John B. Campbell

Neonatal Small Left Colon Syndrome: Its Relationship to Aganglionosis and Meconium Plug Syndrome

Neonatal small left colon syndrome is a cause of functional colon obstruction in newborn infants, with a high association of maternal diabetes. There has been much confusion regarding its relationship to meconium plug syndrome and aganglionosis. Six cases of aganglionosis (2 in infants of diabetic mothers) were encountered in which the radiographic findings were identical to small left colon syndrome, indicating the need for caution in dismissing aganglionosis solely on the basis of a small left colon seen radiographically in a newborn infant with colon obstruction. Small left colon and meconium plug syndrome are overlapping entities in the total spectrum of functional intestinal obstruction in newborn infants.

Malignancies in pediatric patients with ataxia telangiectasia

Background. Patients with ataxia telangiectasia (AT), known to have an inherent increased susceptibility to the development of cancer, may present with malignancies that are unusual for the patients age, are often difficult to diagnose clinically and radiographically and respond poorly to conventional therapy. Materials and methods. We reviewed the clinical presentation and imaging studies of 12 AT patients who developed malignancies. Results. Eight of the twelve patients developed non-Hodgkins lymphoma (CNS, thorax, bone), two developed Hodgkins disease, and two were diagnosed with gastrointestinal mucinous adenocarcinoma. Conclusion. The lymphomas were commonly extra nodal, and infiltrative rather than mass-like. The recognition of the tumors was often delayed due to confusion with the known infectious complications in AT patients.

Upper extremity and rib stress fractures in a child

Abstract Stress fractures in children are rare compared with the incidence in adults. This report describes an 11-year-old girl with stress fractures of the acromion, clavicle, and first rib on the left and contralateral fractures of the first and second ribs. It was eventually discovered that these fractures were caused by a nervous tic consisting of repetitive, vigorous shrugging and translation of the shoulders.

Pulmonary Varix in Early Infancy: Case Report with 8-year Follow Up

The second case of a pulmonary varix documented in the neonatal period is reported. It is important to be aware that this entity may be encountered in the pediatric age group when considering the differential diagnosis of a solitary density in the lung of an infant or young child.

Hirschsprung's disease presenting as calcified fecaloma

Fecaloma, a discrete mass of intestinal debris and fecal material usually occurring in the colon, is a rare finding in children. The case of a six-year-old boy is presented who developed signs of partial colonic obstruction which was due to a large, calcified fecaloma of the rectosigmoid. Barium enema revealed changes indicating Hirschsprungs disease which were confirmed with rectal biopsy. Any child presenting with fecaloma should be evaluated for Hirschsprungs disease.

Jansen type of spondylometaphyseal dysplasia

Abstract Metaphyseal dysplasia, type Jansen (JMD), is a rare skeletal dysplasia with characteristic radiographic abnormalities. Of the various types of metaphyseal dysplasia, JMD shows the most severe alteration in metaphyseal architecture. All of the long tubular bones, including those of the hands and feet, show metaphyseal irregularity with a fragmented appearance and slight widening. The adjacent physes are abnormally widened, while the epiphyses tend to be slightly enlarged, rounded but otherwise normal. The spine in infancy and childhood usually appears normal. This report describes a young girl with metaphyseal changes typical of JMD except for the hands and feet, which appeared normal. She also showed very unusual abnormalities of the spine. This appears, therefore, to represent a unique osteochondrodysplasia for which we propose the term spondylometaphyseal dysplasia, type Jansen.

