Posttreatment complications in pediatric cervical neuroblastoma: A retrospective case series at a tertiary cancer center.

BACKGROUND: Neuroblastomas rarely occur as primary tumors in the cervical region. Therefore, very little has been reported regarding treatment strategies, complications, and outcomes of these cervical neuroblastomas. The goal of this study is to review the presentation, management, and outcomes of all primary cervical pediatric neuroblastoma cases at a single tertiary care center. METHODS: A retrospective cohort review of all neuroblastoma patients treated at a single center were performed. All patients with primary cervical neuroblastoma were reviewed for demographic information, tumor characteristics, treatment, and outcomes. RESULTS: Thirty (1.8%) patients were found to have undergone treatment for cervical neuroblastoma tumors diagnosed on average at 2.1 years old. Most presented with a swollen neck/palpable mass ± Horner's syndrome. Based on features including tumor staging, N-myc proto-oncogene protein (MYCN) amplification status, histology, most were deemed intermediate or high risk. Treatment strategies centered around chemotherapeutic regimens with surgery when possible as well as various adjuvant treatments including radiation therapy, immunotherapy, bone marrow transplant, and a neuroblastoma vaccine. Ten (33.3%) of patients experienced treatment-related complications and four (13.3%) died as a result of their disease progression. All four patients were high-risk patients, two of which had MYCN amplification. CONCLUSION: Cervical neuroblastomas generally have favorable outcomes. These tumors can be treated effectively with chemotherapy and surgical intervention with various adjuvant therapies. MYCN amplification and higher stage disease presentation contribute to worse outcomes.

Emergency laparotomy in infants born at <26 weeks gestation: a neonatal network-based cohort study of frequency, surgical pathology and outcomes.

OBJECTIVE: Identify the proportion of infants born at <26 completed weeks' gestation who require emergency laparotomy, and review the surgical pathology, incidence of subsequent surgical procedures and outcome. DESIGN: Retrospective cohort review. SETTING: Tertiary neonatal surgical unit. PATIENTS: All infants born at <26 weeks' gestation in a neonatal network over an 8-year period. RESULTS: Of 381 infants, laparotomy was indicated in 61 (16%) and performed in 57. Surgical pathology encountered included spontaneous intestinal perforation (SIP) (28), necrotising enterocolitis (NEC) (14), volvulus without malrotation (1), strangulated inguinal hernia (1), milk curd obstruction (4), NEC stricture (1) and meconium obstruction of prematurity (2). No intestinal pathology was found in six. Four infants with indications for laparotomy and severe comorbidity had intensive care withdrawn without surgery. The most frequent procedure performed was resection with primary anastomosis. Nine infants (16%) required more than one laparotomy. Of the 16 infants who had stoma formation, eight had closure before discharge. Fifteen infants required surgical patent ductus arteriosus ligation following laparotomy, and 17 had laser therapy for retinopathy of prematurity. Overall 42 infants with indication for laparotomy (69%) survived to discharge. CONCLUSIONS: Nearly one in six infants born at <26 weeks required emergency laparotomy. The most frequent pathology encountered was SIP (49%), followed by NEC (25%). Over one-quarter required subsequent gastrointestinal surgery, with many also requiring cardiothoracic and ophthalmic procedures. These data are important for those caring for extremely preterm infants, the provision of information to parents and organisation of neonatal services.

13 ribs as a predictor of long gap esophageal atresia: myth or reality? Analysis of associated findings of esophageal atresia and abnormal rib count.

BACKGROUND: The presence of 13 pairs of ribs on pre-operative chest x-ray has been postulated to be an indicator for long gap esophageal atresia (EA). This study sought to determine the validity of this theory and identify associated pathological conditions in patients with EA and abnormal rib count. METHODS: Babies with EA from January 2005 - December 2012 were retrospectively analyzed. Information was gathered from neonatal health records and operation notes. Chest x-rays were reviewed to determine rib count. Long gap EA was defined as failure to achieve primary esophageal anastomosis. Statistical analysis performed with Fisher's exact test. RESULTS: Seventy-six patients were identified. Eight patients had long gap EA, with none of these patients having 13 pairs of ribs. Paradoxically, 10 patients with esophageal atresia +/- trachea-esophageal atresia (EA +/- TEF) and supernumerary ribs underwent primary repair. Nine patients had 11 pairs of ribs, of which 2 had pure EA and a long gap. Using Fisher's exact test to compare the groups of supernumary ribs and non-supernumary ribs there is a p value of 0.587. VACTERL association was identified in 40% of those with supernumerary ribs. Various associated syndromes and concomitant abnormalities were identified. CONCLUSION: We found no association between 13 pairs of ribs and long gap in esophageal atresia. Those with 13 pairs of ribs were more likely to have associated anomalies, although this was not statistically significant. Our cohort of patients was found to have a range of pathology related to genetic syndromes, further atresias, and malformations, which is well known to be associated with children born with EA +/- TEF. LEVEL OF EVIDENCE: Prognosis study - level IV.

Antenatally Detected Congenital Pulmonary Airway Malformations: The Oxford Experience.

