Lipoblastoma-Related Volvulus in a Nine-Year-Old Female.

Lipoblastomas are benign neoplasms that arise from embryonal adipocytes. They predominantly impact the pediatric population, with most cases occurring in the first few years of life. These tumors typically present as a soft, palpable, painless mass and tend to involve the mesenchymal tissues of the extremities and trunk. Intraabdominal involvement and intraabdominal complications secondary to lipoblastoma are incredibly rare. Here, we present the case of a nine-year-old female who presented to the emergency department (ED) with one week of intermittent lower abdominal pain. The CT abdomen/pelvis demonstrated a well-circumscribed hypodense omental mass measuring 10.1 cm x 4.7 cm x 13.4 cm with minimal mass effect or bowel displacement. At that time, the patient's abdomen was soft without tenderness, distention, or rigidity. Her initial laboratory studies and vital signs were within normal limits. She was evaluated by pediatric surgery, who, given her clinical stability, planned for an anticipated elective resection. Thirteen days after her initial ED visit, the patient returned to the ED with nausea, vomiting, and diffuse abdominal pain. Repeat CT abdomen/pelvis revealed shifting of the omental mass from the left hemi-abdomen to the right hemi-abdomen with associated mesenteric 'swirl sign' and dilated loops of small bowel consistent with small bowel obstruction. Given the patient's CT findings and signs of peritonitis on a physical exam, she was emergently taken to the operating room, where the mass along with 20 cm of small bowel intimately associated with the mass was resected. The proximal end of the involved bowel was found to be twisted and necrotic, consistent with volvulus. A specimen was sent for cytogenetics and found to be positive for FLAG1, ultimately revealing a diagnosis of lipoblastoma. The majority of lipoblastoma development is underpinned by gene rearrangements in the zinc-finger transcription factor PLAG1. Although benign, these tumors can exhibit rapid proliferation and have high recurrence rates. Patients should be monitored long-term with ultrasound (US) or MRI following surgery to assess for recurrence.

Progressive reduction of hospital length of stay following minimally invasive repair of pectus excavatum: A retrospective comparison of three analgesia modalities, the role of addressing patient anxiety, and reframing patient expectations.

PURPOSE: Management of postoperative pain is a significant challenge following the Nuss procedure. Epidurals, PCAs, and newer analgesia modalities have been used elsewhere without demonstrating consistent improvement in the reported length of hospital stays (LOS). We reviewed a large single surgeon experience identifying three different methods of analgesia used over time to highlight marked improvement in patient LOS. METHODS: IRB approval was obtained and patient clinical information was retrospectively reviewed from 2001 to 2017. The primary outcome variable was length of hospital stay. An expanded preoperative consultation reviews the issue of pain, the negative impact of anxiety on recovery, and our current success of shortened hospital stays with our patients. RESULTS: One hundred and seventy-three patients representing three different analgesia approaches had a LOS of 4.4 days (epidural); 2.2 days (PCA/intercostal nerve block); and 1.6 days (scheduled oral pain meds/intercostal nerve blocks). The current LOS for patients is 1.0 day. Patients successfully stop using narcotics by the end of the first week postoperatively. CONCLUSIONS: Intraoperative intercostal nerve blocks, scheduled postoperative pain medications, and enhanced preoperative consultation aimed to educate patients about anxiety and reframe patient pain expectations have collectively decreased LOS, and reduced postoperative narcotic usage. TYPE OF STUDY: Clinical research LEVEL OF EVIDENCE: Level III.

Reduced and Compressed Cisplatin-Based Chemotherapy in Children and Adolescents With Intermediate-Risk Extracranial Malignant Germ Cell Tumors: A Report From the Children's Oncology Group.

