Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee.

OBJECTIVE: Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD. METHODS: Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed. RESULTS: 66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder. CONCLUSIONS: A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.

Laparoscopic excision of a giant splenic vascular tumor.

The splenic vascular tumor referred to as a hemangioma is rare and typically presents as a small asymptomatic lesion. We report a case of a giant splenic cyst in a 13-year-old boy with abdominal distension. He underwent laparoscopic excision of the splenic cyst without complication. Pathology revealed a vascular tumor. At 15 months of follow-up, he continued to be asymptomatic, and abdominal ultrasound showed no recurrence of his disease. Laparoscopic excision of giant splenic cysts is a viable option in children, allowing for preservation of normal splenic tissue.

Epidural vs patient-controlled analgesia for postoperative pain after pectus excavatum repair: a prospective, randomized trial.

PURPOSE: Management of postoperative pain is a challenge after the minimally invasive repair of pectus excavatum. Pain is usually managed by either a thoracic epidural or patient-controlled analgesia with intravenous narcotics. We conducted a prospective, randomized trial to evaluate the relative merits of these 2 pain management strategies. METHODS: After obtaining permission/assent (Institutional Review Board no. 06 08 128), patients were randomized to either epidural or patient-controlled analgesia with fixed protocols for each arm. The primary outcome variable was length of stay with a power of .8 and α of .05. RESULTS: One hundred ten patients were enrolled. There was no difference in length of stay between the 2 arms. A longer operative time, more calls to anesthesia, and greater hospital charges were found in the epidural group. Pain scores favored epidural for the few days and favored patient-controlled analgesia thereafter. The epidural catheter could not be placed or was removed within 24 hours in 12 patients (22%). CONCLUSIONS: There is longer operating room time, increase in calls to anesthesia, and greater hospital charges with epidural analgesia after repair of pectus excavatum. Pain scores favor the epidural approach early in the postoperative course and patient-controlled analgesia later.

The utility of computed tomography in the management of patients with spontaneous pneumothorax.

BACKGROUND: Spontaneous pneumothorax may result from rupture of subpleural blebs. Computed tomography (CT) has been used to identify blebs to serve as an indication for thoracoscopy. We reviewed our experience with spontaneous pneumothorax to assess the utility of CT in these patients. METHODS: A retrospective review was conducted of all patients who underwent an operation for spontaneous pneumothorax from January 1999 to October 2009. All procedures were performed thoracoscopically. RESULTS: We identified 39 pneumothoraces in 34 patients who underwent evaluation and a procedure for spontaneous pneumothorax. Mean age was 16.1 years (range, 10-23 years), with an average of 1.7 spontaneous pneumothoraces before operation (range, 1-4). Preoperative chest CT scans were obtained in 26 cases. Blebs were demonstrated on 8 CT scans. The presence of blebs was confirmed at operation in all 8 patients. Of the 18 negative scans, 14 (77.8%) were found to have blebs intraoperatively, 7 of these patients were initially managed nonoperatively and developed recurrence. The sensitivity of CT for identifying blebs was 36%. CONCLUSIONS: Chest CT does not appear to be precise in the identification of pleural blebs and a negative examination does not predict freedom from recurrence. Operative decisions should be based on clinical judgment without the use of preoperative CT.

Laparoscopic adrenalectomy in children: a multicenter experience.

INTRODUCTION: Laparoscopic adrenalectomy is now being recognized as the standard approach for adrenalectomy for benign lesions in adults. The published experience in children and adolescents has been limited to sporadic small case series. Therefore, we conducted a large multicenter review of children who have undergone laparoscopic adrenalectomy. METHODS: After Institutional Review Board's approval, a retrospective review was conducted on all patients who have undergone laparoscopic adrenalectomy at 12 institutions over the past 10 years. Operative times included unilateral adrenalectomy without concomitant procedures. RESULTS: About 140 patients were identified (70 males [50%]). Laterality included 76 (54.3%) left-sided lesions, 59 (42.1%) right, and 5 (3.6%) bilateral. Mean operative time was 130.2 ± 63.5 minutes (range 43-406 minutes). The most common pathology was neuroblastoma in 39 cases (27.9%), of which 23 (59.0%) had undergone preoperative chemotherapy. Other common pathology included 30 pheochromocytomas (21.4%), 22 ganglioneuromas (15.7%), and 20 adenomas (14.3%). There were 13 conversions to an open operation (9.9%). Most conversions were because of tumor adherence to surrounding organs, and tumor size was not different in converted cases (P=.97). A blood transfusion was required in 2 cases. The only postoperative complication was renal infarction after resection of a large neuroblastoma that required skeletonization of the renal vessels. At a median follow-up of 18 months, there was only one local recurrence, which was in a patient with a pheochromocytoma. CONCLUSIONS: The laparoscopic approach can be applied for adrenalectomy in children for a wide variety of conditions regardless of age with a 90% chance of completing the operation without conversion. The risk for significant blood loss or complications is low, and it should be considered the preferred approach for the majority of adrenal lesions in children.

