Search PubMed⌕ Search

Biomedical subjects

D H Teitelbaum

Publications and source records attributed to D H Teitelbaum.

At least 19 recordsLinked to original sources

Recent advances in the management of Hirschsprung's disease.

BACKGROUND: Major advances have occurred in the management of Hirschsprung's disease since Swenson described his definitive operation in 1948. These advances have occurred in the following areas: genetics, neurophysiology, definitive management in the newborn, total colonic aganglionosis (TCA), Hirschsprung's-associated enterocolitis (HAEC), intestinal neuronal dysplasia (IND), and laparoscopic and perineal approaches for definitive pull-through and redo pull-through operations. METHODS: This paper will focus on the definitive management of the newborn, TCA, and HAEC, areas in which we have had considerable experience at our institution. RESULTS: We have treated almost 90 newborns with the definitive pull-through with minimum morbidity. We have managed 25 patients with TCA, of whom 5 had total intestinal involvement and died. The remaining 20 have undergone a total colectomy and endorectal pull-through (ERPT), with zero mortality and a very acceptable stooling pattern and continence rate. Our experience with more than 350 patients with Hirschsprung's disease over the past 25 years has demonstrated an incidence of HAEC of between 20% and 30%. During this period, we have performed 19 redo pull-through operations, the majority of which were ERPTs, with results comparable with those seen with a primary pull-through operation. CONCLUSIONS: The major advances that have occurred in the management of Hirschsprung's disease include the definitive management of the newborn, our understanding of Hirschsprung's-associated enterocolitis and the treatment of this entity, and the recent successful management of the very complex form of this disease, total colonic aganglionosis.

Age Factors↗

Altered development of intestinal intraepithelial lymphocytes in P-glycoprotein-deficient mice.

Intraepithelial lymphocytes (IEL) that reside in the intestinal epithelium are known to exhibit phenotypic and functional characteristics that are distinct from other T cells. We have recently shown that peripheral T cells exclusively express an isoform of P-glycoprotein (P-gp) encoded by the mdr1a gene, but do not require mdr1a expression for normal proliferative, cytokine, or cytotoxic responses. In the present study, we have used mdr1-type knockout (KO) mice to demonstrate that IEL also utilize mdr1a, but only preferentially, in that the mdr1b isoform can be expressed in the absence of mdr1a expression. We also report that a high level of P-gp activity appears to be necessary for the normal development of certain IEL subpopulations. In specific, while the total number of IEL was relatively unaffected by the absence of mdr1a expression, the proportions of CD8 alpha beta and TCR alpha beta+ IEL increased significantly in mdr1a and mdr1a/b KO mice at the expense of CD8 alpha alpha and TCR gamma delta+ IEL, respectively. Moreover, these subset alterations also appeared to have functional consequences, in that proliferative, IL-2, and IFN-gamma responses of IEL from KO mice were distinct from those of normal IEL. In summary, our data suggest that mdr1a expression is required for the development of certain IEL subpopulations, most notably TCR gamma delta+ cells, and thereby indirectly influences the balance of T cell subsets in the intestinal epithelium.

ATP Binding Cassette Transporter, Subfamily B, Mem↗

Long-term follow-up of redo pull-through procedures for Hirschsprung's disease: efficacy of the endorectal pull-through.

BACKGROUND/PURPOSE: The purpose of this study was to review the authors' 25-year experience with redo pull-through procedures for Hirschsprung's disease including surgical technique and long-term outcome. METHODS: From 1974 to now, over 325 patients with Hirschsprung's disease have been treated at C.S. Mott Children's Hospital. This includes 30 patients referred after an unsuccessful pull-through at another hospital and 2 patients with an unsuccessful pull-through from C.S. Mott. All redo pull-throughs (n = 19) were performed in these patients, and their clinical courses are reviewed. RESULTS: Twelve patients required reoperation secondary to a mechanical problem with their first pull-through. The other 7 patients had evidence of residual segments of dilated colon leading to functional failure of their initial operation including 5 patients with documented aganglionic bowel present at the second pull-through. Ten of the patients requiring reoperation initially had an endorectal pull-through (ERPT), 5 had a Duhamel procedure, 3 had a Swenson procedure, and 1 had a Rehbein procedure. Choice of revision was an ERPT in 8 patients in whom an adequate rectal cuff could be developed. Additional redo procedures included a Duhamel in 8 patients and a Swenson in 3 patients. Follow-up ranges from 3 months to 23 years (mean, 13.8 years). There were no deaths in the series, and 1 patient required a third pull-through. All patients who are not neurologically impaired and are over age 3 are continent except one (94%). Stools per day range from 1 to 10 (mean, 3.2). CONCLUSIONS: Redo pull-through operations for Hirschsprung's disease appear to be as effective as primary procedures in terms of continence and stooling frequency. Distinct from other series, we found an ERPT to be the procedure of choice if an adequate rectal cuff was present.

