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Biomedical subjects

J C Langer

Publications and source records attributed to J C Langer.

At least 19 recordsLinked to original sources

A laparoscopic approach to partial splenectomy for children with hereditary spherocytosis.

BACKGROUND: Partial splenectomy is sometimes used for children with hereditary spherocytosis (HS) to reduce hemolysis while retaining some splenic immune function. Previous reports have described a partial splenic resection through a laparotomy incision. Whereas laparoscopic total splenectomy for HS is well-established, laparoscopic partial splenectomy (LPS) has not been described. The authors have developed a novel LPS technique that combines the benefits of partial splenectomy with those of a laparoscopic approach. METHODS: A chart review was conducted for three children with HS who underwent LPS, with approximately one-fourth of the spleen left on the basis of the short gastric arterial supply. RESULTS: The mean preoperative spleen size was 17.6 cm. The mean preoperative hemoglobin count was 100 g/l, and the postoperative hemoglobin count was 133 g/l. All three patients reported reduced malaise and increased energy levels. There was no recurrent anemia at the 1- to 2-year follow-up evaluation. CONCLUSION: The LPS procedure is a safe and effective approach to HS that resolves anemia, potentially retains some splenic immunity, and confers the benefits of a minimal access technique.

Adolescent↗

Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities.

BACKGROUND: Heterotaxia syndrome involves multiple anomalies, including cardiac malformations and intestinal rotation abnormalities. Most authors recommend routine radiological evaluation, with laparotomy and Ladd procedure if a rotation abnormality is found. AIMS: To determine if routine radiological screening is necessary, and if there is a group of children that can safely be managed expectantly. METHODS: Retrospective chart review of all children with heterotaxia syndrome from 1968 to 2002. RESULTS: Complete data were available for 177 patients. Twenty five (14%) had neonatal gastrointestinal symptoms (feeding intolerance, vomiting). Eleven of these had gastrointestinal contrast studies, of which seven were abnormal and led to surgery. Of the 152 asymptomatic neonates, nine had radiological screening and six of these were abnormal. Only one was thought to have a narrow based mesentery, but did not undergo surgery due to cardiac disease. There were no intestinal complications on follow up in this group. The other 143 asymptomatic children did not undergo radiological screening and were closely followed. Four subsequently developed gastrointestinal symptoms and had contrast studies; only one of these had malrotation and underwent a Ladd procedure. Of the remaining 139 patients who remained asymptomatic, 60 (43%) died of cardiac disease and none developed intestinal symptoms or complications related to malrotation on follow up. CONCLUSION: Asymptomatic children with heterotaxia syndrome have a low risk of adverse outcome related to intestinal rotation abnormalities. Routine screening may not be necessary as long as close follow up is done, and prompt investigation is performed for those that develop gastrointestinal symptomatology.

Cardiovascular Abnormalities↗

Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung's disease: Implications for surgical approach.

BACKGROUND/PURPOSE: The anticipated level of aganglionosis can influence the surgical approach to Hirschsprung's disease. The aim of this study was to determine the accuracy of the contrast enema in predicting this level. METHODS: Over a 6-year period (1995 through 2000), 88 patients with Hirschsprung's disease underwent surgical correction. Preoperative contrast enema findings were available for 75 of these patients and were compared with operative and pathology reports. Data were analyzed by chi(2). RESULTS: The contrast enema showed a transition zone suggestive of Hirschsprung's disease in 67 of 75 patients (89%). In 59 of 67 (88%), the pathologic and radiographic transition zones were concordant. Seven of the 8 patients with discordant studies had total colonic (n = 5) or long-segment (n = 2) disease. Contrast enema correctly predicted the level of aganglionosis in 55 of 62 (89%) patients with rectosigmoid disease but only 4 of 13 (31%) of those with long-segment or total colonic disease (P <.01). Of the patients with a radiographic transition zone in the rectosigmoid, 54 of 60 (90%) had a matching level of aganglionosis. CONCLUSIONS: In rectosigmoid Hirschsprung's disease, the location of the radiographic transition zone correlates accurately with the level of aganglionosis in 90% of cases. However, the small incidence of discordance between anticipated level of aganglionosis and operative findings should be recognized, particularly when planning a one-stage transanal pull-through.

