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Biomedical subjects

J L Grosfeld

Publications and source records attributed to J L Grosfeld.

At least 55 records · Page 3Linked to original sources
PubMed1997

The "other side" of pediatric hernias: the role of laparoscopy.

The role of contralateral inguinal exploration in neonates, infants, and children presenting with unilateral hernias is controversial. Factors considered by surgeons include the patient's age, sex, and side of the clinically apparent hernia. The purpose of this study was to evaluate the role of diagnostic laparoscopy performed through the clinically apparent hernia sac to identify a contralateral patent processus vaginalis (CPPV) in children and limit contralateral exploration to CPPV-positive patients. One hundred neonates, infants, and children underwent laparoscopic evaluation for a CPPV through the ipsilateral hernia sac. There were 79 boys and 21 girls. Forty-eight of 100 (48%) had a CPPV identified, which was confirmed operatively. Thirty-one of 68 patients (46%) with a right-sided and 18 of 32 (56%) with a left-sided hernias had a CPPV (P = 0.39). Thirty-six of 56 (64%) patients younger than 6 months of age had a CPPV compared to 13 of 44 (30%) older than six months (P = 0.001). Fourteen of 21 (67%) girls had a CPPV compared to 35 of 79 (44%) boys (P = 0.087). Laparoscopy through the hernia sac is a safe and effective means of identifying the presence of a CPPV and avoiding unnecessary contralateral inguinal exploration. Infants (< 6 months) are much more likely to have a CPPV.

Age Factors↗
PubMed1997

Intraepithelial neoplasms of the pancreas.

Intraepithelial neoplasms of the pancreas have been recently described and relatively rare, but these lesions represent a distinct, however pathologically heterogeneous entity with shared clinical features. We analyzed the clinicopathologic and cytogenetic characteristics of eight patients with intraepithelial neoplasms. Based on our hypothesis that tumor ploidy pattern correlates with ploidy, synthetic (S) phase and proliferative fractions of each neoplasm by flow cytometry. Analysis of the nuclear DNA content of pancreatic intraepithelial neoplasms in this study supports our hypothesis. Each neoplasm demonstrated diploid stemline, with a low S-phase fraction. The diploid DNA pattern and the low proliferative activity are consistent with the nonaggressive behavior of the tumors and, in part, explain the favorable prognosis of intraepithelial neoplasms. Intraepithelial neoplasms of the pancreas constitute a rare, but surgically curable, localized disease with a good prognosis following radical resection. The most valuable tool in the diagnosis of these preinvasive lesions is ERCP combined with brush cytology. This study supports the potential value of flow cytometric DNA analysis in determining outcome, as the diploid DNA pattern and low S-phase fractions correlate with the nonaggressive biological behavior.

Adult↗
PubMed1996

Improved survival in children with esophageal perforation.

OBJECTIVE: To analyze the cause, location, signs and symptoms, presence of underlying disease, time interval to diagnosis, treatment, and morbidity and mortality in 24 children (19 boys and 5 girls) with esophageal perforation who were treated from 1975 to 1995. DESIGN: Data were collected retrospectively from hospital and office records. SETTING: A tertiary care children's hospital. RESULTS: The average age at diagnosis was 58 months (range, 1 day to 19 years). Fourteen children had underlying esophageal disease (atresia, n = 7 and gastroesophageal reflux, n = 7). Iatrogenic perforations occurred in 17 children: 8 during dilatation, 5 during an antireflux procedure, 2 during endoscopy, and 2 after passage of a feeding tube. Trauma was the cause of perforation in 6 children. In 2 cases the cause was unknown. Perforation occurred in the thoracic esophagus in 12 cases, abdominal esophagus in 7, cervical esophagus in 5, and involved both the thoracic and abdominal esophagus in 1. Signs and symptoms included dysphagia (15 patients), dyspnea (14), fever (12), cyanosis (8), abdominal pain (6), chest pain (5), and subcutaneous emphysema (3). Management of esophageal perforation included nonoperative management (7 patients), drainage alone (1), primary closure (16), and resection and diversion (1). Two perforations occurred in 1 child. Complications occurred in 11 (44%) of the 25 cases and were more common after delayed diagnosis (73%). The average hospital stay was 20 days. There was 1 death (4%) attributed to esophageal perforation. CONCLUSIONS: Morbidity and mortality are directly related to delays in diagnosis and therapy. Most cases of esophageal perforation in children can be closed primarily and the esophagus salvaged despite delayed presentation. The mortality rate in children with esophageal perforation (4%) is significantly less than that for adults (25%-50%).