Congenital airway pulmonary malformations are increasingly being diagnosed, but their management continues to remain controversial. Our approach has been to offer surgery to mitigate the risk of infection and possible malignancy. All patients routinely undergo a CT scan of the chest postnatally and once the diagnosis is confirmed, minimal access surgery is offered. Our anesthetists provide single-lung ventilation to enhance the operative view. We conducted a retrospective review over a 10-year period, during which 91 patients were prenatally suspected to have a cystic lung lesion. There were 88 live births of which 29 (33%) cases were initially managed conservatively based on CT findings. Five of these patients, however, became symptomatic needing surgery. A total of 64 (73%) patients underwent surgery with the most common lesions being congenital pulmonary airway malformations (CPAMs) (24), hybrid lesions (19), and pulmonary sequestrations (12). The median age at surgery was 5 months (1 day to 17 months). Using a minimal access approach, 41 (64%) cases were completed with 17 performed open from the onset. Open surgery was indicated in neonates who became symptomatic within the first few weeks of life as well as patients in respiratory distress that would not tolerate either single-lung ventilation or gas insufflation. There were six further conversions to open from minimal access surgery due to poor visualization or technical difficulties. One patient needed a perioperative blood transfusion and one patient had a more prolonged stay due to persistent air leak managed conservatively. Among asymptomatic patients, evidence of microscopic disease was seen, which included infection as well as two cases of tumors, one pleuropulmonary blastoma seen as part of a CPAM, and one rhabdomyomatous dysplasia seen in the CPAM component of a hybrid lesion. In our experience, excising asymptomatic lesions is safe with minimal complications. Single-lung ventilation in combination with thoracoscopy provides excellent vision. There is a risk of infection and a definite, albeit low, risk of malignancy, which may outweigh the benefits of conservative management.

Pathology of asymptomatic, prenatally diagnosed cystic lung malformations.

AIM: The management of asymptomatic congenital cystic lung malformations is controversial. Arguments for excision of asymptomatic lesions are the potential for infection and malignancy. Following antenatal detection, our institute performs a CT at 1month, clinic follow-up by 3months to discuss the controversial management, and offers surgery by 6months of age. We investigated the histopathology of asymptomatic lesions to determine whether there was evidence of subclinical infection or malignancy. METHODS: A retrospective review of prospectively collected antenatal congenital cystic lung malformations more than a 10year period (2005-2014) was conducted. Information was gathered from the antenatal registry and histopathology reports. Infection was defined by the presence of microabscesses or neutrophil/macrophage infiltration, as per histopathological criteria. MAIN RESULTS: From the cohort of 99 patients, the study focused on 69 asymptomatic lesions. These cases comprised 34 congenital pulmonary airway malformations (CPAM), 15 pulmonary sequestrations (PS), and 20 hybrid lesions. Eighteen cases (26%) had microscopic disease - 16 cases of infection and 2 tumors. The infectious cases comprised 7 with microabscesses and 9 with neutrophil/macrophage infiltration. There were two cases of tumors, namely pleuropulmonary blastoma. These tumors were followed up by the oncology team with regular imaging until 3years of age and clinical review thereafter. CONCLUSION: Twenty-six percent of antenatally detected, asymptomatic cystic lung malformations demonstrated either subclinical infection or malignancy. This information can be used for counseling parents and determining the method of treatment.

Pediatric Benign Cystic Peritoneal Mesothelioma.

BACKGROUND: Benign cystic peritoneal mesothelioma is a rare tumor comprising multiple cysts typically found within the abdominal cavity. Although the literature has numerous accounts of this entity in adults, only a handful of cases have been published regarding its presence in the pediatric population. CASE: We present the case of a prepubertal 10-year-old girl with the finding of benign cystic peritoneal mesothelioma at laparoscopy for suspected appendicitis, the management, and outcome. SUMMARY AND CONCLUSION: Although benign cystic peritoneal mesothelioma can be treated with primary excision, the high recurrence rate in adult cases of this disease necessitates the need for long-term follow-up of these patients and provides a challenge to surgeons in providing continuing care.

Paediatric trauma with hyperamylasemia.

In this case report, we describe a paediatric case of hyperamylasemia following parotid trauma. A 12-year-old boy was hit by a motorcycle, sustaining only superficial lacerations to the face overlying the parotid. A hyperamylasemia was noted, and further characterised by a fractionated isoamylase test, as being predominantly of the salivary type. Serum lipase levels were low. Based on these investigations, pancreatic injury was judged unlikely with the minor parotid trauma being the probable source of the hyperamylasemia. The patient was spared further unnecessary investigations and managed conservatively.

Congenital cystic lesions of the lung.

Congenital cystic lesions of the lung are present in 1 in 10,000-35,000 births and present as a spectrum of anomalies. Majority of these cystic lesions comprise congenital cystic adenomatoid malformations, pulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts. Most of these lesions are nowadays detected antenatally, however some will present either in the newborn or during later childhood. A review of the aetiology, classification, natural history, investigations, and treatment of congenital cystic lung lesions is discussed.