI). Thirty-six instances of POI in children (aged 1 month to 18 years) were treated between 1970 and 1987. POI followed Nissen fundoplication in 9 patients, neuroblastoma resection in 5, small-bowel procedures in 4, inguinal herniorrhaphy in 3, pull-through procedures in 3, ureterostomy in 2, thoracic procedures in 2, ventral hernia in 1, nephrectomy in 1, hepatic resection in 1, Heller myotomy in 1, ventriculo-atrial shunt in 1, and gastrocystoplasty in 1. Initial symptoms included bilious vomiting or increased nasogastric drainage (after initial return of gut function) in 26 patients, abdominal distension in 24, irritability in 10, intermittent pain in 7, palpable abdominal mass in 2, rectal bleeding in 2, and lethargy in 1. The symptoms occurred 1 to 24 days (mean, 8 days) after the initial surgery. Plain abdominal radiographs revealed multiple air-fluid levels in 31 and an "adynamic ileus" in five patients. Barium contrast techniques could successfully reduce two ileocolic and one distal ileo-ileal lesions. The remainder necessitated operative management. Manual reduction was possible in 29 cases, and four children with diagnostic delay required bowel resection and an anastomosis for intestinal necrosis. The site of intussusception was ileo-ileal in 23 patients, jejunojejunal in 6, ileocolic in 5, and jejuno-ileal in 2. The diagnosis of POI should be considered in children with signs of bowel dysfunction in the early postoperative period. Contrast studies are of limited value, since most cases are confined to the small bowel. A high index of suspicion and prompt laparotomy will usually allow manual reduction of the lesion. Diagnostic delay may result in bowel necrosis.

Adolescent↗

Vitelline duct anomalies. Experience with 217 childhood cases.

Of 217 children with vitelline duct anomalies, 85 (40%) had symptomatic lesions (mean age, 2.4 years). Forty-eight patients presented with rectal bleeding; 28, with intestinal obstruction; five, with abdominal pain; and four, with bilious umbilical drainage. An asymptomatic Meckel's diverticulum was discovered incidentally at laparotomy in 132 children. Surgical therapy included bowel resection in nine patients with volvulus, four with intussusception, seven with bleeding, three with vitelline cysts, and one with a perforation. Diverticulectomy was performed in 189 cases, and excision of a patent vitelline duct was accomplished in four neonates with umbilical drainage. Ectopic gastric mucosa was present in all 48 patients with bleeding and in four of five with inflammation but in only two asymptomatic specimens. More than one third of the cases were symptomatic and presented in younger patients. This suggests that elective resection of asymptomatic vitelline remnants in early childhood is reasonable at the time of laparotomy for other conditions.

Appendicitis↗

Complications and reoperation after Nissen fundoplication in childhood.

Over a 10 year period, 429 Nissen fundoplications were performed on children with gastroesophageal reflux. Postoperative complications occurred in 69 children (16 percent), including wrap herniation or breakdown in 29; postoperative bowel obstruction in 18; stricture in 10; intraabdominal abscess and enterocutaneous fistula in 3 patients each; and wound infection, wound dehiscence, and inadvertent splenectomy in 2 patients each. The postoperative mortality rate was 0.9 percent (4 of 429 patients) and was related to sepsis in 1 patient, a metabolic disorder in 1 patient, and underlying pulmonary disease in 2 patients. All four patients were neurologically impaired. Fundoplication successfully controlled symptoms of gastroesophageal reflux in 395 children (92 percent) over a follow-up period ranging from 6 months to 10 years. Thirty-eight patients (8.8 percent) required a second antireflux operation because of recurrent symptoms. Twenty-nine patients had severe neurologic impairment (76 percent), 5 had associated congenital malformations (13 percent), and 3 had significant pulmonary problems (8 percent). Only one child requiring reoperation was considered otherwise normal. Indications for reoperation included wrap breakdown or herniation (28 patients), stricture (6 patients), and inadequate wrap (4 patients). Twenty-four of 28 children with wrap herniation or breakdown had neurologic impairment. A second fundoplication was successful in 35 of 38 patients (92 percent). A second procedure failed in three children, who required subsequent resection and colon interposition.

Adolescent↗

Electrical pacing of the Roux limb resolves delayed gastric emptying.