Adolescent↗
PubMed1996

Trophic effects of interleukin-11 in rats with experimental short bowel syndrome.

Interleukin-11 (IL-11) is a multifunctional cytokine, derived from bone marrow stromal cells, that stimulates proliferation of stem/progenitor precursor cells in the small intestinal crypts and accelerates recovery of intestinal mucosa after cytoablative therapy. This study evaluates whether IL-11 can improve the function and structure of the small intestine and enhance adaptation in an experimental model of short bowel syndrome. After 90% small bowel resection, 32 Sprague-Dawley rats were divided randomly into eight experimental groups of four animals each. Four groups were treated with IL-11 (125 micrograms/kg twice daily, subcutaneously), and the four control groups were treated with a similar volume (0.1%) of bovine serum albumin (BSA). The animals were weighed daily and were killed on day 2, 4, 6, or 8; remnant small bowel was evaluated for villus height and crypt cell mitosis. The body weight of the animals that received IL-11 was significantly greater at the beginning of postoperative day 4 in comparison to that of the BSA groups (P < .01 during days 5 to 7). The rats that had IL-11 also had significantly greater villus height and crypt cell mitotic rates (P < .05). These observations suggest that IL-11 has a trophic effect on the small bowel during the adaptive phase that follows massive bowel resection and may be useful in the treatment of short bowel syndrome.

Adaptation, Physiological↗
PubMed1996

Alpha-1-proteinase inhibitor prolongs small intestinal graft preservation and survival.

Organ preservation solutions currently used for cold storage of human donor organs are less effective in preserving small intestinal grafts than other organ grafts. The maximal safe period of cold preservation for human small intestinal graft is only about 6 hours. The pathology of preserved and reperfused small intestinal grafts is characterized by mucosal autolysis and sloughing. The authors speculated that the preservation/reperfusion injury results from a proteinase/proteinase-inhibitor imbalance in the graft that favors tissue degradation. This study evaluates whether the addition of an alpha-1-proteinase inhibitor (alpha-1-PI) to the preservation solution can improve graft survival after small bowel transplantation. Forty small intestinal grafts (20 cm long) were harvested from Lewis rats. The grafts were divided randomly into three groups and were preserved in one of the following solutions: normal saline (NS) (n = 10), alpha-1-PI (25 mg/mL; n = 20), or proteinase-free bovine serum albumin (BSA) (25 mg/mL; n = 10). After 12-hour cold storage in the respective solutions, the grafts were transplanted orthotopically into syngeneic recipients. Full-thickness graft biopsies were performed before and 1 hour after revascularization. The effectiveness of preservation was judged by graft histopathology and recipient survival. Histological studies showed that there was less mucosal autolysis and sloughing of the grafts in the alpha-1-Pl group than in the other two groups. All recipients in the NS and BSA groups died of graft failure within 7 days (NS: median, 4 days; range, 2 to 5 days; BSA: median, 6 days; range, 4 to 7). However, 60% (12 of 20) of the recipients in the alpha-1-PI group survived more than 90 days (median, > 90 days; range, 4 to > 90 days; P < .005 v NS or BSA groups, log-rank method). These data suggest that the inclusion of alpha-1-PI in the preservation solution may enhance graft integrity and improve the surgical outcome after small bowel transplantation.

Animals↗
PubMed1996

Hernia survey of the Section on Surgery of the American Academy of Pediatrics.

