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Objective Early presentation and prompt diagnosis of acute appendicitis are necessary to prevent progression of disease leading to complicated appendicitis. We hypothesize that patients had a delayed presentation of acute appendicitis during the COVID-19 pandemic, which affected severity of disease on presentation and outcomes. Patients and methods We conducted a retrospective review of all patients who were treated for acute appendicitis at Morgan Stanley Children's Hospital (MSCH) between March 1, 2020 and May 31, 2020 when the COVID-19 pandemic was at its peak in New York City (NYC). For comparison, we reviewed patients treated from March 1, 2019 to May 31, 2019, prior to the pandemic. Demographics and baseline patient characteristics were analyzed for potential confounding variables. Outcomes were collected and grouped into those quantifying severity of illness on presentation to our ED, type of treatment, and associated post-treatment outcomes. Fisher's Exact Test and Kruskal-Wallis Test were used for univariate analysis while cox regression with calculation of hazard ratios was used for multivariate analysis. Results A total of 89 patients were included in this study, 41 patients were treated for appendicitis from March 1 to May 31 of 2019 (non-pandemic) and 48 were treated during the same time period in 2020 (pandemic). Duration of symptoms prior to presentation to the ED was significantly longer in patients treated in 2020, with a median of 2 days compared to 1 day (p = 0.003). Additionally, these patients were more likely to present with reported fever (52.1% vs 24.4%, p = 0.009) and had a higher heart rate on presentation with a median of 101 beats per minute (bpm) compared to 91 bpm (p = 0.040). Findings of complicated appendicitis on radiographic imaging including suspicion of perforation (41.7% vs 9.8%, p

Pediatric breast masses cause concern for both the patient and their family, although malignancy represents less than 1% of lesions in this population. No studies have systematically described resection and reconstructive methods for different sizes and locations of pediatric breast fibroadenomas. The aim of this study was to perform a recent systematic review of the literature regarding the surgical resection of pediatric breast fibroadenomas and to propose an algorithm for safe and effective treatment that maximizes both therapeutic and aesthetic goals while acknowledging possible future need for nipple-sparing mastectomy.A search for the phrase "pediatric breast fibroadenoma" yielded 44 results through the PubMed database. Results were reviewed and filtered to only include pertinent articles published within the last 10 years, yielding 27 results. Application of exclusion criteria resulted in 5 applicable articles. A second search was conducted using the phrase "breast fibroadenoma AND resection," which resulted in 37 studies. Fourteen articles met the inclusion and exclusion criteria from this expanded search. All 19 articles were reviewed for techniques based on the characteristics of the masses in terms of their resection patterns, and a treatment algorithm was designed.Thirty-three female subjects with an average age of 14 years were included in analysis. The average number of fibroadenomas was 1.2. The average diameter of a breast fibroadenoma was 9.1 cm, with a range from 2 to 20 cm. Resection techniques were as follows: 31% (n = 6) periareolar incision, 31% (n = 6) inframammary fold incision, 21% (n = 4) reduction mammoplasty, 10% (n = 2) mastectomy, and 5% (n = 1) mastopexy. Sixteen percent (n = 3) of studies reported the use of implants or myocutaneous flaps when breast reconstruction was warranted. No articles described long-term follow-up with regard to need for eventual mastectomy or skin complications based on prior biopsy. Based on the results of this review, an algorithm was developed to guide treatment of pediatric breast fibroadenomas.This is the first time a treatment algorithm for surgical resection has been proposed in the literature. It is our hope that this systematic review will provide guidance to surgeons in the operative management of pediatric breast fibroadenomas.

Congenital absence of the common bile duct (CBD), also known as âcholecystohepatic ductâ or âinterposition of the gallbladderâ is a rare extrahepatic biliary anomaly characterized by the hepatic ducts entering the gallbladder directly and the cystic duct draining the entire biliary tree into the duodenum. There have been only been four reports of children with congenital absence of the CBD, with three of these also carrying a diagnosis of esophageal atresia, suggesting an association between the two entities. We report an additional case of a child with a history of tracheoesophageal fistula and congenital absence of the CBD as well a review of the literature. Keywords: Gallbladder interposition, Biliary tract anomaly, Pediatric cholecystitis, Acalculous cholecystitis, Roux-en-y hepaticojejunostomy

