Comparison of 68Ga-DOTATATE PET/CT and 123I-MIBG SPECT/CT in the Imaging of Functional Pheochromocytoma in an Adolescent Patient With Von Hippel-Lindau Syndrome.

ABSTRACT: A 17-year-old boy with Von Hippel-Lindau syndrome presented with hypertension, raised plasma catecholamines, and MRI findings of a new pancreatic tail lesion and 2 stable right adrenal lesions concerning for functional neuroendocrine tumors. A 68Ga-DOTATATE PET/CT demonstrated intense tracer avidity within the pancreatic lesion with minimal uptake in the adrenal lesions. Conversely, a 123I-MIBG SPECT/CT study demonstrated high-grade tracer uptake within the adrenal lesions, with no significant uptake appreciated in the pancreatic lesion. The adrenal lesions were resected, and pathology was consistent with pheochromocytoma. Plasma catecholamines returned to within the normal range and hypertension resolved.

Conservative Treatment of an Unusual Presentation of Iliopsoas Phlegmon Related to Infected Intrauterine Contraceptive Device.

Iliopsoas phlegmon/abscess is uncommon, and individuals often present with nonspecific symptoms. Diagnosis is often delayed and almost always requires advanced imaging techniques such as computed tomography or magnetic resonance imaging. We report a case of a 51-year-old woman who presented with right lower limb swelling and associated rash with imaging demonstrating iliopsoas abscess secondary to an infected intrauterine contraceptive device. This rare case highlights the nonspecific presentation of iliopsoas abscess and the need to consider unusual sources of infection such as an intrauterine contraceptive device in women presenting with iliopsoas phlegmon and abscess.

Does preoperative imaging for scalp non-melanocytic skin cancer accurately predict invasion of the cranial vault? A systematic review and meta-analysis.

PURPOSE: This study aimed to determine the diagnostic accuracy of CT and MRI in the preoperative detection of bone involvement for non-melanoma skin cancers (NMSCs) located on the scalp. This study further aimed to evaluate the predictive value of these imaging modalities in determining the need for craniectomy and to identify gaps in the existing literature. METHODS: Electronic searches of the MEDLINE, Embase, Cochrane and Google Scholar databases were performed for English language studies of any type. Studies reporting detection or exclusion of histopathologically confirmed bone involvement through preoperative imaging were identified according to PRISMA guidelines. Studies reporting dural involvement, non-scalp tumours, and lacking tumour type(s) or outcome data were excluded. Outcomes were preoperative imaging result and histopathologically confirmed bone invasion. Meta-analysis was performed and sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated (excluding case report and MRI data due to insufficient quality and quantity respectively). RESULTS: Four studies with a total of 69 patients were included in the final review, of which two studies totalling 66 patients were included in the meta-analysis. Preoperative CT had a sensitivity of 38%, specificity of 98%, PPV of 90% and NPV of 73%. CONCLUSIONS: The available data suggests that a preoperative CT finding of calvarial involvement by a scalp NMSC is likely to be real, but the absence of such a finding is unreliable. Current evidence suggests that preoperative imaging cannot exclude the necessity for craniectomy and future research is needed, particularly on the role of MRI.

Thyroid nodules in children and adolescents: Investigation and management.

Clinically detectable thyroid nodules are less common in children than adults. However, they are associated with an increased risk of malignancy. Therefore, thorough evaluation of paediatric thyroid nodules is necessary, and an understanding of the features associated with a higher risk of malignancy is important to guide management and referral. Thyroid cancer in children differs significantly from that seen in adults in terms of genetics, presentation, response to treatment and prognosis. Children often present with more advanced disease, but the vast majority have excellent long-term prognosis. Evaluation and management of thyroid nodules and thyroid cancer require a multidisciplinary team approach and involvement of specialists with experience in this field. This review summarises investigative pathways for thyroid nodules in children and outlines current management strategies for paediatric thyroid nodules and cancer.

Balanced Chest Drainage Prevents Post-Pneumonectomy Pulmonary Oedema.

