Henry W. Giles

Cricopharyngeal achalasia responsive to balloon dilation in an infant

This report describes an infant with vomiting and significant weight loss attributable to cricopharyngeal achalasia, a rare finding in children. The infant responded to balloon dilation of the upper esophageal sphincter, with resolution of symptoms and return to presymptomatic growth parameters. A brief description of the clinical features, diagnostic evaluation, and treatment options for cricopharyngeal achalasia is included.

Massive low-grade fibromyxoid sarcoma presenting as acute respiratory distress in a 12-year-old girl

Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue sarcoma that usually presents in young adults as a painless, slow-growing mass. Evans first described LGFMS in 1987 as a spindle-cell sarcoma with bland histological features and paradoxically aggressive behavior. Although young adults are most frequently affected, recent reports describe pediatric cases being increasingly more common. Males and females are affected approximately equally and common locations include the deep soft tissue of the lower extremity, particularly the thigh and trunk. Primary occurrence within the chest cavity is exceedingly rare. Local recurrence and metastasis are not uncommon and present the clinician and radiologist with challenges regarding follow-up recommendations. Review of the literature reveals many cases of slowly progressive symptoms related to a mass effect. We present a healthy 12-year-old African-American girl who interestingly developed acute symptoms of shortness of breath and chest pain while playing with her brother.

Uncommon presentation of rare disorder-duodenal ulcer secondary to invasive pancreatic primitive neuroectodermal tumor: case report and review of the literature.

The authors report an unusual presentation of a rare tumor in childhood, a pancreatic primitive neuroectodermal tumor invading the duodenum leading to anemia. A review of intra-abdominal primitive neuroectodermal tumors is discussed.

Esophageal diverticulum arising from a prolonged retained esophageal foreign body.

Esophageal foreign body impaction (EFBI) is a rare condition in childhood which needs urgent removal. However, if left untreated, its chronic impaction may lead to serious secondary complications. Symptoms associated with chronic EFBI are attributed to airway disease or gastroesophageal reflux, which further delays definitive diagnosis and management. We present a girl with ingestion of a bottle cap diaphragm that was embedded in her esophagus resulting in esophageal narrowing and a secondary diverticulum formation. As the disk was unable to be removed using standard grasping forceps, the authors used an injector needle to impale the disk and successfully removed it without any complications.

Streptococcus agalactiae myositis in a child with perinatally acquired human immnodeficiency virus.

Pyomyositis is a musculoskeletal infection with formation of an intramuscular abscess. Endemic in tropical climates, it is being reported with increasing frequency in temperate climates such as the United States. The most common causative organism is Staphylococcus aureus, present in greater than 90% of reported cases. Risk factors include underlying chronic illness, malnutrition, immunocompromised state, and muscle trauma. We present a case in which Streptococcus agalactae was the causative agent of myositis in a child with a history of perinatally derived human immunodeficiency virus infection.

Pediatric osteosarcoma: a single institution's experience.

Objectives The aim of the study was to evaluate outcomes with an examination of individual predictors influencing survival at a single institution. Methods This was a retrospective review of the 28 pediatric osteosarcoma patients diagnosed and studied from 2000 through 2012. Twenty-eight patient charts and imaging studies were reviewed for age, race, sex, location, extent of disease at presentation, imaging results, histology, treatment options, and overall survival. Results Of the 28 patients who were identified, the median age at diagnosis was 14 years. The majority of the patients were male African Americans with the tumor located in the lower long bones and most had conventional osteosarcoma histology. Four patients had metastasis at diagnosis. Of the 28 patients, 16 patients underwent limb salvage surgery, 6 underwent amputation, 4 had biopsy only, 1 had hip disarticulation, and 1 moved out of state and had no information available. All 28 patients received chemotherapy. Four patients received additional radiation therapy. On follow-up, 15 patients were still alive at last clinical contact and 13 died. Of the deceased, the median survival time was 2.3 years. The patient who lived the longest survived 8.3 years. Metastasis at diagnosis was associated with poorer outcome (P = 0.002). The 5-year overall survival rate was 40% (95% confidence interval 18–62) for our entire population of patients. Conclusions Survival in our patient cohort tended to be at the lower end of the spectrum reported by other contemporary treatment centers of excellence or Surveillance, Epidemiology, and End Results databases probably because of the large number of African American patients with associated poor socioeconomic status. Future studies should be conducted to explore biological and nonbiological factors that may affect the prognosis in this disease.

Synovial sarcoma leading to a paraesophageal abscess in a child

BACKGROUND Synovial sarcoma (SS) is an uncommon malignant neoplasm arising from primitive pluripotential mesenchyme primarily affecting the soft tissues of the extremities. Rarely other locations are involved, including the mediastinum. CASE REPORT After treatment for mediastinal SS by surgical resection, radiation therapy, and chemotherapy, an 11-year-old boy developed an esophageal stricture and fistula, the latter resulting in a paraesophageal abscess. Management of the esophageal stricture and fistula required a multi-disciplinary approach. We report our experience with the management of this difficult complication, as well as a brief review of the literature on SS. CONCLUSIONS Rare conditions, particularly those with unusual complications, present therapeutic challenges requiring a multi-disciplinary team approach. Reporting experiences with difficult cases can benefit providers faced with similar problems in the future.

Arterial tortuosity syndrome: An extremely rare disease presenting as a mimic of pulmonary sling

Pulmonary sling is the anatomic variant defined by the aberrant origin of the left pulmonary artery from the right pulmonary artery. This patient presented with a mimic of pulmonary sling as a result of an extremely rare condition, arterial tortuosity syndrome (ATS). The patient was first diagnosed with pulmonary sling on prenatal echocardiogram performed by cardiology. Computed tomography angiography of the chest obtained at birth to evaluate respiratory depression demonstrated ATS. The early detection of ATS has been demonstrated to improve patient outcome. This case provides an overview of the typical imaging features of ATS to aid radiologists in making this uncommon diagnosis.

Giant hepatocellular adenoma in a child

An 8-year-old female presented with fever, shortness of breath, bdominal pain, and abdominal distention of 2 days duration. xamination was unrevealing except for a large abdominal mass. bnormal laboratory findings included haemoglobin (8.4 g/dl), ematocrit (25.4%), ALT (92 U/L), AST (99 U/L), and total bilirubin 4.5 mg/dl). Computed tomography (coronal view) of the abdomen evealed a large irregular contoured rounded hepatic mass, with reas of mixed density consistent with haemorrhage (Fig. 1). Biopsy f the mass showed well-differentiated hepatocytes without assoiated bile ducts consistent with hepatic adenoma (Fig. 2). The child

Sternoclavicular Joint Arthritis as the Initial Presentation of Crohn’s Disease in an Adolescent

Crohn’s disease (CD) is protean in its presentation, manifesting with symptoms and signs referable to the gastrointestinal tract and/or secondary to extraintestinal involvement. It is estimated that up to 35% of individuals with inflammatory bowel disease (IBD) experience extraintestinal manifestations, which typically involve the musculoskeletal system, skin, eyes, and hepatobiliary system. Extraintestinal manifestations may predate intestinal symptoms and signs, causing a delay in the diagnosis of CD. We report an adolescent with sternoclavicular joint (SCJ) arthritis as her initial presentation of CD, 6 months before the onset of gastrointestinal symptoms. We present this case to highlight the importance of recognizing that extraintestinal manifestations may be the presenting symptom in IBD.