Adolescent↗

A decade of experience with the primary pull-through for hirschsprung disease in the newborn period: a multicenter analysis of outcomes.

OBJECTIVE: To determine whether use of a primary pull-through would result in equivalent perioperative and long-term complications compared with the two-stage approach. SUMMARY BACKGROUND DATA: During the past decade, the authors have advanced the use of a primary pull-through for Hirschsprung disease in the newborn, and preliminary results have suggested excellent outcomes. METHODS: From May 1989 through September 1999, 78 infants underwent a primary endorectal pull-through (ERPT) procedure at four pediatric surgical sites. Data were collected from medical records and a parental telephone interview (if the child was older than 3 years) to assess stooling patterns. A similar group of patients treated in a two-stage fashion served as a historical control. RESULTS: Mean age at the time of ERPT was 17.8 days of life. Comparing primary ERPT with a two-stage approach showed a trend toward a higher incidence of enterocolitis in the primary ERPT group compared with those with a two-stage approach (42.0% vs. 22.0%). Other complications were either lower in the primary ERPT group or similar, including rate of soiling and development of a bowel obstruction. Median number of stools per day was two at a mean follow-up of 4.1 +/- 2.5 years, with 83% having three or fewer stools per day. CONCLUSIONS: Performance of a primary ERPT for Hirschsprung disease in the newborn is an excellent option. Results were comparable to those of the two-stage procedure. The greater incidence of enterocolitis appears to be due to a lower threshold in diagnosing enterocolitis in more recent years.

Child, Preschool↗

Congenital cystic adenomatoid malformation: impact of prenatal diagnosis and changing strategies in the treatment of the asymptomatic patient.

OBJECTIVE: This study was designed to assess the effect of prenatal sonographic diagnosis on the treatment of congenital cystic adenomatoid malformation of the lung. MATERIALS AND METHODS: The medical records of 27 patients with pathologically proven congenital cystic adenomatoid malformations were retrospectively reviewed. Patients were divided into four groups based on mode of presentation: with or without abnormal findings on prenatal sonography and with or without symptoms at birth. Age at diagnosis, age at surgical intervention, complications, and length of hospital stay were recorded for each group. RESULTS: Twenty-seven patients with 31 proven congenital cystic adenomatoid malformations were included. Eleven patients underwent prenatal sonography establishing the diagnosis (6 asymptomatic at birth, 5 symptomatic), and 16 did not have a prenatal diagnosis (10 asymptomatic at birth, 6 symptomatic). In the symptomatic populations, prenatal diagnosis had no impact on age at surgery, length of stay, or surgical complication rate (p = 0.78-0.83). In the asymptomatic population, prenatal diagnosis allowed early diagnosis (p < 0.001) and resection in the asymptomatic period. It was also associated with a shorter length of stay at the time of surgical resection (mean time, 4.2 days for patients with prenatal diagnosis versus 12.9 days for those without it;p < 0.001) and with a trend toward lower serious complication rate (3 patients without prenatal diagnosis versus 1 patient with it). CONCLUSION: Prenatal sonography provides the radiologist a means to identify congenital cystic adenomatoid malformations in a population of infants who are asymptomatic at birth. Surgical intervention in the asymptomatic infant is associated with a shorter length of stay, a trend toward fewer complications, and decreased medical cost compared with intervening after symptoms develop.

Case-Control Studies↗

Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications, and natural history.

BACKGROUND/PURPOSE: Regression of a cystic adenomatoid malformation (CAM) in a fetus is well described. Little, however, is known about the postnatal course of these infants. This study attempts to correlate the prenatal course of CAMs with postnatal symptoms, radiological manifestations, and need for surgery. METHODS: The clinical course of patients with a CAM diagnosed prenatally were retrospectively reviewed. Inclusion in the study required a prenatal ultrasound scan documenting a CAM. RESULTS: Over 10 years, 14 patients with a CAM were diagnosed prenatally. Six (43%) showed a partial in utero regression. Four patients were symptomatic at birth and underwent a resection as newborns. Ten patients were asymptomatic at birth, and eight of these had normal chest x-rays. Elective resection has been performed in 3 of these 10, and two additional children are scheduled to undergo an excision near 1 year of age. The remaining five patients have undergone follow-up nonoperatively for a mean of 36 +/- 15 months. Of the seven asymptomatic patients not undergoing immediate surgery, only one has shown a slight postnatal regression, despite five of these showing regression in utero. None have become symptomatic. CONCLUSIONS: The results suggest that regression of a CAM on prenatal ultrasound scan is common, but this process does not continue after birth. A normal chest x-ray does not indicate complete regression of a CAM; a computed tomography (CT) scan is required to evaluate such patients, and will generally demonstrate a CAM. Asymptomatic patients with a CAM may be followed up nonoperatively with no apparent adverse effects. The decision and timing of an excision in an asymptomatic patient remains controversial among pediatric surgeons.