The members of the Section on Surgery of the American Academy of Pediatrics were surveyed to determine the practice of North American pediatric surgeons in infants with inguinal hernia (IH). Case-scenario multiple-choice-design questionnaires regarding hernias and hydroceles were sent to all members of the Surgical Section, and responses were received from 292 (50%). In healthy full-term infant boys with asymptomatic reducible IH, 82% of responders perform repair electively, no matter what the age or weight. In full-term girls with a reducible ovary, 59% perform surgery at the next available time; if the ovary is nonreducible but asymptomatic, 44% operate emergently or urgently and 42% at the next elective slot. In former preemies, the pattern of repair is as follows. (1) For those recently discharged after 2 months in the neonatal intensive care unit (NICU) with reducible IH, 65% perform the repair when convenient. (2) A general anesthetic is used in 70%; 15% use spinal anesthesia, and 11% use caudal block with sedation. (3) If the repair is done in the hospital outpatient (same-day) unit, 36% wait until 50 weeks postconception (PC) and 33% wait until 60 weeks PC. (4) if the baby's weight is at least 1,000 g. 71% perform the repair before discharge. The pain control choice after childhood IH repair is Tylenol for 30%, local infiltration biquivacaine for 30%, caudal block for 22%, regional block for 11%, and Tylenol/codeine combined for 7%. In 6-week-old full-term infants with communicating hydroceles without definite "hernia," two thirds treat as an IH with elective repair as soon as possible. With respect to contralateral exploration in infants with unilateral IH, 65% perform it in males if they are < or = 2 years of age and 84% use it in females of up to 4 years of age. This approach is not influenced by presenting side, presence of hydrocele, or history of prematurity. Laparoscopic evaluation of the contralateral IH is performed by only 6% of responders, 40% of whom use the open ipsilateral sac for laparoscope introduction.

Age Factors↗
PubMed1996

Avoidance of stomas and delayed anastomosis for bowel necrosis: the 'clip and drop-back' technique.

Necrotizing enterocolitis (NEC) and midgut volvulus (MGV) often are associated with extensive bowel necrosis. These cases may require extensive enterectomy and the formation of high or multiple stomas, and frequently are complicated by short bowel syndrome, excessive fluid losses, fistulas, stenosis, and skin breakdown. This report describes a "clip and drop-back" technique, followed by delayed anastomosis performed 48 to 72 hours later. The technique was successful in five severely ill infants (3 NEC, 2 MGV) with extensive necrosis, bowel perforation(s), and peritonitis, who required either a high stoma near the ligament of Treitz or multiple resections and enterostomies. This method removes obvious necrotic perforated bowel, controls contamination, avoids stomas (and their inherent complications in this age group), and preserves bowel length. All five babies survived. The technique is a useful addition to the pediatric surgeon's operative armamentarium in selective cases.

Anastomosis, Surgical↗
PubMed1996

Gastrointestinal perforation and peritonitis in infants and children: experience with 179 cases over ten years.

BACKGROUND: Premature infants continue to have a high mortality after gastrointestinal perforation. This report describes 179 patients with gastrointestinal perforation and peritonitis and compares etiologic factors, mortality, and causes of death in premature infants and older children in an attempt to predict outcome. METHODS: The 113 boys (63.1%) and 66 girls (36.9%) had an age range of newborn (n = 139, 77.6%) to 17 years. Site of perforation was gastric in 16, duodenal in 9, small bowel in 105, colon in 37, and undesignated in 12. Eighteen had multiple perforations. Etiologic factors in newborns (younger than 2 months) included necrotizing enterocolitis (NEC) (75, 41.9%), isolated ileal perforations (30, 21.5%), malrotation/volvulus (8), iatrogenic causes (5), and others (6). Gestational age was 29.6 +/- 4.3 weeks for NEC versus 31.4 +/- 5.4 weeks for non-NEC. Birth weight for patients with NEC was 1.45 +/- 0.8 gm and 1.81 +/- 1.0 gm for non-NEC babies. Etiologic factors in 33 older children (older than 2 months to 17 years) were trauma (10), Meckel's diverticulum (4), intussusception (2), pseudomembranous colitis (2), adhesions (2), stomal leak (2), others (4), and nondesignated (7). Gastric perforations (n = 16) were iatrogenic in 7, idiopathic in 5, and caused by an ulcer in 4. RESULTS: Mortality for NEC was 36 of 75 (48%), 15 of 55 (27.2%) for non-NEC infants (p < 0.05 versus NEC), 15.1% (5 of 33) for older children (p < 0.05 versus NEC), and 4 of 16 (25%) for gastric perforation. Infant deaths were related to overwhelming sepsis, immaturity of systems, and multiorgan failure. Deaths for older children were a result of sepsis, multiorgan failure, and immunodeficiency. CONCLUSIONS: Gastrointestinal perforation is more common in premature infants with the highest mortality (48%) noted in NEC. Despite surgical intervention and advances in neonatal intensive care unit care, premature low birth weight infants (especially NEC) continue to have a high mortality.