Purpose To investigate whether event-free survival (EFS) can be maintained among children and adolescents with intermediate-risk (IR) malignant germ cell tumors (MGCT) if the administration of cisplatin, etoposide, and bleomycin (PEb) is reduced from four to three cycles and compressed from 5 to 3 days per cycle. Patients and Methods In a phase 3, single-arm trial, patients with IR MGCT (stage II-IV testicular, II-III ovarian, I-II extragonadal, or stage I gonadal tumors with subsequent recurrence) received three cycles of PEb. A parametric comparator model specified that the observed EFS rate should not be significantly < 92%. As recommended for trials that test a reduction of therapy, a one-sided P value ≤ .10 was used to indicate statistical significance. In a post hoc analysis, we also compared results to the EFS rate of comparable patients treated with four cycles of PEb in two prior studies. Results Among 210 eligible patients enrolled from 2003 to 2011, 4-year EFS (EFS4) rate was 89% (95% confidence interval, 83% to 92%), which was significantly lower than the 92% threshold of the comparison model ( P = .08). Among 181 newly diagnosed patients, the EFS4 rate was 87%, compared with 92% for 92 comparable children in the historical cohort ( P = .15). The EFS4 rate was significantly associated with stage (stage I, 100%; stage II, 92%; stage III, 85%; and stage IV, 54%; P < .001). Conclusion The EFS rate for children with IR MGCT observed after three cycles of PEb was less than that of a prespecified parametric model, particularly for patients with higher-stage tumors. These data do not support a reduction in the number of cycles of PEb from four to three. However, further investigation of a reduction in the number of cycles for patients with lower-stage tumors is warranted.

Impact of central surgical review in a study of malignant germ cell tumors.

BACKGROUND: Verification of surgical staging has received little attention in clinical oncology trials. Central surgical review was undertaken during a study of malignant pediatric germ cell tumors. METHODS: Children's Oncology Group study AGCT0132 included central surgical review during the study. Completeness of submitted data and confirmation of assigned stage were assessed. Review responses were: assigned status confirmed, assignment withheld pending review of additional information requested, or institutional assignment of stage disputed with explanation given. Changes in stage assignment were at the discretion of the enrolling institution. RESULTS: A total of 206 patients underwent central review. Failure to submit required data elements or need for clarification was noted in 40%. Disagreement with stage assignment occurred in 10% with 17/21 discordant patients reassigned to stage recommended by central review. Four ovarian tumor patients not meeting review criteria for Stage I remained in that stratum by institutional decision. Two-year event free survival in Stage I ovarian patients was 25% for discordant patients compared to 57% for those meeting Stage I criteria by central review. CONCLUSIONS: Central review of stage assignment improved complete data collection and assignment of correct tumor stage at study entry, and allowed for prompt initiation of chemotherapy in patients determined not to have Stage I disease.

Surveillance after initial surgery for pediatric and adolescent girls with stage I ovarian germ cell tumors: report from the Children's Oncology Group.

PURPOSE: To determine whether overall survival (OS) can be preserved for patients with stage I pediatric malignant ovarian germ cell tumor (MOGCT) with an initial strategy of surveillance after surgical resection. PATIENTS AND METHODS: Between November 2003 and July 2011, girls age 0 to 16 years with stage I MOGCT were enrolled onto Children's Oncology Group study AGCT0132. Required histology included yolk sac, embryonal carcinoma, or choriocarcinoma. Surveillance included measurement of serum tumor markers and radiologic imaging at defined intervals. In those with residual or recurrent disease, chemotherapy with compressed PEB (cisplatin, etoposide, and bleomycin) was initiated every 3 weeks for three cycles (cisplatin 33 mg/m(2) on days 1 to 3, etoposide 167 mg/m(2) on days 1 to 3, bleomycin 15 U/m(2) on day 1). Survivor functions for event-free survival (EFS) and OS were estimated using the Kaplan-Meier method. RESULTS: Twenty-five girls (median age, 12 years) with stage I MOGCT were enrolled onto AGCT0132. Twenty-three patients had elevated alpha-fetoprotein (AFP) at diagnosis. Predominant histology was yolk sac. After a median follow-up of 42 months, 12 patients had evidence of persistent or recurrent disease (4-year EFS, 52%; 95% CI, 31% to 69%). Median time to recurrence was 2 months. All patients had elevated AFP at recurrence; six had localized disease, two had metastatic disease, and four had tumor marker elevation only. Eleven of 12 patients experiencing relapse received successful salvage chemotherapy (4-year OS, 96%; 95% CI, 74% to 99%). CONCLUSION: Fifty percent of patients with stage I pediatric MOGCT can be spared chemotherapy; treatment for those who experience recurrence preserves OS. Further study is needed to identify the factors that predict recurrence and whether this strategy can be extended successfully to older adolescents and young adults.

Schwannoma of the diaphragm--a pediatric case report and review of the literature.

Diaphragmatic tumors are uncommon and difficult to diagnose in the pediatric population. Schwannoma is a benign, slow-growing peripheral nerve sheath tumor that is most commonly associated with the extremities in childhood. We herein report a schwannoma of the diaphragm in a pediatric patient and review the pediatric literature.