Cystic Adenomatoid Malformation of Lung, Congenita↗

An analysis of the morbidity and mortality of short-bowel syndrome in the pediatric age group.

Twenty-two children with short-bowel syndrome (SBS) were treated at the C. S. Mott Children's Hospital in the University of Michigan Medical Center between June 1983 and May 1993. Definition of SBS was loss of 70% or more of the total small bowel. Seventeen of these children are currently alive, a 77% survival rate. Patients were followed for a mean of 1,148 days. The mean age of SBS development was 71 days of life. The only predictive indicator of patient survival was direct bilirubin levels. Sixty-seven percent of the children died if they had a direct bilirubin of > 4 mg/dl > or = 6 months duration.

Child↗

Specialty networking in pediatric surgery: a paradigm for the future of academic surgery.

OBJECTIVE: To review retrospectively a 4-year experience with pediatric surgical networking at a major academic medical center in the Midwest. BACKGROUND: The growth of managed care in the United States during the past decade has had a major impact on the practice of medicine in general, but especially on academic medicine. In some academic medical centers, the loss of market share has not only affected clinical activity but has also compromised the educational and research missions of these institutions. METHODS: At the authors' institution, a networking strategy in pediatric surgery was established in 1993 and implemented on July 1, 1994. In 1994, one new satellite practice was established; over the next 4 years, four additional practices were added, including one in another state. To assess the impact on financial status, clinical activity, education, and academic productivity, the following parameters were analyzed: gross and net revenue, surgical cases, clinic visits, ranking of the pediatric surgery residency, publications, grant support, and development and endowment funds. RESULTS: Gross and net revenue increased from $3,273,000 and $302,000 in 1993 to $10,087,000 and $2,826,000, respectively, in 1998. Surgical cases and clinic visits increased from 1240 and 3751 in 1993 to 5872 and 11,604, respectively, in 1998. At the medical center's children's hospital, surgical cases and clinic visits increased from 1240 and 3751 to 2592 and 4729 during the same time period. During this 4-year period, the faculty increased from 4 to 11. Since 1997, the National Resident Matching Program has provided data on how pediatric surgery residency candidates ranked a training program. In 1997, this program received the second-most one to five rankings; in 1998, it tied for first. This exceeds the faculty's perception of previous years' rankings. Publications increased from 26 in 1993 to a peak number of 62 in 1996; in 1997 and 1998 the publications were 48 and 37, respectively. External grant support increased from $139,882 in 1993 to a total of $6,109,971 in 1998. Development and endowment funds increased from $103,559 in 1993 to $2,702,2777 in 1998. CONCLUSIONS: Pediatric surgical networking at the authors' institution has had a markedly positive impact on finances, clinical activity, education, and academic productivity during a 4-year period. The residency training program appears to have improved in popularity among candidates, probably because of the increased referral of complex cases to the medical center from the various networking satellites. External grant support and basic laboratory research significantly increased, most likely because of the greater number of faculty with protected time for research recruited. Development and endowment funds dramatically grew because of the excellent fiscal health of the pediatric surgical program. This experience may serve as a model for other academic surgical specialties.

Academic Medical Centers↗

Alteration of the intestinal intraepithelial lymphocytes during total parenteral nutrition.

Total parenteral nutrition (TPN) may cause increased rates of bacterial translocation (BT), possibly due to a loss of epithelial integrity. Cultured epithelial cells have been shown to lose tight junction integrity with interferon gamma (INF-gamma) an action which may be blocked by transforming growth factor beta (TGF-beta). Because intraepithelial lymphocytes (IEL) are a rich source of these cytokines in the epithelium, we hypothesized that changes in the IEL, while mice were receiving TPN, may be responsible for the mediation of such cytokine responses. C57BL/6 mice were randomized to a Control group which received intravenous saline and mouse chow, or a TPN group which received intravenous TPN with no oral feeding. At 7 days mice were assessed for BT. Isolated IEL were stained for CD4, CD8, and CD44 (as a marker for memory T-cells) and flow cytometry was performed. mRNA was extracted from remaining IEL for cytokine expression. Reverse transcriptase polymerase chain reaction was performed to detect TGF-beta1 and INF-gamma mRNA expression. Densities were standardized to beta-actin expression. The incidence of BT to mesenteric lymph nodes was 40 and 12.5%, for the TPN and Control groups, respectively. TPN led to statistically significant decreases in the CD4+, CD8-; CD4+, CD8+; and the CD8+, CD44+ IEL subpopulations (P < 0.05). mRNA expression for INF-gamma was increased by 53% (P < 0.05), and TGF-beta1 mRNA expression was decreased by 75% (P = 0.1) in the IEL of TPN mice when compared with Controls. TPN led to significant changes in the IEL. Such alterations of the IEL phenotype and function may be a critical mechanism by which epithelial integrity is lost.

