Pediatric short bowel syndrome: adaptation after massive small bowel resection.

BACKGROUND: Short bowel syndrome (SBS) develops after massive small bowel resections. Patients with less than 12 cm of jejunoileum have a slim possibility of being weaned from parenteral nutrition (PN). PATIENTS AND METHODS: In a retrospective review of records of consecutive patients with SBS, 8 patients were evaluated for treatment by adaptation and weaning from PN. These included 4 patients with class I SBS (0-10 cm of small bowel), one with class II SBS (>10-25 cm), one with class III SBS (>25-50 cm), and 2 with class IV SBS (>50-75 cm). Adaptation was assessed by measuring growth in the small bowel and the ability to be weaned from PN. RESULTS: Adaptation was achieved primarily by extending the length of jejunoileum by approximately 450% over the first 2.5 years after resection and by increasing the degree of colonic fermentation and absorption of nutrients. As of July 1, 2005, all of the patients were off PN, with the exception of 2 patients with class I-A SBS: patient 3 had a remaining jejunoileum of only 2.5 cm and patient 4 had a remaining jejunoileum of 9 cm but developed eosinophilic enterocolitis. These 2 patients continued with PN on alternate months. CONCLUSIONS: Bowel growth after massive small bowel resection provides an objective parameter of adaptation and a means of predicting ability to be weaned from PN. Aggressive nutritional support makes even patients with class I SBS, whose disease was previously considered hopeless, likely candidates to achieve freedom from PN.

Colonic neuroendocrine carcinoma in a child.

A 10-year-old boy with congenital immunodeficiency (X-linked agammaglobulinaemia) presented with loss of appetite and weight, right-sided abdominal pain, diarrhoea and low-grade fever. Radiological investigations with barium follow-through, CT, PET and octreotide scans revealed a primary caecal/ascending proximal colonic mass with liver and bony metastases. Urine screen for 5HIAA was positive. Percutaneous liver biopsy confirmed the diagnosis of neuroendocrine carcinoma. The radiological work-up and the usefulness of various imaging modalities in the diagnosis of this rare paediatric tumour are discussed. The PET scan demonstrated the primary tumour and the metastatic locations more vividly than the octreotide scan, which is currently considered to be the most specific imaging modality for neuroendocrine masses.

Primary alveolar capillary dysplasia (acinar dysplasia) and surfactant protein B deficiency: a clinical, radiological and pathological study.

BACKGROUND: Full-term infants with severe and prolonged respiratory distress represent a diagnostic challenge. Plain radiographic findings may be nonspecific or similar to classic surfactant deficiency disease for infants with surfactant protein B deficiency and acinar dysplasia. OBJECTIVES: To describe the similar clinical-radiolgical patterns of two rare neonatal conditions. MATERIALS AND METHODS: Six newborn babies with severe respiratory distress at birth demonstrated clinical and radiographically prolonged and progressive diffuse pulmonary opacification. RESULTS: All infants demonstrated hyperinflation of the lungs. The diffuse hazy opacification, which varied from mild (n=3) to moderate (n=3), progressed to severe diffuse opacification preceding death, which occurred at 12-36 days of life. Open lung biopsy confirmed the diagnosis of primary alveolar acinar dysplasia (AD) in four infants and surfactant protein B deficiency (SPBD) in two infants. CONCLUSIONS: In full-term babies with unexplained progressive respiratory distress from birth and progress of radiological changes, both AD and SPBD should be considered.

Radiological findings in NAO syndrome.

BACKGROUND: Diseases exhibiting osteolysis in children are rare hereditary conditions. Several types have been recognised with different clinical manifestations. One type includes subcutaneous nodules, arthropathy and osteolysis and has been termed NAO syndrome. Previous radiological reports have described the affected bones, usually the carpal and tarsal regions, but a detailed analysis of the radiological findings of both the axial as well as the appendicular skeleton has not been reported. OBJECTIVES: To describe the radiological findings in a large group of children with an autosomal recessive disease characterized by nodules, familial arthropathy and osteolysis. MATERIALS AND METHODS: The study comprises 14 patients from 9 families and all patients had the triad of nodulosis, arthropathy and osteolysis (NAO). RESULTS: The most common radiological manifestations were osteopenia, undertubulation of long bones, arthritic changes, sclerotic sutures of the calvaria, osteolysis and muscle contractures. Other common findings were squared vertebrae, broad medial clavicles and brachycephaly. Progress of disease was documented in more than half of the patients. CONCLUSIONS: Our study is the first report of the detailed radiological findings of NAO syndrome. In NAO syndrome, both the axial and appendicular skeleton are involved