Fibrogenesis imperfecta ossium: imaging correlation in three new patients

Abstract Objective. Fibrogenesis imperfecta ossium is an extremely rare disorder that can easily be misdiagnosed. We retrospectively reviewed the clinical and imaging data of three confirmed cases of fibrogenesis imperfecta. Design and patients. The patients consisted of two men and one woman, ranging in age from 40 to 53 years. Radiography was performed in all the patients. One patient had a 3-year follow-up of the thoracolumbar spine with conventional radiography and thoracolumbar magnetic resonance (MR) imaging. Open biopsy was performed in all cases, confirming the diagnosis of fibrogenesis imperfecta ossium. Results. All our cases demonstrated ”fishnet” trabecular pattern by conventional radiographs, and a pelvis radiograph of one patient showed an equivocal sclerosis pattern. Multiple fractures were noted in two patients. A pseudoexostosis was present in the ilium in one patient. Thoracolumbar MR imaging demonstrated diffuse low signal intensity within the medullary space on both T1-weighed and T2-weighted images, except for a region of increased signal intensity in the L1 and L2 vertebral bodies on T2-weighed images due to edema from acute collapse. Conclusions. Although uncommon, fibrogenesis imperfecta ossium should be considered in a previously healthy patient with a combination of progressive bone pain, unexplained fractures, a radiologic pattern of fishnet osteopenia and MR imaging of low signal intensity bone marrow on both T1-weighted and T2-weighted images.

Cushing proximal symphalangism and the NOG and GDF5 genes

Proximal symphalangism (SYM1) is an autosomal-dominant developmental disorder of joint fusion. This disorder is best known from famous historical descriptions of two large kindred: Cushing’s description in 1916 of the “straight-fingered” Brown family of Virginia and Drinkwater’s description in 1917 of the British Talbot family of noble blood, descended from the English war hero John Talbot, the first Earl of Shrewsbury (1388–1453). Recent genetic studies link this phenotype to expression of abnormal genes at future joint sites: too little expression of NOG, a growth antagonist, or overexpression of GDF5, a growth agonist, results in cartilage overgrowth and bony fusion. This review unites in depth the first historical accounts of SYM1 with a clinical description and reviews the current understanding of the molecular mechanism underlying what is likely the oldest dominant trait ever studied.

R. Parker Allen, MD (March 13, 1919–October 1, 2010).

Dr. R. Parker Allen was a true pioneer in the field of pediatric radiology, working with the likes of John Caffey and using his skills and knowledge to contribute greatly to the development and teaching of a generation of radiologists. R. Parker Allen was born March 13, 1919, in Red Bank, NJ. He attended Rutgers University as an undergraduate and Columbia University School of Medicine. He entered the Army during WWII and received training in military radiology at Ft. Logan, CO. He remained in Denver as a radiology resident at the Veteran’s Administration Hospital and, in 1949, became an associate of Dr. Frank Stephenson of the Presbyterian group and helped cover Denver Children’s Hospital. In January 1951 he became chief of radiology, the first full-time radiologist at Denver Children’s Hospital. Dr. Allen was one of the founding members of the Society for Pediatric Radiology and served as its sixth president, from 1963 to 1964. He was a charter member of the Caffey Society, a small group of pediatric radiologists who worked with Dr. John Caffey and met yearly to show cases and discuss this emerging field. Dr. Caffey spent a year at Denver Children’s Hospital in 1960. Parker Allen’s knowledge and skills were legendary in Colorado and the entire referral area. Dr. Allen said, “I think in my 23 years there, it was much more important, in terms of training a great number of general radiologists and a few pediatric radiologists.”Dr. Allen continued as chief of radiology at the Children’s Hospital until 1971, when he was succeeded by Dr. William S. Davis and in 1976 by Dr. John B. Campbell. In the 1950s, Dr. Allen helped develop pediatric angiocardiography, working with the Fairchild aerial camera, the predecessor of the cine angiography equipment capable of providing rapid-sequence radiographs of injected contrast material passing through the heart. From 1958 to 1975, he was an important and innovative contributor to the literature. In 1956, he proposed using “a magnet on a string or catheter... to remove magnetic foreign bodies from the stomach, duodenum or jejunum” (Pediatrics 48:563). His writings covered many topics, including lymphangioma of bone (with Charles E. Shopfner, 1960), congenital generalized fibromatosis (with Virgil R. Condon, 1960), transitory extraperitoneal hernia of the bladder in infants (bladder ears) (with Virgil R. Condon, 1961), and T. L. Slovis (*) Pediatric Imaging, Children’s Hospital of Michigan, 3901 Beaubien Blvd., Detroit, MI 48201, USA e-mail: tslovis@med.wayne.edu