BACKGROUND: Pneumonectomy in the adult patient is associated with a mortality of 1-9%. Death is often due to post pneumonectomy pulmonary oedema (PPPO). The use of balanced chest drainage system (BCD) in the setting of post pneumonectomy has been reported to be of benefit in the prevention of PPPO. This study seeks to compare the incidence of PPPO in patients who underwent pneumonectomy and whose empty pleural space was managed either with CRD or BCD. METHODS: This retrospective observational cohort study involved 98 patients who were operated on by one surgeon at Liverpool Hospital, Sydney, Australia from 1997 to 2019. The patients were divided into two groups according to the era in which they had their pneumonectomy. Group 1 consisted of 18 patients managed with clamp-release drainage between 1997 and 2002. Group 2 consisted of 80 patients managed with balanced chest drainage between 2003 and 2019. The primary outcomes of interest were the development of PPPO and death. Demographic and clinico-pathological variables between the groups were compared including whether the phrenic nerve was sacrificed, volume of infused intraoperative fluid, duration of single lung ventilation, intraoperative tidal volumes, agents of anaesthetic induction and maintenance, mean urine output in the first 4 postoperative hours, institution of a postoperative 1.5 L fluid restriction, total chest drainage, day of chest drain removal, presence of radiological postoperative mediastinal shift, post-pneumonectomy pulmonary oedema and death. Group characteristics were compared using t-test and chi-squared for continuous and categorical variables respectively. Univariate and multivariate analysis was also undertaken using the Firth method of logistic regression for rare occurrences in a stepwise fashion. RESULTS: Through univariate analysis, balanced chest drainage, postoperative fluid restriction and intraoperative fluid infusion showed significant effect on PPPO. Through multivariate analysis, balanced chest drainage was found to have independent protective value for PPPO and mortality. CONCLUSION: Compared with clamp-release drainage, balanced chest drainage results in a lower incidence of post-pneumonectomy pulmonary oedema and death.

Colon Cancer in Patients Under 25 Years Old: A Different Disease?

BACKGROUND: The aim of this study was to compare the stage-for-stage overall (OS) and recurrence-free (RFS) survival between adult and pediatric/adolescent colon cancer patients. STUDY DESIGN: A retrospective review of pediatric/adolescent patients less than 25 years old, treated between 1991 and 2017 at University of Texas MD Anderson Cancer Center, was compared with a prospectively maintained database of adult patients. Outcomes variables were compared, and OS and RFS were estimated using the Kaplan-Meier method and compared between groups using the log rank test and multivariable Cox models. RESULTS: The cohort contained 94 pediatric patients and 765 adult patients. Overall, the 3-year OS rates for adult and pediatric patients, respectively, were 90% and 41.92% (95% CI 87% to 92%) (p < 0.0001), and the 3-year RFS rates were 78% and 32% (p < 0.0001). The stage-for-stage 5-year OS rates for adult vs pediatric patients were: Stage 1: 96% vs 100% (p = 0.793); stage 2: 90% vs 64% (p < 0.0001); stage 3: 85% vs 58% (p < 0.0001); stage 4; 55% vs 16% (p < 0.0001). The stage-for-stage 5-year RFS rates for adults vs children were: stage 1: 95% vs 100%; stage 2: 85% vs 55% (p = 0.0002); stage 3: 73% vs 31% (p < 0.0001); stage 4: 27% vs 5% (p < 0.0001). Pediatric/adolescent patients had a higher risk of recurrence or death than adult patients on multivariate analysis (hazard ratio [HR] 2.312, 95% CI: 1.615 to 3.313 (p < 0.0001). Peritoneal metastasis was significantly higher in pediatric patients. (p = 0.00001) CONCLUSIONS: Stage-for-stage, pediatric/adolescent patients had shorter 3- and 5-year OS and RFS rates than adult patients. Peritoneal disease and carcinomatosis were significantly higher in pediatric, adolescent, and young adult patients less than 25 years old. Predisposing conditions, such as polyposis or congenital colon disease, did not contribute to this difference.

An update in the management of pediatric sarcoma.

PURPOSE OF REVIEW: Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) is a rare subgroup of malignancy in childhood that is composed of a variety of soft tissue and bony tumors. Prognosis for resectable localized disease is usually good and improved with systemic treatment. However, survival from locally advanced and metastatic disease remains poor. There have been numerous preclinical and clinical studies to define histopathology, biology, and genetic alteration of sarcomas. The purpose of this review is to clarify the progress in the management of NRSTS. RECENT FINDINGS: Genomic analysis, including the use of next-generation sequencing, has revealed fusion transcripts or specific genetic alterations which provide diagnostic biomarkers and potential targets for novel therapies. SUMMARY: Most cases are sporadic, but some are associated with genetic predispositions. Most present as a painless mass and diagnosis is frequently delayed because of a low index of suspicion. There is a wide array of histopathological subtypes. Investigations usually involve core, incisional or excisional biopsy for tissue diagnosis, and cross-sectional and nuclear imaging for staging. Management of pediatric sarcoma is largely dependent on the patient's histopathological diagnosis, age, disease stage, and co-morbidities but usually involves a combination of systemic and local therapies. Preclinical studies and phase I/II trials of newer targeted therapies are ongoing.