Barium Sulfate↗

Bochdalek diaphragmatic hernia: not only a neonatal disease.

AIMS: To characterise the clinical manifestations of late presenting Bochdalek diaphragmatic hernia (DH), the incidence of misdiagnosis, and prognosis; and to explore the sequence of events that leads to this clinical picture. METHODS: Retrospective chart review. All children with Bochdalek DH were identified. Children 1 month of age and older at the time of diagnosis were included. RESULTS: Twenty two children with Bochdalek DH met the inclusion criteria. Three clinical presentations could be defined. Fourteen children presented with acute onset of symptoms, predominantly vomiting and respiratory distress. Four had chronic non-specific gastrointestinal or respiratory symptoms, and in four the DH was found incidentally. Although five children were initially misdiagnosed, in 20 children (91%) the correct diagnosis was made on x ray examination. One child experienced a complicated course when the x ray picture was misinterpreted as pneumothorax. All children had favourable outcome. Two children had previously normal chest imaging, suggesting acquired herniation. A large pleural effusion without DH in a 9.5 year old girl with an abdominal infection prior to presenting with herniation suggests a pre-existing defect in the diaphragm. CONCLUSIONS: Late presenting Bochdalek DH can present with acute or chronic gastrointestinal, or less frequently, respiratory symptoms. It can also be found incidentally. Misdiagnosis can result in significant morbidity. Favourable outcome is expected when the correct diagnosis is made. The sequence of events is probably herniation of abdominal viscera through a pre-existing diaphragmatic defect. Although rare, DH should be considered in any child presenting with respiratory distress or with symptoms suggestive of gastrointestinal obstruction.

Acute Disease↗

Is laparoscopic subtotal colectomy better than open subtotal colectomy in children?

PURPOSE: The role of laparoscopic colectomy is not defined clearly. The aim of this study was to compare clinical outcomes of laparoscopic versus open subtotal colectomy in children with inflammatory bowel disease. METHODS: Eight consecutive patients undergoing laparoscopic subtotal colectomy were compared with 10 consecutive patients undergoing open subtotal colectomy. All patients were refractory to medical management on immunosuppressive regimens. Operating time, length of postoperative stay and intravenous narcotic use, time to return of intestinal function, and perioperative complications were compared between the groups. RESULTS: Operating times were significantly longer in the laparoscopic group (mean laparoscopic, 4 hours 40 minutes v mean open 2 hours 25 minutes; P <.01). There was no difference between the 2 groups in length of postoperative intravenous narcotics or hospital stay. Ileostomy output occurred earlier (mean laparoscopic, 2.5 days v mean open 3.8 days; P =.01), and there was a trend toward earlier oral intake in the laparoscopic group. A total of 6 complications occurred in 4 patients in the laparoscopic group compared with 5 complications in 5 patients in the open group. CONCLUSIONS: Perioperative clinical outcomes, including complication rates, are similar with laparoscopic and open subtotal colectomy. Laparoscopic subtotal colectomy can be performed safely in children with improved cosmesis.

Adolescent↗

Prenatal diagnosis of esophageal atresia using sonography and magnetic resonance imaging.

BACKGROUND: The diagnosis of esophageal atresia may be suspected on prenatal ultrasound scan in fetuses with a small or absent stomach or unexplained polyhydramnios. However, these findings are thought to have a low positive predictive value and clinical decisions affecting timing or site of delivery may be made erroneously. The authors evaluated the accuracy of fetal sonography followed by magnetic resonance imaging (MRI) for the diagnosis of this lesion. METHODS: Fetuses considered to be at risk for esophageal atresia based on detailed obstetric sonography underwent fetal MRI using a single-shot rapid-acquisition technique, and the T(2)-weighted images were evaluated prospectively. Scans were considered to be positive if the proximal esophagus was dilated, and the distal esophagus was not seen and negative if the esophagus was visualized throughout its length. RESULTS: Ten fetuses underwent MRI scanning. All had a small or absent stomach bubble with unexplained polyhydramnios. Four scans were considered to be negative for esophageal atresia; all 4 were found to have a normal esophagus after delivery. Six scans were considered to be positive; 5 had esophageal atresia (2 with tracheoesophageal fistula and 3 without), and one had a neurologic syndrome with a normal esophagus. CONCLUSIONS: Magnetic resonance imaging appears to be accurate for establishing or ruling out a prenatal diagnosis of esophageal atresia, and should be considered in fetuses who are at high risk based on ultrasound findings.