Adolescent↗
PubMed1996

Increased risk of necrotizing enterocolitis in premature infants with patent ductus arteriosus treated with indomethacin.

OBJECTIVE: The authors evaluated the risk of necrotizing enterocolitis (NEC) in very low birth weight infants receiving indomethacin (INDO) to close patent ductus arteriosus (PDA). BACKGROUND DATA: Controversy exists regarding the best method of managing very low birth weight infants with PDA and whether to employ medical management using INDO or surgical ligation of the ductus. METHODS: Two hundred fifty-two premature infants with symptomatic PDA were given intravenously INDO 0.2 mg/kg every 12 hours x 3 in an attempt to close the ductus. Patients were evaluated for sex, birth weight, gestational age, ductus closure, occurrence of NEC, bowel perforation, and mortality. RESULTS: There were 135 boys and 117 girls. The PDA closed or became asymptomatic in 224 cases (89%), whereas 28 (11%) required surgical ligation. Ninety infants (35%) developed evidence of NEC after INDO therapy. Fifty-six were managed medically; surgical intervention was required in 34 of 90 cases (37.8%) or 13% of the entire PDA/INDO study group. Bowel perforation was noted in 27 cases (30%). Factors associated with the onset of NEC included gestational age < 28 weeks, birth weight < 1 kg, and prolonged ventilator support. The overall mortality rate was 25.5%, but was higher in infants with NEC versus those without. The highest mortality was noted in perforated NEC cases. The PDA/INDO patients were compared with a control group of 764 infants with similar sex distribution, birth weights, and gestational ages without PDA who did not receive INDO. Necrotizing enterocolitis occurred in 105 of 764 control patients (13.7%), including 13 (12.3%) with perforation. The overall mortality rate of controls was 25%, which was similar to the overall 25.5% mortality rate in the PDA/INDO study group. CONCLUSION: These data indicate that there is increased risk of NEC and bowel perforation in premature infants with PDA receiving INDO. Mortality was higher in the PDA/INDO group with NEC than those PDA/INDO infants without NEC.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1996

Delayed presentation of a penetrating biliary tract injury in a child.

An 8-year-old child presented with obstruction of the common hepatic duct from a 9-mm bullet that had entered her liver 22 months earlier. Evaluation revealed migration of the bullet with complete obstruction of the common hepatic duct. Surgical removal and reconstruction with hepaticojejunostomy has provided long-term drainage.

Child↗
PubMed1995

Analysis of morbidity and mortality in 227 cases of esophageal atresia and/or tracheoesophageal fistula over two decades.