Ingestion of neodymium magnets carries risks of dangerous intestinal complications including necrosis of the intestinal wall, bowel perforation, enteroenteric fistulae, and even death. Prompt endoscopic or surgical removal can prevent these complications. Here we report a case of unwitnessed ingestion of 19 neodymium magnets that went unrecognized for two weeks before presenting with small bowel obstruction due to a phytobezoar and numerous gastrojejunal and jejunojejunal fistulae. The child underwent a challenging laparotomy, lysis of adhesions, removal of foreign bodies, and repair of intestinal fistulae. She remained hospitalized for 12 days without significant complications and was discharged home. This case illustrates the potential for catastrophic abdominal complications to develop in a subacute fashion in patients with minimal or no initial symptoms. Pediatricians should have a low threshold to obtain plain radiographs in toddlers who present with inexplicable abdominal pain. Keywords: Magnet ingestion, Enteroenteric fistula, Phytobezoar, Small bowel obstruction

Background/purpose: Pancreatic injury is a rare complication of blunt abdominal trauma in pediatric populations. Non-operative management is increasingly common in pediatric pancreatic injury, using endoscopic interventions. We report our institutional experience with non-operative management of pediatric pancreatic trauma, with a focus on the role of endoscopic interventions. Methods: We performed a retrospective review of all cases of blunt pancreatic trauma in children presenting to our urban, academic, level I pediatric trauma center from 1996 to 2016. Results: We identified six cases of blunt pancreatic trauma in children aged 2–15 years. One patient required operative intervention for duodenal perforation, but no patients underwent pancreatic surgery. Endoscopic interventions were used in four cases to manage complications, such as pancreatic fluid collections and ductal stenosis. Conclusions: Pancreatic trauma can be managed safely and effectively using non-operative interventions in the pediatric population. Keywords: Pancreatic injury, Trauma, Non-operative management, Endoscopy, Type of study: therapeutic, Level of evidence: level IV

Congenital esophageal stenosis (CES) is a rare congenital abnormality that is difficult to diagnose and often masquerades as other types of structural esophageal disease. We report three cases of CES with different presenting symptoms. We advocate for balloon dilation as the preferred first approach to therapeutic intervention. CES is an important clinical entity in the evaluation of pediatric esophageal disorders and should be suspected in young infants with dysphagia.

An 18-month-old male infant was placed on high-frequency oscillatory ventilation for profound hypoxemia and subsequently developed tension pneumoperitoneum. He underwent a bedside exploratory laparotomy for suspected perforated viscous. No intestinal perforation was identified, and a diagnosis of tension pneumoperitoneum secondary to pneumatosis cystoides intestinalis was made. To our knowledge, this is the only report of a pediatric patient developing tension pneumoperitoneum from high-frequency oscillatory ventilation. A review of the literature examines the differential diagnosis, physiology, and treatment of tension pneumoperitoneum.

Since the first description by Frantz in 1959 of a papillary cystic tumor of the pancreas, solid pseudopapillary tumors have been increasing in incidence. However, management of these lesions remains controversial. Patients under the age of 20 years more often undergo local excision than do their older counterparts, resulting in increased recurrence rates. The cause of this discrepancy is not clear but may be related to an inability to make a definitive preoperative diagnosis. The authors report a case of a solid pseudopapillary tumor of the pancreas in a 13-year-old girl in which the diagnosis was established preoperatively by endoscopic ultrasound scan with fine-needle aspiration (EUS-FNA). EUS-FNA represents a diagnostic technique commonly used in adults that may be useful in identifying the rare pediatric patient with pancreatic malignancy.

A 1.4 kg ex-preterm infant recovering from staphylococcal sepsis/pneumonia developed increasing respiratory distress. Chest X-ray showed large bilateral pneumatocoeles, R>L (figure 1A). Mean airway pressure on continuous positive airway pressure was increased (8 cm H2O) and the baby positioned right-side-up aiming to expand the atelectatic right lung. Subsequent chest X-ray and chest CT showed an enlarging …

Computed tomography (CT) has been used more frequently to diagnose acute appendicitis in children. The purpose of this study was to determine whether the use of CT has any influence on negative appendectomy or perforation rates.Review of a prospective database of children having appendectomy for suspected acute appendicitis. Negative appendectomy and perforation rates were determined by correlation with final pathology reports.Eighty-five consecutive patients underwent appendectomy for the suspicion of acute appendicitis. The overall negative appendectomy rate was 17.6%, being 19.4% in females and 16.6% in males (p = 0.75). The overall accuracy, sensitivity and positive predictive value of CT were 75%, 91%, and 81%, respectively. Patients that had CT did not have a significantly lower rate of negative appendectomy (17.9% vs. 19.3%, p0.99) or perforation (26% vs. 17%; p = 0.53).The use of CT for the diagnosis of appendicitis in children does not change the negative appendectomy rate. Results of studies performed in adults may not be extrapolated to the evaluation of children with suspected acute appendicitis.