Esophageal Atresia↗

Laparoscopic full-thickness intestinal biopsies in children.

BACKGROUND: Laparoscopy may reduce postoperative pain and hospital stay, compared with laparotomy. The use of laparoscopic surgery to obtain full-thickness intestinal biopsies in children has not been previously reported. METHODS: Eleven children aged 1.6 to 19 years (median, 4.5 years) underwent laparoscopic full-thickness biopsy of the stomach, small bowel, colon, or a combination thereof. Each procedure used one 12-mm and two 5-mm ports. RESULTS: Eight children with obstructive symptoms after a pull-through for Hirschsprung disease underwent multiple colon and small bowel biopsies (range, 3-6; median, 5); intestinal neuronal dysplasia was found in two. Two patients with cystic fibrosis had diffuse colonic narrowing; a diagnosis of enzyme-induced fibrosing colonopathy was made in one and nonspecific inflammation was found in the other. One child had a thickened stomach, and a gastroscopic-directed full-thickness biopsy revealed plasmacytoma. Nine of the 11 patients had a previous laparotomy, and ports were placed through preexisting scars. Median hospital stay was 2 days. No patient required more than 24 hours of narcotics. There were no leaks, and no other morbidity or mortality occurred. None of the patients required conversion to an open procedure. Biopsy results significantly affected treatment for each patient. CONCLUSIONS: Laparoscopic full-thickness intestinal biopsy is safe and effective for a variety of gastrointestinal problems in children. This technique is associated with a short hospital stay, minimal pain, and a very low risk of complications and can be performed even in patients who have had a previous laparotomy.

Adolescent↗

Laparoscopic-assisted versus open ileocolic resection for adolescent Crohn disease.

BACKGROUND: Laparoscopic-assisted ileocolic resection for Crohn disease has been reported as an acceptable alternative to the open procedure in adults. We evaluated our experience with this procedure in the adolescent population. METHODS: All adolescents undergoing ileocolic resection for Crohn disease during a 3-year period were retrospectively reviewed. Intraoperative and early postoperative results were analyzed, comparing those undergoing the laparoscopic-assisted approach with those having open resection. RESULTS: Eleven patients (mean age, 15.6 years) underwent open and 12 patients (mean age, 16.5 years) underwent laparoscopic-assisted resection. None had undergone previous resection. The two groups did not differ with respect to time from diagnosis to surgery, indications for surgery, preoperative medical therapy, operative time, or length of intestine resected. One patient in the laparoscopic-assisted group was converted to an open procedure. There were no intraoperative complications in either group. Although no statistically significant differences were noted for number of days on narcotic, total dosage of narcotic, and time to regular diet, patients undergoing laparoscopic-assisted resection were discharged 2.2 days earlier (5.4 vs. 7.6; P < 0.05). There was one wound infection and one intraabdominal abscess in the open resection group, and a single patient in the laparoscopic-assisted group with postoperative fever and a wound infection. CONCLUSIONS: Laparoscopic-assisted ileocolic resection is a safe alternative to open surgery in adolescent patients with Crohn disease.

Adolescent↗

Effect of pediatric surgical practice on the treatment of children with appendicitis.

OBJECTIVE: Acute appendicitis in children is managed by both general surgeons (GSs) and pediatric surgeons (PSs). Our objective was to investigate the economics of surgical care provided by either GSs or PSs for appendicitis. METHODS: The outcome of children within our state who underwent operative treatment for appendicitis (January 1994 to June 1997) by board-certified GSs were compared with the results of PSs. Data were sorted according to patient age and diagnosis according to the International Classification of Diseases, Ninth Revision. Analysis of variance was performed on continuous data, and chi(2) analysis was performed on nominal data; data are depicted as mean +/- standard error of the mean. RESULTS: GSs (n = 2178) managed older children when compared with PSs (n = 1018; 11.0 +/- 0.1 vs 9.1 +/- 0.1 years) and less frequently treated perforated appendicitis (18.8% vs 31.9%). Independent of diagnosis (simple or perforated appendicitis), younger children (0-4 years, 5-8 years, and 9-12 years) who were treated by PSs had a significantly shorter hospital stay and/or decreased hospital charge when compared with those who were treated by GSs. However, older children (13-15 years) seemed to have comparable outcomes. CONCLUSIONS: Younger children with appendicitis have reduced hospital days and charges when they are treated by PSs.