OBJECTIVE: This report analyzes the morbidity and mortality in 227 infants (127 boys and 100 girls) with variants of esophageal atresia and/or tracheoesophageal fistula who were treated from 1971 to 1993. DESIGN: Data were collected retrospectively from hospital and office records. Mean follow-up was 76 months, ranging from 1 month to 22 years. SETTING: Patients were treated at a tertiary care children's hospital. RESULTS: The mean birth weight was 2557 g (range, 1100 to 4460 g), and the mean gestational age was 38 weeks (range, 28 to 42 weeks). Classification included 29 cases of type A esophageal atresia (13%); two cases of type B (1%), 178 cases of type C (78%), five cases of type D (2%), and 13 cases of type E (6%). Associated anomalies occurred in 146 infants (64%), including cardiac defects in 86 (38%), skeletal defects in 44 (19%), neurological defects in 34 (15%), renal defects in 35 (15%), anorectal defects in 18 (8%), and other abnormalities in 30 (13%). A single-layer anastomosis was performed in 81%, and a two-layer repair, in 17%. Esophagomyotomy was necessary in 9% of the patients. Anastomotic complications included leakage (16%), symptomatic stricture (35%), and recurrent tracheoesophageal fistula (3%). Gastroesophageal reflux was present in 127 cases (58%), with 56 (44%) requiring an antireflux procedure. Tracheomalacia occurred in 32 cases (15%), and 13 required operative treatment. Postoperative esophageal dysmotility was documented in 56 children (30%). The overall survival rate was 95%. The cause of death in 12 patients included severe cardiac anomalies (n = 3), fatal sleep apnea (n = 1), renal failure (n = 1), trisomy 18 (n = 2), accidental decannulation of tracheostomy (n = 1), pulmonary failure (n = 1), and unknown causes (n = 3). CONCLUSIONS: Early diagnosis, improved surgical technique, neonatal anesthesia, sophisticated ventilatory support, advanced intensive care management, early treatment of associated anomalies, responsiveness of anastomotic strictures to dilatation, and aggressive treatment of gastroesophageal reflux have influenced survival positively. Improved survival rates were noted irrespective of the traditional Waterston criteria, which now seem outdated. With few exceptions, most infants with esophageal atresia and/or tracheoesophageal fistula should survive in the current era.

Abnormalities, Multiple↗
PubMed1995

Complications after surgical intervention for necrotizing enterocolitis: a multicenter review.

Necrotizing enterocolitis (NEC) is a serious condition affecting predominantly the premature infant. The purpose of this study is to report a multicenter experience of complications in 252 infants requiring surgical therapy for NEC. Data from eight institutions for the years 1980 through 1990 were collected and analyzed for infants undergoing surgical therapy for NEC. Records were reviewed for gestational age, birth weight, age at operation, indications for operation, degree of intestinal involvement, operation(s) performed, complications, and 30-day mortality rates. A total of 264 infants underwent surgical intervention for NEC during the study period. Complete information was available for 252 patients. The mean gestational age was 31 +/- 5 weeks and the mean birth weight was 1,552 +/- 823 g. The mean age at operation was 18 +/- 35 days. Pneumoperitoneum was the most common indication for operation (42%). The 30-day survival rate was 72%. Eighty-one percent of patients underwent primary laparotomy, whereas peritoneal drainage was performed in 48 (19%) patients. Postoperative complications were identified in 119 (47%) patients. The most common postoperative complications were sepsis (9%), intestinal strictures (9%), and short gut (9%). Wound infections occurred in 6%, and the incidence of intraabdominal abscess formation was only 2.3%. Gestational age < 27 weeks (P < .005) and birth weight < 1,000 g (P < .005) were associated with significantly increased mortality but no increase in postoperative morbidity. The incidence of complications was similar in the very low birth weight (< 1,000 g) infants (51%) compared with infants > or = 1,000 g (46%).(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗
PubMed1995

Favorable outcome in children with Beckwith-Wiedemann syndrome and intraabdominal malignant tumors.

Children with Beckwith-Wiedemann syndrome (BWS) have an increased risk of developing Wilms' tumors, hepatoblastomas, and adrenal tumors. This study evaluates disease-free survival in children with BWS and intraabdominal tumors. Sixteen tumors occurred in 13 children with BWS (8 boys, 5 girls). Diagnoses included Wilms' tumor (10) (2 bilateral, 20%), hepatoblastoma (2), bladder rhabdomyosarcoma (1), and adrenal cortical tumor (1). In the 10 children with Wilms' tumor, the average age at diagnosis was 3.5 years (range, 7 months to 5 years). Nine of 10 had initial tumor resection, chemotherapy, and radiation therapy (when indicated). One child with bilateral disease had tumor biopsy, chemotherapy, and partial nephrectomy. Tumors were classified as stage I (5), stage II (2), stage IV (1) and stage V (2), all with favorable histology. Disease-free survival rate was 100% with median follow-up of 9 years (range, 4 to 22 years). One patient had a left adrenal tumor detected during screening sonography 11 years after Wilms' tumor resection. Two infants with advanced-stage hepatoblastoma responded to chemotherapy, allowing subsequent complete hepatic resection. Both tumors had unfavorable histology. Both completed postoperative chemotherapy and have no evidence of disease (NED) with normal alpha-fetoprotein levels at 21 and 12 months, respectively, after tumor detection. One patient with stage III (group 3) bladder rhabdomyosarcoma underwent partial cystectomy following chemoradiation and is alive (NED) after 20 months. Children with BWS should be screened at regular intervals (every 3 to 6 months) for renal, adrenal, and hepatic tumors. The exact duration of screening is not yet determined.(ABSTRACT TRUNCATED AT 250 WORDS)