This study compares antegrade gastric and Roux-limb electrical pacing in the evaluation and treatment of delayed gastric emptying following vagotomy, antrectomy and Roux-Y enterostomy. Twenty-four male Sprague-Dawley rats (250 g) underwent midline laparotomy, truncal vagotomy, antrectomy, and Roux-Y jejunostomy. Pacemaker leads were implanted 1 cm apart in both the gastric fundus and proximal Roux limb. Pacing was accomplished using a 0.5 mA, 50 msec, 0.33 Hz signal and monitored by an oscilloscope. Animals were fasted for 2 hr and then gavaged with 1.0 cc of 99mTc-labeled egg white. At 1 hr rats were anesthetized. The stomach, Roux limb, small intestine, and colon were doubly ligated and excised without disturbing their contents. The total number of counts per minute per rat was determined in a gamma radiation counter, and percentage gastric emptying (GE) was evaluated. Group I controls (n = 8) retained 76 +/- 15.8% for a GE of 24%. Group II gastric paced rats (n = 8) retained 64.5 +/- 19.2% (GE 35.5%) and Group III Roux-limb paced rats (n = 8) retained 46.8% +/- 13.2 (GE 53.2%) (P less than 0.005 III vs I, P less than 0.05 III vs II). The amount of radioactive meal distal to the Roux limb was also evaluated. Group I had 15.7 +/- 16.1%, Group II (gastric paced) 20.5 +/- 19.0%, and Group III (Roux-limb paced) 37.2 +/- 11.9% (P less than 0.005 III vs I, P less than 0.05 III vs II). These data imply that Roux-en-Y limb dysmotility may contribute to delayed gastric emptying following vagotomy, antrectomy, and Roux-Y enterostomy.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Changes in lower esophageal sphincter pressure (LES) after Stamm gastrostomy.

Previous clinical and experimental reports have implicated placement of a Stamm gastrostomy (SG) as a cause of gastroesophageal reflux (GER) in children. This study evaluates this problem by measuring alterations in the lower esophageal sphincter pressure (LES) after SG with and without maintenance of the esophagogastric angle of Hiss. In 20 cats (2.8-3.2 kg) general anesthesia was induced using 20 mg/kg ketamine im. Esophageal manometrics were measured using a continuous perfusion catheter and recording system, evaluating three measurements for each animal. Eight cats (Group I) underwent SG placement in the anterior stomach wall two-thirds of the way down from the fundus. This was tacked to the anterior abdominal wall 3 cm lateral to the midline at the appropriate level. Six cats (Group II) had standard SG tube placement and in addition, two interrupted sutures were placed between the fundus and the esophagus maintaining the gastroesophageal angle of Hiss. Six cats (Group III) had sham laparotomy. After awakening, the animals were fed cat chow and water ad libitum. At 7 and 14 days, the animals were reanesthetized with ketamine and manometrics were repeated. Preoperative LES pressure measured 11.3 +/- 4.7 Torr. LES pressure in Group I decreased to 6.61 +/- 1.6 Torr at 7 days (P less than 0.01) and 4.8 +/- 1.6 Torr at 14 days postoperatively (P less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Reduction of lower esophageal sphincter pressure with Stamm gastrostomy.

Although recent clinical studies suggest an association between Stamm gastrostomy and gastroesophageal reflux (GER), no significant experimental data is available. This study evaluates alterations in lower esophageal sphincter (LES) pressure after Stamm gastrostomy in cats. Eight male cats (average weight 3 to 4 kg) were induced under general anesthesia using 20/mg/kg ketamine intramuscularly (IM). Esophageal manometrics were determined for each animal using a continuous perfusion catheter and recording system. Three measurements were taken for each animal. Stamm gastrostomy was then placed in the anterior wall of the stomach two thirds of the way down from the fundus. This was tacked to the anterior abdominal wall 3.0 cm lateral to the midline at the appropriate longitudinal level. Esophageal manometry was repeated after abdominal closure. The animals were awakened and returned to their cages for ad libidum feedings. The animals were reanesthetized with ketamine and manometrics repeated at 7 and 14 days. Each animal served as its own control. Five animals underwent barium esophagram 14 days postoperatively to evaluate for GER. Preoperative mean LES pressure measured 11.4 +/- 3.5 torr. This decreased to 7.8 +/- 2.8 torr immediately after Stamm gastrostomy (P less than .025). When evaluated at 1 and 2 weeks after gastrostomy, further decrease in LES pressures to 6.6 +/- 1.6 torr and 4.8 +/- 1.6 was observed (P less than .02 v preoperative). Three of five cats undergoing barium swallow demonstrated significant reflux radiographically. Stamm gastrostomy caused significant reduction of the LES pressure in all cats studied. This resulted in clinical GER as documented by barium swallow in three of five cats.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