Adolescent↗

Roux-en-Y jejunostomy button in infants.

Establishing enteral feeding in high-risk infants with significant gastroesophageal reflux is a difficult challenge. Some patients are considered at very high risk for fundoplication and gastrostomy due to unstable medical conditions, dense upper-abdominal adhesions due to previous surgical procedures, or unfavorable anatomy. We describe a less invasive operation that provides jejunal feeding in a way that is safe, reliable, and convenient for the family.

Enteral Nutrition↗

Surgical approaches to pediatric defecatory disorders.

Defecation is one of the most basic of human activities. Problems with evacuation of feces have plagued people since the beginning of time. In modern society, defecatory disorders are the source of embarrassment, discomfort, and stress. Society's lack of openness on this subject forces many people to endure their affliction silently. Nevertheless, although these conditions consume significant medical resources, little research has been done on their causes and treatment. This review summarizes recent developments in surgical treatment of children with these disorders, either in the ability to pass stool or the ability to control defecation.

Anus, Imperforate↗

Gliomatosis peritonei as a complication of a ventriculoperitoneal shunt: case report and review of the literature.

Gliomatosis peritonei, the implantation of neuroglial tissue upon the peritoneal surfaces, is a rare event most often associated with solid or immature teratomas of the ovary in young girls. The authors report a case of a 10-month-old girl with a ventriculoperitoneal shunt (VPS) who presented with bilateral inguinal hernias. Herniorrhaphy was uneventful. Microscopic examination of the hernia sacs showed exuberant mesothelial hyperplasia containing multiple nests of differentiated glial tissue. Subsequent computed tomography and laparoscopy disclosed normal ovaries with no evidence of intraabdominal or pelvic abnormalities. Gliomatosis peritonei in this case was attributed to transport of glial tissue from the cerebrospinal fluid into the peritoneal cavity via the shunt. With the exclusion of an ovarian germ cell neoplasm and in the presence of a VPS, the clinical course with regard to the glial implants in these children is uneventful. If it is appreciated that gliomatosis peritonei may be a complication of a VPS, an extensive clinical evaluation generally is unnecessary.

Choristoma↗

Cost comparison of electrocardiography versus fluoroscopy for central venous line positioning in children.

BACKGROUND: Although most central venous lines in children are positioned using fluoroscopy, electrocardiography (ECG) has been shown to be accurate, and avoids unnecessary radiation exposure. We studied whether ECG may also have cost advantages. STUDY DESIGN: All ports and Hickman/Broviac catheters placed during a 2.5-year period were reviewed. Two surgeons routinely used fluoroscopy, and two used ECG. Costs included surgeon and anesthesia fees, operating room use, and fluoroscopy equipment and personnel. RESULTS: There were 287 cases with sufficient data to be included in the study (167 fluoroscopy and 120 ECG). In the ECG group, 12 (10%) were converted to fluoroscopy because an adequate tracing could not be obtained, but they were kept in the ECG group for data analysis. The groups were similar with regard to age, gender, indication, previous catheters, and intraoperative or postoperative complications. Time for surgical placement of the line was not significantly affected by the positioning technique. Ports placed using ECG were less costly than those placed fluoroscopically ($2,880+/-408 versus $3,595+/-357, p<0.001), and the same was true for tunneled external catheters ($2,249 +/- 435 versus $2,923+/-350, p<0.001). CONCLUSIONS: The ECG technique was less costly than fluoroscopy, despite a 10% conversion rate. At our center, the savings were approximately $700 per procedure. Because operating room time used is similar, the additional cost of fluoroscopy can be attributed to the need for x-ray equipment and personnel.