Background. Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm that has recently been characterized. It has not been associated with a primary visceral organ. In women, cases are even more rare and often have some ovarian involvement. Case. An 11-year-old girl presented with abdominal pain, nausea, and vomiting. A CT scan revealed a large heterogeneous pelvic mass with cystic components and an 8-cm midabdominal mass. During exploratory laparotomy, the patient was found to have a pelvic mass measuring 12.9 cm replacing normal ovarian tissue. The midabdominal mass was also removed. Pathology, cytology, and immunohistochemistry confirmed a desmoplastic small round cell tumor. Even with aggressive surgical and medical intervention, the patient died 11 months after initial diagnosis. Conclusion. We present a rare small cell tumor that is associated with ovarian involvement. The prognosis in these patients is extremely poor and very few survivals have been reported.

Catheter-related central venous thrombosis is a complication seen with long-term indwelling central venous catheters. The uses of total parenteral nutrition, catheter location, and duration of catheter use have been shown to increase the risk of thrombus formation. However, organized calcification of such a thrombus is a rare occurrence and, to our knowledge, has never been reported in a patient unexposed to total parenteral nutrition. We report a patient with an extensive, organized, calcified "cast" surrounding a central venous catheter used solely for chemotherapy administration.

Sacrococcygeal teratomas (SCTs) can present a challenging problem and can be associated with significant perinatal morbidity and mortality. A female child was born at 36 weeks' gestation with a large, vascular Type 1 SCT originally identified by prenatal ultrasound. A CT scan showed two large feeding vessels arising from both internal iliac arteries that were successfully embolized during angiography. A radiofrequency probe was then used to ablate a zone between normal tissue and the tumor. The SCT was subsequently surgically excised with minimal blood loss. This case is presented to illustrate two useful and previously unreported postnatal adjuncts to the surgical treatment of massive, hypervascular sacrococcygeal tumors.

Pancreatitis has been noted to be a potential complication in 2% to 16% of patients undergoing treatment with L-asparaginase for a variety of pediatric neoplasms, but rarely has surgical intervention been necessary. The authors present two fulminant cases of L-asparaginase-induced pancreatitis and review the current literature. The first patient is a 15-year-old boy who underwent induction chemotherapy with L-asparaginase for non-Hodgkin's lymphoma with bone marrow involvement. He presented with diffuse patchy necrosis of the pancreas as well as a large infected pancreatic pseudocyst. He subsequently required operative debridement of the pancreas and external drainage of the pseudocyst. He is currently doing well. The second patient is a 5-year-old boy who was treated with L-asparaginase for a diagnosis of acute lymphocytic leukemia. Within 3 weeks of initiation of therapy, fulminant pancreatitis developed, which progressed to multisystem organ failure. Computed tomography scan demonstrated extensive pancreatic necrosis involving 90% of the gland. He underwent surgical debridement of his necrotic pancreas and wide drainage of the lesser sac. Postoperatively he improved but subsequently multiple complications developed including erosion of his gastroduodenal artery with significant intraabdominal bleeding, which was controlled with angiographic embolization. Subsequently erosion of his endotracheal tube into the innominate vein developed, and he died. L-asparaginase-induced pancreatitis has been described after therapy for various pediatric neoplasms, and the reported cases have usually been self-limiting. However, our cases demonstrate potentially fatal sequelae of this complication and mandate early diagnosis with appropriate surgical intervention in this setting.

We present a case of congenital cystic adenomatoid malformation (CCAM) of the lung diagnosed at 17 weeks and 6 days of gestation, initially classified as a type II lesion and reclassified as a type I lesion at 19 weeks, which shows that the natural evolution of CCAM of the lung can include cyst enlargement leading to reclassification. At 22 weeks of gestation ascites developed. The second successful in-utero treatment of hydrops secondary to this condition hy serial simple aspiration is described.

The authors report 2 cases of premature neonates who had enterocutaneous fistula complicating necrotizing enterocolitis. Pulmonary hypertension developed after administration of a somatostatin analogue, octreotide, to enhance resolution of the fistula. The authors discuss the mechanism of the occurrence of this complication and recommend caution of its use in high-risk premature neonates.