Giant ossifying malignant thymoma in a child.

We present the first reported case of an ossifying pediatric thymoma. Our patient was diagnosed with a massive thymoma replacing the whole of the left thoracic cavity. Percutaneous biopsy was attempted 3 times followed by an open incisional biopsy and adjuvant chemotherapy. Complete resection required a median sternotomy and a "trap door" thoracotomy after the tumor failed to respond to chemotherapy. Histology confirmed World Health Organization type B1 lymphocyte-rich thymoma, Masaoka stage I, with extensive osseous metaplasia.

Does an upper gastrointestinal study change operative management for gastroesophageal reflux?

BACKGROUND: In patients with gastroesophageal reflux disease, an upper gastrointestinal (UGI) contrast study is often the initial study performed for those patients being considered for fundoplication. The accuracy of UGI for diagnosing reflux is known to be poor, but there are no data on how often this study influences management. Therefore, we reviewed our experience in patients undergoing fundoplication to quantify the impact of the UGI. METHODS: A retrospective analysis of our most recent 7-year experience with patients undergoing fundoplication was performed. Results of the diagnostic tests and operative course were recorded. RESULTS: From January 2000 to June 2007, 843 patients underwent fundoplication. An UGI study was obtained in 656 patients. A pH study was also performed in 379 of these patients who had an UGI. The sensitivity of the UGI for reflux compared with pH study was 30.8%. An abnormality besides gastroesophageal reflux disease or hiatal hernia that impacted the operative plan was found on the UGI in 30 patients (4.5%). The most common anomaly was malrotation, which was found in 26 patients (4.0%). Malrotation was confirmed in 16 patients and ruled out in 6 patients during fundoplication, and 4 patients had undergone a previous Ladd procedure. Esophageal dilation was performed in 5 patients with the fundoplication for a stricture found on the UGI. Pyloroplasty was performed with the fundoplication in 2 patients, and 1 patient underwent exploration of the duodenum for possible obstruction. CONCLUSIONS: The UGI study is a poor study for accurately delineating which patients have pathologic reflux. However, it reveals a finding that may influence management in approximately 4% of cases.

Does routine nasogastric tube placement after an operation for perforated appendicitis make a difference?

BACKGROUND: Divergent opinions exist regarding the routine use of nasogastric (NG) tubes in the postoperative management of patients undergoing abdominal surgery. Empiric use of an NG tube after abdominal surgery is presumed to prevent abdominal distension, vomiting, and ileus, which may complicate the postoperative course. To investigate the validity of this assumption, we compared the postoperative course of patients who underwent appendectomy for perforated appendicitis who subsequently either had or did not have an NG tube placed postoperatively. METHODS: A retrospective chart review of all children operated for perforated appendicitis between 1999 and 2004 was performed. Patients with prolonged hospitalizations were excluded to eliminate bias created by patients with multiple operations and opportunities for NG placement. The use of an NG tube, time to first and to full oral feeds, length of hospitalization, and complications were compared between groups. RESULTS: Patients with NG tubes left in place (N = 105) were compared with those who did not receive an NG tube (N = 54) following appendectomy for perforated appendicitis. Mean time to first oral intake was 3.8 d in those with NG tubes compared with 2.2 d in those without NG tubes (P < 0.001). Similarly, mean time to full feeds was 4.9 d when an NG tube was left compared with 3.4 d in those without tubes (P < 0.001). Mean length of stay was 6.0 d in those with NG tubes compared to 5.6 d in those without (P = 0.002). CONCLUSIONS: The use of NG decompression after an operation for perforated appendicitis does not appear to improve the postoperative course and we recommend that it is not routinely used in this patient population.

Laparoscopic duodenal atresia repair using surgical U-clips: a novel technique.

Laparoscopic repair of duodenal atresia has been reported. Reports to date have indicated use of standard laparoscopic suturing and knot tying. Unfortunately, there has been a high leak rate associated with the technique. We report our technique of using U-clips for the duodenoduodenostomy, thus limiting trauma to the duodenum during the anastomosis and less risk for postoperative leakage.