Anesthesiology↗

A prospective study of botulinum toxin for internal anal sphincter hypertonicity in children with Hirschsprung's disease.

BACKGROUND: Internal anal sphincter hypertonicity with nonrelaxation can cause persistent constipation and obstructive symptoms in children after surgery for Hirschsprung's disease. Intractable symptoms traditionally have been treated with anal myectomy, which may be ineffective or complicated by long-term incontinence. The authors evaluated prospectively the use of intrasphincteric botulinum toxin for these patients. METHODS: Eighteen children were studied (age 1 to 13; median, 4 years). Botulinum toxin was injected (total dose 15 to 60 U) into 4 quadrants of the sphincter. Resting sphincter pressure was measured in 14 patients before and after injection. Ten have had 1 to 5 additional injections (total dose, 30 to 60 U per injection). RESULTS: Four patients had no improvement in bowel function, 2 had improvement for less than 1 month, 7 had improvement for 1 to 6 months, and 5 had improvement more than 6 months. Nine of those with symptomatic improvement longer than 1 month had pressures measured, with a documented decrease in 8. Five with no significant clinical improvement had pressure measurements, with a decrease in 3. There were no adverse effects associated with botulinum toxin injection. Four children had new encopresis postinjection, which was mild and resolved in each case. CONCLUSIONS: Intrasphincteric botulinum toxin is a safe and less-invasive alternative to myectomy for symptomatic internal sphincter hypertonicity. Persistent symptoms, despite a fall in sphincter pressure, suggest a nonsphincteric etiology. Repeat injections often are necessary for recurrent symptoms.

Adolescent↗

Laparoscopic versus open splenectomy in children.

BACKGROUND: The authors have reviewed their initial experience with laparoscopic splenectomy (LS) to identify the indications, success rate, and complications associated with this procedure compared with a series of children undergoing open splenectomy (OS) during the same time period. METHODS: The records of 51 children who underwent splenectomy from 1993 through 1998 were reviewed retrospectively. RESULTS: Thirty-five patients aged 1 to 17 years (mean, 9.4 years) underwent LS for the following indications: ITP (n = 20), sickle cell disease or thalassemia (n = 6), hereditary spherocytosis (n = 5), other hematologic disorders (n = 4). Seventeen patients aged 2 to 17 years (mean, 11.8 years) underwent OS during the same time period for ITP (n = 4), sickle cell disease or thalassemia (n = 4), hereditary spherocytosis (n = 5), and other indications (n = 4). Concomitant cholecystectomy was performed in 4 of 35 LS and 4 of 17 OS. Accessory spleens were identified in 10 of 35 LS and 2 of 17 OS cases. Eleven spleens were enlarged in the LS group, and 8 were enlarged in the OS group. One LS required conversion to an open procedure because the spleen did not fit in the bag. No other cases were converted. Median estimated blood loss was 50 mL for both the LS and OS groups. The only intraoperative complication in the LS group was a splenic capsular tear, which had no effect on the successful laparoscopic removal of the spleen. No patient in either group required a blood transfusion. The LS patients had a shorter length of hospital stay (1.8 +/- 1 versus 4.0 +/- 1 day, P = .0001). Total hospital charges were not significantly different. Follow-up ranged from 6 to 40 months. One LS patient died 47 days postoperatively from unrelated causes. Two LS patients had recurrent ITP; accessory spleens were found in one and resected laparoscopically. CONCLUSION: LS in children can be performed safely with a low conversion rate (2.9%) and is associated with a shorter hospital stay and comparable total hospital cost when compared with OS.

Adolescent↗

One-stage Soave pull-through for Hirschsprung's disease: a comparison of the transanal and open approaches.