Abdominal Neoplasms↗
PubMed1995

Preservation of postprandial intestinal hyperemic response after small bowel transplantation.

Postprandial intestinal hyperemia (PIH) is a normal physiological phenomenon and is thought to accommodate the increased intestinal metabolic demand during food digestion. Lack of such a response could lead to malabsorption. The purpose of this study was to determine whether transplanted intestine retains the ability to mediate PIH. Nineteen rats with syngeneic orthotopic small bowel transplantation (SBT) and 13 normal controls were further divided into two subgroups to receive gastric gavage of either 2 mL of Ensure Plus (EP) high-calorie liquid nutrition or 2 mL of normal saline. Intestinal blood flow was determined before, and 20 and 45 minutes after the gavage, employing a colored microsphere technique. The results showed that basal blood flow to the transplanted intestine (56.7 +/- 26.7 mL/min/100 g) was similar to that of normal intestine (64.2 +/- 35.8 mL/min/100 g; P > .1). EP feeding elicited a marked increase in intestinal blood flow (PIH phenomenon) in both normal and SBT animals. The magnitudes of flow increase were similar for the two groups (249 +/- 99% versus 241 +/- 76% of baseline 45 minutes after EP feeding in the normal and transplanted intestine, respectively; P > .1). The results indicate that hyperemic response to feeding is normal in syngeneic orthotopic SBT.

Animals↗
PubMed1995

Diagnosis and treatment of symptomatic breast masses in the pediatric population.

Between 1980 and 1993, 74 children and adolescents were referred for surgical evaluation of palpable breast masses. Thirty-two were managed nonoperatively for unilateral thelarche (26), fibroadenoma (3), gynecomastia (2), or hemorrhagic cyst (1). The other 42 children had surgical intervention for giant or painful fibroadenomas (19), breast abscesses (5), painful gynecomastia (6), metastatic disease (4), or other conditions (8). No instances of primary breast malignancy were noted. Physical examination and minimal (selective) diagnostic testing can conserve health care dollars in cases of pediatric patients with breast masses.

Abscess↗
PubMed1995

The role of spiral (helical) computerized tomography with three-dimensional reconstruction in pediatric solid tumors.

The ability to accurately assess tumor size and orientation to surrounding vital structures is an important consideration during preoperative evaluation. The authors report on nine children with solid tumors (hepatoblastoma [1], neuroblastoma [2], adrenal cortical carcinoma [2], liver adenoma [1], primitive neuroectodermal tumor [PNET] [1], and stage V Wilms' tumor [2]) for whom tumor resectability was questioned because of the tumors' close proximity to major blood vessels (noted through conventional radiographic imaging). The children had scanning with spiral volumetric acquisition computerized tomography, (CT) which obtains images during continuous rotation of the x-ray source while the patient moves at a constant velocity through the gantry. This technique is rapid (18 to 30 seconds), and is similar with respect to radiation exposure; little or no sedation is required, and the contrast dose is lower than that of conventional CT. Three-dimensional reconstruction of spiral CT imaging provided useful information that allowed successful resection in all nine cases. The authors suggest that spiral CT may become an important imaging modality in the preoperative evaluation of pediatric solid tumors and that further evaluation of this new methodology is warranted.

Adolescent↗