Animals↗

Split notochord syndrome with prolapsed congenital colostomy.

A case of split notochord syndrome associated with a prolapsed colostomylike dorsal enteric opening, a foreshortened colon, imperforate anus, and meningocele is presented. The surgical management of this disorder is discussed and available literature is reviewed. The patient was successfully treated with a combined, single-stage surgical correction.

Abnormalities, Multiple↗

Latex sensitization in general pediatric surgical patients: a call for increased screening of patients.

BACKGROUND/PURPOSE: Latex sensitization is a well-documented occurrence in children with myelodysplastic and urologic anomalies. The incidence of latex allergy in general pediatric surgical patients, however, has not been previously addressed. The purpose of this study was to examine the risk of perioperative latex reactions in a general pediatric surgical practice over a 1-year period. METHODS: This study examined the occurrence of latex sensitization using two methods. First, the preoperative anesthesia records of patients that have undergone surgery from October 1995 through September 1996 at Mott Children's Hospital were reviewed retrospectively. Second, all patients who had intraoperative anaphylaxis attributable to latex sensitization, including those from three additional hospitals, were evaluated. RESULTS: During a 12-month period, 1,523 pediatric general surgical operations were performed at the C.S. Mott Children's Hospital. Of these, only 11 operations on five patients were performed under latex precautions. All of these patients had a preoperative diagnosis of latex sensitivity. During the same period, intraoperative anaphylactic reactions caused by latex allergy occurred in two of the general surgical patients (0.13%) at the C.S. Mott Hospital. Four additional cases are also reported from other study hospitals. None of these patients were suspected, based on current screening methods, of having a latex allergy before their surgery. CONCLUSIONS: Latex allergy is a potentially life-threatening condition in the pediatric general surgical population. Further study is needed to develop criteria to preoperatively identify patients at risk for latex sensitization.

Adolescent↗

The lateral approach for open splenectomy.

BACKGROUND: Laparoscopic splenectomy (LS) has been used increasingly to treat children with hematologic disorders and has been reported to have advantages over open splenectomy performed through a standard vertical or subcostal incision. The authors perform open splenectomy (OS) through a lateral, muscle-splitting approach, and believe their approach is more reasonable in comparison with LS. METHODS: Thirty-nine consecutive open splenectomies performed between 1991 and 1995 were reviewed retrospectively and compared with recent reports of LS. The series included 24 boys and 15 girls with an average age of 9 years and average weight of 37.5 kg. Indications included immune thrombocytopenic purpura (n = 20), hereditary spherocytosis (n = 18), and sickle cell anemia (n = 1). The operation was performed with the child in the lateral decubitus position through a left upper abdominal muscle-splitting incision (off the 11th rib), sparing the rectus muscle. RESULTS: All 39 cases were completed without intraoperative complications with an average surgical time of 98.0 minutes (range, 30 to 302). The average surgical blood loss was 89 mL (range, 10 to 300). The children started feeding an average of 1.2 days (range, 0 to 4) postoperatively, were on a regular diet at an average of 2.0 days (range, 1 to 6) postoperatively, and had an average length of stay of 2.7 days (range, 1 to 6). There was no mortality or morbidity. CONCLUSIONS: Open lateral splenectomy is performed with shorter surgical times, less blood loss, an excellent cosmetic result, no complications, and a length of stay comparable to any of the published series on laparoscopic splenectomy in children. This approach provides a reasonable basis for comparison with laparoscopic splenectomy.

Adolescent↗

Enterocolitis.

Enterocolitis continues to be the major cause of morbidity and mortality in patients with Hirschsprung's disease. The exact etiology of Hirschsprung's-associated enterocolitis is not known. This review focuses on the clinical aspects, etiology, and therapy of Hirschsprung's-associated enterocolitis.

Enterocolitis↗

Simplified insertion of a transpyloric feeding tube.

Placement of a transpyloric feeding tube is a common procedure done through a previous gastrostomy site. Conventional fluoroscopic and endoscopic methods can be tedious because of the difficulty in cannulating the pylorus. Described here is a simplified method to place a transpyloric feeding tube under fluoroscopy.

Child↗