Nodular fasciitis is a benign rapidly proliferating fibrous tumor that is common in adults but relatively uncommon in children. When present in children, nodular fasciitis is typically subcutaneous in location and involves the head and neck. We present a case of intramuscular nodular fasciitis involving the rectus abdominis muscle in an 11-year-old girl and discuss the importance of distinguishing this rare but benign lesion from a more aggressive sarcomatous process.

H-type rectovestibular fistula is a rare anorectal malformation with poor consensus on an optimal operative management. We report our management of a recurrent fistula and review previously described operative techniques. Full excision of the tract without apposing suture lines or perineal body dissection simplifies the repair while minimizing complications and recurrence risk.

Right-sided diverticulitis remains an exceedingly rare condition in the pediatric population. The clinical presentation mimics acute appendicitis, and the correct diagnosis is often difficult to make prior to operative exploration. We report two cases of cecal diverticulitis, and discuss the diagnosis and management of this disease, with a review of the literature.

Reduction en masse is a complication of inguinal hernia reduction in which a hernia is reduced back into the peritoneal cavity, but a loop of bowel remains trapped inside the hernia sac after reduction. Reduction en masse can be difficult to diagnose because of generalized and nonspecific symptoms. We describe a 7-year-old boy with an atypical presentation of reduction en masse after hernia reduction. The patient was a previously healthy child who presented with a mildly painful right inguinal mass of 1 day duration. An incarcerated hernia was noted, and after reduction, the pain dissipated for a short period, only to return with pain in the right lower quadrant and generalized peritoneal irritation. At surgical exploration, a reduction en masse was definitively diagnosed, and the incarcerated bowel was released from the hernia sac.

Background Hair tourniquets are commonly described in the pediatric literature. Prompt recognition of a hair tourniquet and treatment with complete removal of the hair by pediatricians, pediatric emergency room physicians, or gynecologists is essential to prevent ischemia and necrosis of affected tissue. Case Herein we present the case of a 12-year-old female referred to the pediatric surgery clinic for labial pain and swelling. She was found to have a hair tourniquet of the labia minora caused by pubic hair. The patient was taken to the operating room for examination under anesthesia and removal of the hair. Her post-operative course was unremarkable and she was discharged home the following day. On follow-up visit to the clinic her labial edema had completely resolved and she was pain free. Summary and Conclusions Unlike previous case reports that describe hair tourniquets as originating from hair on the head, our patient had a hair tourniquet caused by pubic hair. In adolescents where personal hygiene of the perineum is difficult, clinicians need to be aware of the possibility of a hair tourniquet forming from pubic hair.

Objective To report a rare case of right hemidiaphragmatic paralysis after tracheoesophageal fistula repair, and successful nonoperative management. Design Case report and literature review. Setting Pediatric intensive care unit in an academic, tertiary care medical center. Patient A neonate born to a G1P1Ab0 mother was diagnosed with tracheoesophageal fistula in the immediate postnatal period. He underwent gastrostomy and colostomy with mucous fistula on day 1 of life, and definitive repair of his esophageal atresia and tracheoesophageal fistula via a right posterolateral thoracotomy and a retropleural approach on day 6 of life. The patient failed several attempts at postoperative extubation, and a radiograph on day 11 of life revealed a persistently elevated right hemidiaphragm. Intervention Nonoperative management with noninvasive positive-pressure ventilation. Measurements and main results Restoration of normal diaphragmatic motion. Conclusions This case highlights two important points to be considered when tracheoesophageal fistula repair is performed in infants. First, phrenic nerve injury should be included in the differential diagnosis of any patient who has difficulty being weaned from the ventilator in the postoperative period after tracheoesophageal fistula repair. Second, when paralysis of the hemidiaphragm does occur, patients should receive an initial trial of nonoperative management, with diaphragmatic plication reserved for those patients who fail to regain diaphragmatic function after 4 to 6 wks.

Segmental intestinal dilatation is a rare entity presenting overwhelmingly in infants and young children with congenital malformations, anemia, or history of gastrointestinal pathology, characterized by a focally distended segment of bowel with abrupt transition points without an obstructing barrier. We present a 16-year-old girl with no significant medical history who presented with bowel obstruction clinically. Segmental dilatation of the ileum was evident on a CT scan and small bowel series. Following surgical resection, pathologic examination of the segment revealed the presence of heterotopic gastric mucosa. The girl’s symptoms resolved after surgery. Awareness of the imaging presentation of this entity can inform the evaluation of older children with nonspecific symptoms mimicking bowel obstruction.