Adhesive small bowel obstruction after appendectomy in children: comparison between the laparoscopic and open approach.

INTRODUCTION: Adhesive small bowel obstruction (SBO) is a common postoperative complication. Published data in the pediatric literature characterizing SBO are scant. Furthermore, the relationship between the risk of SBO for a given procedure is not well described. To evaluate these parameters, we reviewed the incidence of SBO after laparoscopic appendectomy (LA) and open appendectomy (OA) performed at our institution. METHODS: With institutional review board approval, all patients that developed SBO after appendectomy for appendicitis from January 1998 to June 2005 were investigated. Hospital records were reviewed to identify the details of their postappendectomy SBO. The incidences of SBO after LA and OA were compared with chi2 analysis using Yates correction. RESULTS: During the study period, 1105 appendectomies were performed: 477 OAs (8 converted to OA during laparoscopy) and 628 LAs. After OA, 7 (6 perforated appendicitis) patients later developed SBO of which 6 required adhesiolysis. In contrast, a patient with perforated appendicitis developed SBO after LA requiring adhesiolysis (P = .01). The mean time from appendectomy to the development of intestinal obstruction for the entire group was 46 +/- 32 days. CONCLUSIONS: The overall risk of SBO after appendectomy in children is low (0.7%) and is significantly related to perforated appendicitis. Small bowel obstruction after LA appears statistically less common than OA. Laparoscopic appendectomy remains our preferred approach for both perforated and nonperforated appendectomy.

The use of fundoplication for prevention of apparent life-threatening events.

OBJECTIVE: Gastroesophageal reflux disease (GERD) is cited by many to be a common cause of apparent life-threatening events (ALTEs). However, there are few reports in the literature regarding the surgical treatment of GERD to prevent a recurrent ALTE. METHODS: A retrospective review of infants undergoing fundoplication between 2000 and 2005 for the prevention of another ALTE was undertaken. Preoperative, operative, and postoperative data as well as follow-up information were collected. RESULTS: During the study period, 81 patients underwent fundoplication after presenting with an ALTE. All but 3 patients (96.3%) had been treated with antireflux medication. Moreover, 71 infants (87.7%) were taking antireflux medication at the time of their ALTE. A significant number of infants (77.8%) were hospitalized with a second ALTE before referral for fundoplication. After fundoplication, only 3 patients (3.7%) experienced a recurrent ALTE during the follow-up period; 2 required a second fundoplication and 1 underwent pyloromyotomy. None of these 3 patients have experienced a recurrent ALTE after the second operation. The median follow-up has been 1738 days. CONCLUSION: Our data suggest that among patients who had an ALTE and are found to have GERD, fundoplication appears to be an effective method for preventing recurrent ALTE.

A comparison of chest tubes versus bulb-suction drains in pediatric thoracic surgery.

BACKGROUND/PURPOSE: Chest tubes are commonly used to evacuate the pleural space of air and fluid after thoracic surgery. The safety and efficacy of postoperative traditional chest tubes (CTs) versus soft bulb-suction drains (BDs) in the management of pediatric patients undergoing thoracic procedures were investigated. METHODS: An institutional review board-approved, retrospective review was performed on all patients who required noncardiac, nontraumatic thoracic operations from January 2000 to December 2005. Patient data included BD or CT drainage, age at operation, indication for surgery, open or thoracoscopic approach, days of postoperative drainage, the development of a postremoval pneumothorax, and complications. Statistical comparisons were made using t test and chi2 test. RESULTS: During the study period, 186 patients with complete records underwent a thoracic operation. One hundred twenty (65%) received a CT, whereas 66 (35%) received a BD. Patients who received CT averaged 5.6 days of drainage compared with 4.4 days in the group that received BD. Postremoval pneumothorax developed in 5 (4%) patients with CT compared with 4 (6%) patients with BD. Two patients in the CT group required reinsertion of another CT. None of the BD patients required further intervention. CONCLUSION: For thoracoscopic and open thoracic operations, BDs are as safe and efficacious as traditional CT.

VACTERL anomalies in patients with esophageal atresia: an updated delineation of the spectrum and review of the literature.