Do international rotations make surgical residents more resource-efficient? A preliminary study.

OBJECTIVE: Interest in international surgery among general surgery residents in the United States has been shown in several publications. Several general surgery residency programs have reported their experiences with international surgery rotations (ISRs). Learning to use limited resources more efficiently is often cited as a benefit of such rotations. We hypothesized that general surgery residents become more resource efficient after they have completed an ISR. STUDY DESIGN: Laboratory, radiologic, and diagnostic studies ordered on 2900 patients by 21 general surgery residents over 65 months at a single institution were analyzed retrospectively. The patient populations they wrote orders on were assessed for similarity in age, gender, and diagnoses. The outcomes in those patient populations were assessed by duration of stay and in-hospital mortality. Six (29%) of these residents (ISR residents) completed a 1-month ISR during their third year of residency. Their orders were compared with their classmates who did not participate in an ISR (NISR residents). The results were compared between the 2 cohorts from both before and after their international rotations. An analysis focused on comparing the changes from pre-ISR to post-ISR. A survey was also sent after objective data were collected to all residents and alumni involved in the study to assess their subjective perception of changes in their resource efficiency and to characterize their ISRs. RESULTS: Patient populations were similar in terms of demographics and diagnoses. ISR residents generated an average of $122 less in orders per patient per month after their ISR compared with before. NISR residents generated an average of $338 more in orders per patient per month after the ISRs compared with before (p = 0.04). Pre-ISR order charges were statistically similar. Similar results were observed when radiologic/diagnostic study orders were analyzed independently. Differences in outcomes were statistically insignificant. The survey revealed that most of the ISR residents perceived that their attitude toward ordering tests and laboratories was influenced greatly by their ISR, and all the ISR residents perceived that they became more resource efficient than their peers after their ISRs. CONCLUSION: These preliminary findings seem to indicate increased resource efficiency among general surgery residents who completed an ISR. However, the sample size of residents was small, and we could not establish conclusively a causal relationship to their ISRs. A more extensive study is needed if reliable conclusions are to be drawn regarding the effect of ISRs on the resource efficiency of residents.

Professional values, value conflicts, and assessments of the duty-hour restrictions after six years: a multi-institutional study of surgical faculty and residents.

BACKGROUND: the aim of this study was to explore professional values, value conflicts, and assessments of the Accreditation Council for Graduate Medical Education's duty-hour restrictions. METHODS: questionnaires distributed at 15 general surgery programs yielded a response rate of 82% (286 faculty members and 306 residents). Eighteen items were examined via mean differences, percentages in agreement, and significance tests. Follow-up interviews with 110 participants were explored for main themes. RESULTS: residents and faculty members differed slightly with respect to core values but substantially as to whether the restrictions conflict with core values or compromise care. The average resident-faculty member gap for those 13 items was 35 percentage points. Interview evidence indicates consensus over professional values, a gulf between individualistic and team orientations, frequent moral dilemmas, and concerns about the assumption of responsibility by residents and "real-world" training. CONCLUSIONS: the divide between residents and faculty members over conflicts between the restrictions, core values, and patient care poses a significant issue and represents a challenge in educating the next generation of surgeons.

A new professionalism? Surgical residents, duty hours restrictions, and shift transitions.

BACKGROUND: Some anticipated that the Accreditation Council for Graduate Medical Education duty hours restrictions would foster a team-focused "new professionalism" among residents. This study explores the prevalence and challenges of a new professionalism and whether they vary by program size. METHOD: Questionnaires distributed in 15 general surgery programs produced an 82% response rate (N = 306); 52 semistructured follow-up interviews were completed. Results include means, percentage who "agree or strongly agree," significance tests, and main themes from the interviews. RESULTS: A new professionalism is limited by residents' reluctance to pass work from day to night teams, unclear guidance regarding stay-or-go decisions during shift transitions, little educational emphasis on sign-outs, and the practice of long hours in the name of professionalism. Program size is largely unassociated with these beliefs and behaviors. CONCLUSIONS: A new professionalism represents a stalled revolution among surgical residents. The new professionalism's emphasis on teamwork requires additional attention to staffing and workload management.

Gastric duplication cyst: a unique presentation.