Aberrant migration of thymic tissue occurs with ectopic thymus in the mediastinum, base of the skull, tracheal bifurcation, and cervical region. A recent review of the literature showed a total of 76 reported cases of aberrant thymus or thymic cysts in patients who presented with primary neck masses. We report three additional cases of ectopic cervical thymus. All three patients presented with asymptomatic cervical masses, and preoperative diagnosis included branchial cleft cyst, cervical lymphangioma, and cervical teratoma. All patients underwent complete surgical resection of the masses. Aberrant cervical thymus rarely produces symptoms because it does not invade contiguous strictures. Despite its rarity, it should be considered in the differential diagnosis of asymptomatic neck masses in children.

Gastroschisis and hypertrophic pyloric stenosis rarely occur in the same patient, with few reports of this unusual association published to date. We describe a neonate with prenatally diagnosed gastroschisis treated with interval silo reduction and closure. Following definitive repair, the patient had a 30-day interval of difficulty feeding and occasional emesis that progressed to projectile vomiting. Upper GI series was consistent with hypertrophic pyloric stenosis. Upon surgical exploration, multiple gastric adhesions were found to cause a 180° organoaxial gastric volvulus with aberrant relocation of the pylorus to the left upper quadrant. Following adhesiolysis and reduction of the volvulus, a Ramstedt pyloromyotomy was performed on the hypertrophied pylorus. Postoperatively, the patient's diet was advanced as tolerated, and he was discharged home on postoperative day 10.

Background . Despite improvements in survival, for infants born with esophageal atresia tracheoesophageal fistula, or both, the morbidity associated with repair of these anomalies remains high. Methods . This report retrospectively analyzes 81 patients with esophageal atresia, tracheoesophageal fistula, or both presenting to our institution between 1975 and 1995, with a focus on anastomotic complications. Results . There were 46 male and 35 female patients with a mean gestational age of 37 weeks and mean birth weight of 2443 g. Forty-four patients underwent primary esophageal anastomoses, 7 underwent delayed primary anastomoses, 12 patients underwent staged repairs, and 5 underwent repair of H-type fistulas. Among 62 patients with anastomoses, complications included stricture in 25/62 patients (40%), leakage in 12/62 patients (19%), and recurrent tracheoesophageal fistulas in 6/62 patients (10%). Stricture rates for esophagocolonic anastomoses versus esophagoesophageal anastomoses were 4/8 cases (50%) versus 21/54 cases (39%). This difference was not statistically significant. All esophagoesophageal strictures were managed successfully with dilations; three of four esophagocolonic strictures required anastomotic revision. The leakage rate for esophagocolonic anastomoses versus esophagoesophageal anastomoses was 6/8 cases (75%) versus 6/54 cases (11%). This difference was statistically significant ( p = 0.0003). Two patients required revision of their colon grafts secondary to necrosis. Eighteen of 81 patients (22%) died. Operative mortality was 9/74 (12%). Causes of death included associated anomalies (n = 15), recurrent aspiration and sepsis secondary to missed fistula (n = 1), and unknown (n = 2). Conclusions . Although the morbidity associated with surgical repair of these anomalies is high, this does not affect the overall survival. The high complication rate associated with colonic interposition suggests that one should preserve the native esophagus as a primary conduit whenever feasible.

Spontaneous perforation of the biliary tract in infancy is rare; fewer than 100 cases have been reported. The authours report on two patients with spontaneous biliary perforation (SBP) who were treated during the past 5 years. One of the patients, a 10-month-old boy, presented with an extensive retroperitoneal mass, without jaundice. He was found to have a perforation in the pancreatic portion of the common bile duct (CBD). The second patient, a 6-week-old boy, had insidious onset of jaundice, abdominal distension, and acholic stools. He was found to have a perforation at the cystic duct/CBD junction and distal CBD stricture. Both patients initially underwent cholecystostomy and drainage. The second patient had a persistent external biliary fistula and high-grade stricture, and subsequently underwent CBD excision and Roux-en-Y hepaticojejunostomy, at age 12 weeks. The authors' findings do not support the widely held concepts proposing that (1) the diminished distal ductal caliber often associated with these lesions occurs secondary to the perforation rather than is a primary causative factor and (2) the distal biliary obstruction does not require treatment because it resolves with adequate drainage. In the absence of ductal abnormalities, the authors advocate external drainage. For SBP associated with an underlying stricture, prompt biliary intestinal bypass is necessary to avoid biliary cirrhosis and portal hypertension.