The VACTERL complex refers to anomalies of the bony spinal column (V), atresias in the gastrointestinal tract (A), congenital heart lesions (C), tracheoesophageal defects (TE), renal and distal urinary tract anomalies (R) and limb lesions (L). The incidence of each of these components has not been precisely quantified in the recent literature and the full array of anomalies within each systemic class of the VACTERL complex has not been well described. Therefore, we reviewed our most recent 20-year experience of patients born with esophageal atresia to comprehensively delineate and accurately describe the type and incidence of associated lesions. A retrospective review was then conducted on all patients diagnosed with esophageal atresia between 1985 and 2005. Patient demographics recorded included gestational age, weight and gender. The specific types of lesions were carefully cataloged. The outcome measure recorded was survival. One hundred and twelve patients were diagnosed with esophageal atresia were identified during the study period. The gestational age range was 28-41 weeks with an average of 36.5 weeks. Average birth weight was 2,557 g (range 1,107-3,890). A male predominance was seen with 62 males and 50 females. The overall survival was 92.9%. The categorical breakdown of anomalies were vertebral (24.1%), atresia (14.3%), cardiac (32.1%), tracheoesophageal fistula (95.5%), urinary (17.0%), skeletal (16.1%) and other (10.8%). VACTERL anomalies are common in patients with esophageal atresia, however, they appear to have little impact on overall survival.

Use of esophagocrural sutures and minimal esophageal dissection reduces the incidence of postoperative transmigration of laparoscopic Nissen fundoplication wrap.

OBJECTIVES: Herniation of the fundoplication wrap through the esophageal hiatus is a common reason for surgical failure in children who have undergone laparoscopic Nissen fundoplication. Extensive mobilization of the gastroesophageal junction in combination with decreased adhesions after laparoscopy may contribute to the development of this complication. In an attempt to decrease the incidence of wrap migration, we changed our technique to minimal mobilization of the intraabdominal esophagus and to placement of esophageal-crural sutures. In this study, we investigate the impact of these modifications on outcome. METHODS: A retrospective analysis was performed on all patients undergoing laparoscopic fundoplication by the senior author (GWH) from January 2000 through December 2004. Those undergoing operation with extensive esophageal mobilization and without esophagocrural sutures (January 2000 to March 2002) (group I) were compared with those in whom there was minimal esophageal dissection with placement of these esophagocrural sutures (April 2002 to December 2004) (group II). RESULTS: Two hundred forty-nine patients underwent laparoscopic Nissen fundoplication during the study period. One hundred thirty patients were in group I, and 119 patients were in group II. The rate of transmigration decreased from 12% in group I to 5% in group II (P = .072). The relative risk of transmigration with extensive esophageal mobilization and without the esophagocrural sutures was 2.29. CONCLUSIONS: This retrospective study has shown that placement of esophagocrural sutures and minimization of the dissection around the esophagus results in a more than 2-fold reduction in the risk of wrap transmigration after laparoscopic Nissen fundoplication.

Laparoscopic evaluation for contralateral patent processus vaginalis in children with unilateral inguinal hernia.

BACKGROUND: Management of the contralateral inguinal region in children with a unilateral inguinal hernia remains controversial. The role of laparoscopy in evaluation for contralateral patent processus vaginalis remains unclear. We report the results of an investigation of 1676 consecutive children younger than 10 years of age who underwent unilateral inguinal hernia repair between May 1992 and January 2003. MATERIALS AND METHODS: Bilateral inguinal hernias were noted preoperatively in 194 of the total of 1870 patients, leaving 1676 patients in the study group. In all patients, the contralateral inguinal region was examined under general anesthesia and the operating surgeon noted whether or not a contralateral patent processus vaginalis was suspected. All patients then underwent attempted laparoscopic evaluation of the contralateral inguinal region at the time of unilateral inguinal hernia repair. RESULTS: Laparoscopy was successful in 1603 cases (95.6%) but 73 patients (4.4%) had hernia sacs that were too thin to allow insertion of a 3-mm cannula and 2.7-mm 70-degree telescope. A contralateral patent processus vaginalis was identified in 643 of the laparoscopically examined children (40.1%). At the examination, it was predicted that 446 of the 1603 patients would have a contralateral patent processus vaginalis. Laparoscopy confirmed the presence of a contralateral patent processus vaginalis in 192 (43.0%) of the children predicted to have a contralateral patent processus vaginalis. A contralateral patent processus vaginalis was not suspected from the examination under anesthesia in 1157 of the examined children, and the absence of a contralateral patent processus vaginalis was confirmed by laparoscopy in 706 of these children (61.0%); however, a contralateral patent processus vaginalis was found in 451 (39.0%) of this group. CONCLUSION: We conclude that examination under anesthesia is a poor predictor for the presence or absence of a contralateral patent processus vaginalis. Laparoscopy can reliably evaluate the contralateral inguinal region and is the best method to evaluate for the presence of a contralateral patent processus vaginalis.