Duplications of the alimentary tract are rare and occur in 1 of 4500 births (Duplication of the stomach: report of a case and review of the English literature. Arch Surg 1961; 82:634-640). Gastric duplications constitute 8% of these or roughly 17 of every 1,000,000 births (Shew SB, Holcomb GW. Alimentary tract duplications. In: Ashcraft KW, Holcomb GW, Murphy JP, editors. Pediatric surgery. Philadelphia, PA: Elsevier, 2005. p. 543-552). Symptoms often occur by 2 years and can include nausea, vomiting, hematemesis, and vague abdominal pain. Occasionally, a palpable abdominal mass may be identified on physical examination. We offer an unusual and previously unreported presentation of a gastric duplication cyst.

Pediatric germ cell tumors.

Pediatric germ cell tumors are a diverse group of neoplasms with variable clinical behaviors, depending upon the age and site of presentation. Most result from sporadic mutations, although environmental exposures and other genetic aberrations may play a role. Platinum-based chemotherapy has dramatically improved the event-free and overall survival outcomes of pediatric patients with malignant germ cell tumors over the past two decades. Prognosis is dependent on tumor stage and location. Patients with gonadal germ cell tumors have at least a 95% 5-year survival for early stage disease and at least a 85% 5-year survival for advanced stages. In general, extragonadal germ cell tumors carry a poorer prognosis with mediastinal location having the worst outcomes (70% 4-year survival). Current trials are focused on maintaining similar excellent outcomes while reducing morbidity by reducing the dose and duration of chemotherapy. Cytogenetic research studies have found chromosomal aberrations specific to some of these tumors that may serve as prognosticators and even direct therapy.

Imperforate anus and colon atresia in a newborn.

Imperforate anus is an uncommon congenital anomaly. Colon atresia is even more infrequent. This report describes a newborn with the simultaneous occurrence of these 2 anomalies, a condition that is exceedingly rare.

Primary thoracoscopic gross total resection of neuroblastoma.

BACKGROUND: Thoracic neuroblastomas are generally less aggressive and have a better prognosis than those arising below the diaphragm. Our purpose was to study the safety and efficacy of thoracoscopic resection and to evaluate tumor data and patient outcomes. PATIENTS AND METHODS: We reviewed the records of patients who underwent primary thoracoscopic resection of neuroblastoma (NB) between 1998 and 2002. Data included demographics, symptoms, size, location, operative time, complications, hospital stay, histology, biologic markers, adjuvant therapy, and outcome. RESULTS: Five patients (age range, 9 to 44 months) underwent thoracoscopic resection of NB. Three of the patients had neurological symptoms. Tumor size ranged from 2.1 to 6.0 cm. Two tumors were apical, three supradiaphragmatic. Primary thoracoscopic gross total resection was achieved in all 5 cases, all of which were stage 1. Operative time ranged from 64 to 175 minutes. The only complications were two cases of small tumor spillage. Hospital stay was 1 to 4 days. Histology ranged from ganglioneuroma to differentiating NB, with a favorable classification in 4 of 5 cases. None of the tumors were N-Myc amplified. Chemotherapy or radiation therapy was not indicated for any patient. All are alive with no evidence of disease at 14 to 55 months' follow-up. CONCLUSION: Primary gross total resection of mediastinal NB can be achieved safely and effectively by a thoracoscopic approach. In our series, most tumors had favorable histology and biology, and all appear to be potentially treatable by primary thoracoscopic resection alone.

Preformed silos in the management of gastroschisis: new progress with an old idea.

Outcomes for infants with gastroschisis have paralleled advances made in neonatal intensive care and the use of parenteral nutrition. Mortality rates approaching 100% a half century ago are now closer to 2% to 8%. Further reduction in morbidity has been the focus of many studies during the past decade. Efforts have been made to identify factors associated with the development of gastroschisis and to evaluate the mode, timing, and location of delivery on the outcome of these infants. The effect of the mode and timing of definitive surgical repair has received attention by more recent reports of newer approaches, including the use of a preformed silo to provide coverage for the exposed viscera and allow a gradual reduction into the abdominal cavity.

Snowmobile injuries and fatalities in children.

BACKGROUND/PURPOSE: Snowmobiling is a popular form of wintertime recreation but can be associated with significant morbidity and mortality. To better understand snowmobile trauma in children, medical records were reviewed, evaluating the relationships between demographic data, mechanisms, and resultant injuries. In addition, because prior studies of childhood snowmobile fatalities have reviewed only national databases, state and national data were combined to evaluate possible underreporting. METHODS: Medical records were reviewed of children

Excellent outcome in patients with stage I germ cell tumors of the testes: a study of the Children's Cancer Group/Pediatric Oncology Group.