We have recently reported in this journal a report of two pediatric patients with cecal diverticulitis, a 15-year-old female and a 3-year-old female. There have been no documented reports subsequent to ours; however, we have since encountered an additional pediatric patient with cecal diverticulitis to add to this case series. This most recent patient is a 10-year-old female who presented to the emergency department with a 1-day history of worsening abdominal pain. Her parents reported subjective fevers at home, but denied any nausea or emesis. In the emergency department, her vital signs were within normal limits. Physical examination was notable for tenderness to palpation in the right lower quadrant. Her white blood cell count was 12,500 mm with 72 % neutrophils. An abdominal ultrasound was performed which could not visualize the appendix, and did not demonstrate any free fluid. A computed-tomography scan was then obtained which showed a cecal wall diverticulum with wall thickening and adjacent marked inflammatory changes consistent with cecal diverticulitis. The appendix was visualized and appeared normal. Free fluid was present in the right lower quadrant, however, no pneumoperitoneum was present. The patient was started on intravenous piperacillin/ tazobactam. Based on the radiographic findings and a worsening abdominal examination, the decision was made to proceed with operative exploration. Similar to our prior cases, a transverse abdominal incision was made in the right lower quadrant. The cecal wall was found to be thickened and a rubbery mass was palpated within the wall of the cecum. An ileocecectomy was performed. Pathology revealed a perforated ‘‘false’’ cecal diverticulum with organizing serositis. The patient did well postoperatively and was discharged on postoperative day 6. This case is very similar in presentation, operative findings, and pathology results to the two previous cases we have reported. We are seeing an increased incidence of pediatric cecal diverticulitis. Further investigations are necessary to understand the etiology of this disease in the pediatric population, and to clarify the possibility of an increasing incidence in childhood.

Duplications of the rectum are extremely rare, with only 60 cases reported in the world literature. We report two patients with cystic duplications of the rectum who underwent surgical intervention in our institution during the past 10 years. A 5-year-old white male presented with constipation and tenesmus as well as a 4-cm cystic mass that protruded from the anus when he strained to pass stool. Rectal examination and ultrasound suggested the clinical diagnosis of rectal duplication. Transanal submucosal resection of a 3-cm cystic mass adherent to the posterior rectal wall was carried out. Histologic examination revealed a rectal duplication containing zones of ciliated respiratory epithelium. The second patient, a 6-year-old white female, presented with a history of chronic constipation. A large, cystic presacral mass was detected by rectal examination and confirmed by CT scan. Complete submucosal resection of the cystic duplication was performed through a posterior trans-sacral approach. Histology revealed a rectal duplication lined by respiratory epithelium. Although duplications are generally lined by mucosa of the adjacent bowel, heterotopic mucosa, most commonly gastric or pancreatic, may be present. Rectal duplications with ectopic gastric mucosa have been previously described, but to our knowledge the presence of respiratory epithelium in a rectal duplication has never been reported. Complete resection of these lesions results in cure.

Malignant thymomas are extremely rare in children, with only 27 cases reported thus far in the pediatric surgical literature. We report four additional cases diagnosed at this institution over the past 20 years (ages 3 to 14 years). Clinical presentations included superior vena cava syndrome, cough, dyspnea, cyanosis, enlarging mediastinal mass, spontaneous pneumothorax, and pleuropericardial effusion. Three patients underwent incomplete resection of the mass or biopsy because of "unresectability" and were treated with radiotherapy and adjunctive chemotherapy. One patient underwent near complete macroscopic resection as well as radiotherapy and chemotherapy. All patients died at intervals ranging from 6 months to 212 years after diagnosis. Three patients were found to have metastatic disease prior to death or at autopsy. In one case, the initial pathological diagnosis was lymphocytic thymoma. After ultrastructural studies were performed, the diagnosis was changed to thymic T-cell lymphoma. This patient subsequently developed acute lymphoblastic leukemia 3 months after surgical resection followed by radiotherapy. Malignant thymomas are highly aggressive tumors in children. A radical surgical approach with complete excision of the tumor and contiguous structures in continuity, with adjunctive radiotherapy and chemotherapy remains the only hope for survival in children with these rare lesions.