PURPOSE: The authors reviewed their experience using the transanal Soave technique, to determine (1) if it offers any advantages over the standard open approach and (2) whether routine laparoscopic visualization is necessary. METHODS: The case reports of 37 consecutive children less than 3 years old undergoing Soave pull-through were reviewed. Patients were excluded from analysis if they had total colon disease or had a previous colostomy. The patients were divided into 3 groups: open Soave (OS, n = 13), transanal Soave with routine laparoscopic visualization (LVS, n = 9), and transanal Soave with selective laparoscopy or minilaparotomy (TAS, n = 15). Cost was calculated based on hospital stay, operating room time, and use of laparoscopic equipment. RESULTS: In the TAS group, suspicion of a longer segment led to the selective use of laparoscopy with or without biopsy in 2 children, and the use of a small umbilical incision for mobilization of the splenic flexure in 2. There were no differences among groups with respect to age, weight, gender, transition zone, operating time, blood loss, intraoperative complications, enterocolitis, or stricture or cuff narrowing. Hospital stay was significantly longer in the OS group (median, 7 days; range, 3 to 47) than the LVS (median, 1; range 1 to 6) or TAS (median, 1, range, 1 to 3) groups. Cost (in thousands of dollars) was also higher in the OS group (median, 6.9; range, 3.9-25.7) than the LVS (median, 3.9; range, 3.6 to 6.4) or TAS (median, 3.4; range, 2.2 to 9.4) groups. Repeat surgery was necessary for 4 OS patients: 2 adhesive small bowel obstructions (1 of whom died), 1 twisted pull-through, and 1 recurrent aganglionosis. Three TAS patients required repeat surgery: 1 twisted pull-through, 1 anastomotic leak, and 1 cuff narrowing. CONCLUSIONS: These data suggest that the transanal pull-through is associated with a significantly shorter hospital stay and lower cost than the open approach, without an increased risk of complications. Because there is no intraabdominal dissection, there probably is a lower incidence of adhesive bowel obstruction. Routine laparoscopic visualization or minilaparotomy is not necessary but should be used in children who are at higher risk for long segment disease.

Child, Preschool↗

Routine insertion of a silastic spring-loaded silo for infants with gastroschisis.

BACKGROUND/PURPOSE: Gastroschisis traditionally is managed by emergency operating room closure (EC), with a silo reserved for cases that cannot be closed primarily. The authors recently began using routine insertion of a SILASTIC (Dow Corning, Midland, MI) spring-loaded silo (SLS), followed by elective closure. METHODS: A total of 43 consecutive neonates with gastroschisis were treated between 1993 and 1998. RESULTS: Thirty patients underwent EC, and 13 underwent closure after insertion of a SLS (10 at bedside, 3 in the operating room). Eight infants treated by EC required staged repair. There were no differences with respect to gestational age, birth weight, gender, Apgar score, maternal age, or mode of delivery. Median length of stay was 32 days for EC and 25 days for SLS (P = .05). The SLS group required fewer days on a ventilator (4 v 6 days, P = .03) and had lower intraoperative (28 v 21, P = .02) and early postoperative peak airway pressures. The time to tolerate full feedings was 21 days for SLS and 27 days for EC (P = .07). The SLS group had fewer complications and a lower median hospital charge ($71,498 v $85,147; P = .05). CONCLUSION: SLS followed by elective repair permits gentle, gradual reduction of the viscera. When compared with EC, SLS is associated with significantly lower airway pressures, earlier extubation, fewer complications, and decreased length of stay and hospital charges.

Coated Materials, Biocompatible↗

Laparoscopic surgery in children with ventriculoperitoneal shunts.

BACKGROUND/PURPOSE: Children with ventriculoperitoneal shunts may require laparoscopic surgery. The authors aimed to determine if this group of children are at greater risk for complications or technical problems. METHODS: Children with ventriculoperitoneal shunts who underwent laparoscopic surgery between 1995 and 1998 were reviewed. In addition, the subset of children undergoing laparoscopic fundoplication were compared with the group of children without shunts who- had the same operation during the same period. RESULTS: Ten children with ventriculoperitoneal shunts underwent laparoscopic surgery. Three had complications, none of which were caused by the shunt. None had any evidence of shunt dysfunction related to the laparoscopic procedure. The 6 children with shunts who underwent laparoscopic fundoplication were compared with 17 similar children without shunts who underwent the same operation during the same period. There were no differences between the groups with respect to operating time, conversion to an open approach, or complications. CONCLUSION: Laparoscopic surgery can be performed safely and effectively in children with ventriculoperitoneal shunts.

Adolescent↗