BACKGROUND/PURPOSE: The aim of this study was to correlate outcomes in patients with stage I testicular germ cell tumors with compliance to surgical guidelines and to confirm previous single-institution experiences that show excellent disease-free survival rates when treated with orchiectomy alone. METHODS: Sixty-three patients were entered into this intergroup study (Children's Cancer Group 8881/Pediatric Oncology Group 9048) between 1990 and 1996. Surgical guidelines for orchiectomy included an inguinal approach, early vascular control of the cord structures, and a hemiscrotectomy if a transscrotal violation occurred. Failure of tumor marker normalization or subsequent elevation suggested advanced disease requiring further surgery and chemotherapy. Survival curves were compared using the log-rank test. RESULTS: The median age was 16 months (range, 1 month to 5.6 years). Progression of disease occurred in 11 patients. The 6-year event-free survival (EFS) was 78.5% + 7%. The overall 6-year survival rate was 100%. Adherence to all guidelines was confirmed in only 43 patients (69%). Overall, there was no statistical significance in event-free survival rates between patients who had a correct surgical approach and all patients who had surgical violations; however, 4 patients with transscrotal violations showed a significantly higher rate of disease recurrence. CONCLUSIONS: Patients with stage I germ cell tumors of the testes have excellent survival rates when treated with surgery alone. Despite numerous surgical guideline violations, event-free survival in this multiinstitutional study was similar to previous single-institution reports. All patients with relapse or progression of their disease appear to be cured with further surgical excision and chemotherapy.

Improved outcomes in the treatment of gastroschisis using a preformed silo and delayed repair approach.

BACKGROUND/PURPOSE: The aim of this study was to critically evaluate the clinical outcomes of two different surgical treatment approaches for infants born with gastroschisis. METHODS: The medical records of 65 infants with gastroschisis treated at one institution from 1991 to 2000 were available. Infants in group I (prior to December 1998) underwent attempted early repair of the gastroschisis defect on their first day of life. Infants in group II had delayed repair after the initial placement of a preformed silo. RESULTS: Group I had 39 patients; group II had 26 patients. The two groups were equal with respect to maternal age, gestational age, and birth weight. Complete reduction and fascial closure were accomplished for 32 patients (82%) in group I and 25 patients (96%) in group II (P <.02). Median time on the ventilator was significantly less for group II (P <.0001). Infants in group II had shorter times until first postoperative feeding (P <.01) and full feedings (P <.006). Group II had fewer complications than group I (23% v 56%; P <.01). There appeared to be less necrotizing enterocolitis in group II. The average length of hospital stay was 14 days less for group II. CONCLUSIONS: The use of a preformed silo initially followed by delayed fascial closure in infants with gastroschisis is associated with improved fascial closure rates, fewer ventilator days, more rapid return of bowel function, and fewer complications compared with attempts at initial early repair.

Successful nonoperative management of typhlitis in pediatric oncology patients.

BACKGROUND/PURPOSE: The optimal management for typhlitis in pediatric oncology patients has not always been clear from the medical literature. Trends have varied between operative and nonoperative approaches. The aim of this study was to review the successful nonoperative management of these patients at our institution over the last decade to further clarify management guidelines for this difficult problem. METHODS: Medical records of pediatric hematology and oncology patients up to 21 years of age with typhlitis diagnosed at the DeVos Children's Hospital from 1990 to 2000 were reviewed. RESULTS: Twelve patients were included. Ten patients (83%) with computed tomography (CT) scans suggestive of the diagnosis were treated successfully nonoperatively. Management usually included bowel rest, antibiotics, and supplemental parenteral nutrition. Two patients (17%) in whom CT scans were not obtained underwent surgery for presumed appendicitis and pneumoperitoneum, respectively. Typhlitis was found incidentally. In the latter patient, the pneumoperitoneum resulted from a perforated jejunum caused by graft-versus-host disease. This patient died of septic complications and was the only mortality in the series (8%). CONCLUSIONS: Pediatric cancer patients with typhlitis can be treated carefully nonoperatively with bowel rest, antibiotics, and supplemental nutrition. Usual indications for surgery (ie, perforation, clinical deterioration) still should be used. The early use of CT scanning helps to facilitate the diagnosis and may provide the ability to differentiate typhlitis from other abdominal diseases for which surgery would be indicated.