Although survival rates for infants undergoing surgical treatment for congenital intrinsic duodenal obstruction are high, long-term follow-up suggests a high complication rate related to surgical therapy. We reviewed 33 neonates who underwent surgery for congenital intrinsic duodenal obstruction during the past 10 years. There were 20 girls and 13 boys; the mean gestational age was 36 weeks, and mean birthweight was 2,485 g. Bilious vomiting and intestinal obstruction were the most frequent presenting symptoms. Hydramnios was present in 75% of cases and 21% had associated Down's syndrome. Findings at laparotomy included duodenal atresia (14), annular pancreas (11), and duodenal diaphragm (8). The most frequent surgical procedure was side-to-side duodenoduodenostomy (DD), followed by duodenojejunostomy and resection of web with Heineke-Mikulicz type duodenoplasty. Bowel transit was reestablished at a mean of 13.1 days (range, 6 to 45 days). Seventy-percent of patients developed postoperative complications, the most frequent being megaduodenum with blind loop syndrome or bile reflux gastritis (22%), cholestatic jaundice (17%), gastroesophageal reflux (17%), delayed transit (8%), and bowel obstruction (8%). Six patients (18%) required secondary surgical procedures for postoperative complications (ie, megaduodenum, nonfunctioning anastomosis, missed intrinsic stenosis). Two patients died (6%). Stagnation and functional obstruction in the proximal duodenum is the main factor influencing the morbidity rate among these patients. Consideration should be given to the tapering duodenoplasty and diamond-shaped anastomosis in order to help reduce problems associated with megaduodenum and help restore earlier bowel transit.

Forty-six neonates were treated for necrotizing enterocolitis (NEC) between 1982 and 1987. The mean gestational age was 33.6 weeks and the mean birth weight was 1865 g. Birth weight less than 1500 g and gestational age less than 32 weeks had an adverse effect on survival. Neonates less than or equal to 30 week's gestation developed symptoms at a mean age of 10.8 days while those greater than 30 weeks developed symptoms at a mean age of 1.7 days. All infants who never had enteral feedings survived; 20 were cured with medical treatment only. Three patients underwent peritoneal lavage (2 of these survived). Twenty-two patients required surgery; the operative mortality was 9%. Four patients underwent elective surgery for primary strictures. The remaining 18 presented with acute perforation (11), obstruction (5), deterioration with medical therapy (1), or peritonitis (1). The most frequent site of perforation was the terminal ileum, while the most common site of stricture was the left colon. Seven patients were found to have strictures after prior emergency surgery. In 6 of the patients who underwent primary enterostomy, the strictures were located in the defunctionalized bowel segment. Evidence of progression of the disease, which most frequently involved the right and transverse colon, necessitated extensive resection. When performing a proximal ileostomy for acute NEC, the surgeon must be aware that the chances of conservation of the defunctionalized distal bowel segment are minimal because of progression of the disease. Complications related to resection of the ileocecal valve have been negligible. Our results with peritoneal lavage are encouraging, and our overall mortality of 13% in contrast with higher rates in earlier series reflects improvement in the management of these neonates.

Leiomyoma of the esophagus is not uncommon in the adult population but is rarely seen in children; only 20 cases have been reported in the pediatric population. In this paper we describe two cases of esophageal leiomyoma in female patients aged 6 and 13 years and review previous reports. Several differences were noted between the pediatric and adult population. The mean age in children is 14 years (range, 4 to 20 years). Leiomyoma appears 1.71 times more often in females than in males. Localized lesions are found in only 9%, whereas the diffuse form predominates in 91%. The entire esophagus may be involved 35% of the time, and encroachment on the cardia or upper stomach occurs in 70%. Leiomyomas associated with familial syndromes (familial leiomyoma and Alport's syndrome) occur in 22% of the cases. Major symptoms include dysphagia (86%), dyspnea (36%), vomiting (27%), retrosternal pain (27%), and coughing (22%). The initial diagnosis following contrast studies is most often achalasia. The diagnosis of leiomyoma is made only with subsequent endoscopy. Enucleation was performed in only 11% of the cases; surgical resection (including part of the stomach) was necessary in 78% with a 21% postoperative mortality. Esophageal leiomyoma should be considered in the differential diagnosis of mediastinal masses and esophageal obstruction. Accurate preoperative diagnosis is desirable in order to plan proper surgical treatment.