Chest wall reconstruction after tumor resection.

Pediatric chest wall tumors are rare. Malignancies predominate of which sarcomas are the most common. Their resection and the subsequent reconstruction of the chest wall has been a surgical challenge since Dr. Frederick W. Parham published his first comprehensive account on the subject in 1898. Chest wall reconstruction is age, site and pathology dependent, must preserve long term function and cosmesis, must accommodate future growth and development, and must not be a hindrance to adjuvant radiotherapy. Bony reconstruction can be relatively simple or complex involving combinations of synthetic meshes, bioprosthetic materials, steel or titanium constructs, autografts, homografts and porcine or bovine xenografts. Soft tissue coverage can be achieved with direct closure, skin grafts, local advancement flaps, pedicled or free myocutaneous or osseomyocutaneous flaps or a combination of these. Complications to be avoided include scoliosis, pain and activity restriction, restrictive pulmonary deficits and interference with adjuvant radiotherapy which may result in tumor recurrence. Advances in cancer therapy have improved short- and long-term survival but significant functional and cosmetic challenges remain particularly for large chest wall defects in the very young. The future may lie with absorbable semi-rigid meshes, biointegratable acellular homografts and xenografts, demineralized bone matrices and bone marrow stromal cells, the patient's own lab-grown stem-cell based vascularized osseomyocutaneous chest wall grafts or the obsolescence of surgical resection altogether in the age of targeted anti-tumor and immune based therapy.

Histiocytic sarcoma in a child-successful management and long-term survival with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

Histiocytic sarcoma is an extremely rare tumor in children. It may occur sporadically or in association with other hematological malignancies. It arises most commonly in the lymph nodes but may occur anywhere in the body and clinical presentation is usually with advanced disease. Following tissue diagnosis and staging, management is with chemotherapy though there are no standard regimes. Surgery has been used successfully for local control. This is the first description of the use of peritonectomy and hyperthermic intraperitoneal chemotherapy to treat histiocytic sarcoma. The 4-year-old patient has been disease free for 6 years.

Pediatric "off-road vehicle" trauma: determinants of injury severity and type.

OBJECTIVES: This study aimed to describe the determinants of the severity and type of injuries sustained by children hurt in off-road vehicle (ORV) accidents. METHODS: This was a retrospective clinical study for which data were obtained from the trauma database at the Children's Hospital at Westmead covering the 10-year period between January 1, 1998, and December 31, 2007. Data points collected included age, sex, Injury Severity Score (ISS), body region injured, type of vehicle, accident setting, mechanism of injury, estimated speed, position of the rider, use of a helmet and/or protective clothing, and hospital length of stay. The study end points were determinants of injury severity and type. Statistical analysis of the collected data was done with the standard statistical software package, SPSS. RESULTS: A total of 288 children (242 male [84%] and 46 female [16%] patients) presented for ORV-related trauma. Helmets significantly diminished the chance of sustaining a head injury occasioning a skull fracture. Jumping was associated with increased ISS and a higher chance of sustaining an abdominal and/or thoracic injury. Older children were more likely to sustain pelvic and spinal injures, be injured while traveling at high speed, and be injured while going over a jump. Mean ISS was significantly lower if trauma was sustained while riding a mini motorcyle in any setting and any ORV at home. CONCLUSIONS: Further research (prospective, federal, and multi-institutional) is needed with a view to optimizing training schedules, rules, regulations, and licensing requirements for pediatric ORV riders.

Trampoline trauma in children: is it preventable?

OBJECTIVES: Trampoline injuries represent a preventable cause of injury in children. This study identified the characteristics of children injured while using trampolines who presented to a pediatric trauma center in Sydney, Australia. METHODS: The Pediatric Trauma Database at our institution was reviewed to identify children with trampoline-related injuries between January 1999 and June 2008. Data collected included age, sex, Injury Severity Score, anatomical region injured, type of injury, mechanism of injury, site of injury and surface fallen onto, level of supervision, treatment, and hospital length of stay. RESULTS: Over the 9.5-year review period, 383 children presented with trampoline-related injuries: 193 (50.4%) were female. Just over a quarter (n = 106, 27.7%) were treated and discharged the same day. The remaining patients accounted for 725 hospital bed days with a mean length of stay of 2.3 days. The most common area of the body injured was the upper limb (n = 246, 64.2%), with a fall from the trampoline to the ground being the most frequent mechanism of injury (n = 257, 67.1%). The majority (n = 345, 90.1%) of children were injured in their home or at the home of a friend or relative. Surgery was required in 236 (61.6%), with closed reduction of an upper limb fracture being the most common procedure (n = 107, 27.9%). CONCLUSIONS: Trampoline-related injuries remain common in children. Implementation of current guidelines and the introduction of innovative trampoline designs should reduce the risk of this injury in children.