Congenital neck masses—thyroglossal duct cysts (TDC) and branchial cleft cysts (BCC)—are frequently encountered in any pediatric surgical practice. While their diagnosis is usually straightforward, unusual or combined presentations may occur. We report eleven cases of unusual patterns of congenital neck masses in children. Two patients underwent resection of a BCC with a sinus tract extending through the carotid bifurcation. Both patients subsequently presented with a new mass near the previous scar, which was thought to be a recurrence but was found at surgery to have TDCs with sinus tracts extending through the hyoid bone. In one patient, a lateral neck mass (presumed to be a BCC) was found at surgery to be a TDC. In all three cases the diagnoses were confirmed histologically. Eight patients presented with a solitary thyroid nodule. Six of these had intrathyroid branchial cleft remnants, and two had intrathyroid TDCs. The diagnosis became apparent at operation in six patients, while in two it was made by the pathologist after hemithyroidectomy. Ages at presentation ranged from 16 months to 14 years. The embryology of these neck structures is closely related. It should not come as a surprise that errors in their development may occur, at times paralleling the occurrence of intrathyroid location of parathyroid glands. The possibility of an embryologic rest in the neck should therefore be kept in mind with all clinically evident neck masses. TDCs and BCCs may coexist in the same patient. The histologic differentiation may be difficult in the presence of inflammation, but differences in structure are often characteristic. While it may not be possible to distinguish the intrathyroid lesions from primary thyroid masses preoperatively, it may be possible to limit the extent of resection once the diagnosis is reached. Such knowledge may greatly assist the surgeon at the time of operation.

Adnexal torsion is rare in children and is usually reported as small series or case reports. We reviewed a series of 19 consecutive cases of children aged 3 to 19 years (mean, 9.6 years) who were treated in our institution between 1977 and 1988. Thirteen patients presented with torsion of a previously normal adnexa, while six presented with torsion of a diseased adnexa. The right adnexa was involved in 84% of cases. Detorsion with recovery of vascularization of the adnexa was possible in only four cases. All patients presented with lower abdominal pain, and onset was sudden in 78% of cases with an average of 5.2 days between the first symptom and hospital admission and a mean delay of 30.2 hours between consultation and surgical intervention. A previous history of abdominal pain was present in nine cases. Nausea or vomiting were present in 84% of cases. An abdominal mass was palpable in 42% of the patients and was associated with a delay in surgical intervention. Ultrasound confirmed the presence of a mass in 94% of cases. The preoperative diagnosis was accurate in 37% of cases, and the most common inaccurate diagnosis was appendicitis or appendiceal abcess. Our series confirms the predominance of right-sided lesions as reported in the literature. It is not clear whether this is an anatomic phenomenon or whether the suspicion of appendicitis leads to the more frequent diagnosis of right-sided lesions, whereas many left-sided adnexal torsions are being missed. We therefore advocate pelvic ultrasound in female patients who present with left lower quadrant pain. Finally, early recognition of this clinical entity and accurate preoperative diagnosis may reduce the number of unnecessary, lengthy, preoperative work-ups and may lead to more frequent salvage of torsioned adnexa.

A case of isolated complete transection of the common bile duct due to blunt abdominal trauma in a 3-year-old child is presented. The rarity of this injury and its initial presentation as a pancreatic pseudocyst warrant its description. This patient, the fifteenth child to be reported in the literature, was diagnosed as having a biliary injury following ultrasound-guided percutaneous drainage. Choledochal transection was documented at laparotomy and was successfully treated by proximal cholecystostomy and choledochojejunostomy with Roux-en-Y reconstruction. Delayed diagnosis is common, but this injury should always be kept in mind. Early diagnosis is feasible with the use of the HIDA scan. A review of the literature, modes of diagnosis, and techniques for surgical repair are described.

We report a case of mixed type IIIb and type IV intestinal atresia. The baby underwent exploratory laparotomy where he was found to have the entire distal bowel supplied by one blood vessel that appeared to have undergone an antenatal volvulus. The volvulus was untwisted and upon further exploration the patient was noted to have twelve distal atresias. We performed a total of six primary small bowel anastomoses. The baby did not require any further procedures and did well post-operatively. We conclude that patients with this congenital abnormality can be treated with primary anastomoses, and when appropriate, without the need for enterostomy or stent.

The Duhamel operation of retrorectal transanal pull-through is a widely used technique in the treatment of Hirschsprung's disease. Modifications of the technique have been made with the introduction of stapling instruments. The construction of a side-to-side colorectal anastomosis has been facilitated with the introduction of the GIA linear anastomosing instrument. The advent of the EEA stapling device has allowed us to perform a modified Duhamel operation in a 9-month-old boy with Hirschsprung's disease using mechanical sutures entirely. The technique for the procedure is described.

A 16 year old female with systemic lupus erythematosus presents with acute appendicitis. Final pathologic analysis of the appendix describes a lupus-associated vasculitis.