Body wall thickness in adults and children--relevance to penetrating trauma.

AIM: Although uncommon in children, penetrating trauma may be associated with considerable risk of morbidity and mortality. In part this reflects the child's thinner body wall. No data exists on the difference in body wall thickness in children as compared to adults. METHOD: Computer tomography (CT) scans (23 adults and 17 children) selected at random were reviewed. Measurements of the thickness of the skin and subcutaneous tissue were taken from anatomically defined regions on the thoracic and abdominal wall. The differences between adults and children were compared and analysed statistically. RESULTS: The mean ages were 51.3 years for an adult and 8.9 years for a child. Indications for the CT scans included malignancy, inflammatory conditions and blunt trauma. Statistically significant differences in the median thicknesses of adult and paediatric thoraco-abdominal wall were found in all of the regions measured. This difference ranged from 7 mm at the xiphisternum to 14 mm in the right and left axillary lines at the level of the iliac crests. CONCLUSIONS: The demonstrated difference in thickness of the thoraco-abdominal wall of an adult as compared to a child means that for a penetrating injury of equal depth, the body wall will be more easily breached and more organs potentially damaged in a child, particularly if the injury is sustained in the flanks. This supports the principle that all penetrating thoraco-abdominal wounds in children should be explored in the operating theatre under general anaesthesia to reduce the risk of a missed injury.

Paediatric vascular birthmarks--the psychological impact and the role of the GP.

BACKGROUND: This study was designed to investigate the psychosocial impact on the family of a child with a vascular birthmark and examine the role of the general practitioner in meeting the family's needs. METHOD: Nineteen families were interviewed with a questionnaire before their assessment at the Sydney Children's Hospital (New South Wales) Vascular Birthmarks Clinic. RESULTS: Sixty-eight percent of parents overestimated the size of their child's lesion when asked to draw it; 15% said some lifestyle modifications had to be made to accommodate their child's condition; 63% were concerned about their child being teased at school; and 36% had issues of self blame and embarrassment. Expectations from the clinic included information (68%), treatment (47%) and reassurance (26%). CONCLUSION: Vascular birthmarks are common. Treatment strategies are improving, but there is a need to adequately address the psychosocial impact that these lesions have, both on parents and children. The GP is the carer best placed to meet these needs.

Pediatric high-flow priapism and super-selective angiography--an Australian perspective.

High-flow priapism is an uncommon entity in the pediatric and adolescent population. It is usually caused by perineal trauma. Here we describe the experience of our institution in this condition over the past 10 years, the various treatment options available, and the successful application of super-selective angiographic embolization as our treatment modality of choice. Included here is the case of a 4-year-old boy (case 3) who, to our knowledge, is the youngest patient described with this condition in the literature.

Appendicitis and low-flow priapism in children.

Priapism is a sustained erection that is maintained for over 4 hours in the absence of sexual stimulation [Postgrad Med J. 2006;82(964):89-94; J Urol. 2003;170:1318-1324]. Distinction is made between low- and high-flow variants [J Urol. 2003;170:1318-1324; Cardiovasc Intervent Radiol. 2002;25(4):326-329]. Low-flow priapism (LFP) and acute appendicitis are rarely associated. Including ours, there are 4 cases reported in the literature, all of which have occurred in children. The complications of LFP are potentially serious and include loss of the phallus altogether. Treatment of LFP is should be prompt and is the same irrespective of the etiology.

Long standing balanitis xerotica obliterans resulting in renal impairment in a child.

Balanitis xerotica obliterans (BXO) is the most common cause of pathological phimosis in boys. Presented here is the case of a previously well 13-year-old boy who developed obstructive renal impairment (serum creatinine = 190 micromol/l) at least in part from phimosis due to BXO. A circumcision and, 2.5 months later, meatal dilatation were done. Nine months after his initial presentation, his serum creatinine returned to a permanently elevated nadir of 119 mumol/l. Presentation with the complications of phimosis can be delayed in teenage boys because they may feel embarrassed to come forward. Circumcision remains the definitive treatment of BXO induced phimosis though if the penile meatus is involved, more complex surgery is sometimes required. Topical steroids are useful for residual disease. Follow-up is very important due to the frequent involvement of the skin of the glans. In the very long term there is an increased chance of penile malignancy, which can occur even after circumcision.