Israel Medical Association Journal

Background: Rectal intussusception, rectocele and rectal prolapse are anatomic disorders in obstructed defecation syndrome. A relatively new surgical approach, Stapled Transanal Rectal Resection, was designed to treat these anomalies.

Objectives: To present our preliminary results with this technique.

Methods: Thirty patients with ODS[1] not responding to medical treatment or biofeedback were operated on with the STARR[2] technique. All the patients underwent a complete workup in the Pelvic Floor Unit. The operation was performed according to the technique described elsewhere.

Results: The patients' mean age was 67.1 years, and the median duration of symptoms was 7 years. The mean operating time was 40 minutes (range 35–80 min) and the mean hospital stay was 2 days (range 1–4 days). The mean follow-up was 26 months (range 6–48 months). ODS symptoms were ameliorated in 27 patients (90%), decreased significantly in 18, and in 9 patients the symptoms disappeared. The procedure failed in 3 patients (10%). Complications included minor bleeding that required homeostasis in eight patients during the operation. Three patients had transient tenesmus and five patients had anal pain. There were no cases of mortality or pelvic sepsis.

Conclusions: STARR is an effective and safe procedure for the treatment of obstructed defecation syndrome due to rectal intussusception, rectocele and small rectal prolapse.

Background: Down syndrome is one of the most common chromosomal abnormalities. Children and adults with DS[1] have significant medical problems and require life-long medical follow-up.

Objectives: To determine the adequacy of medical surveillance of individuals with DS as recommended by the American Academy of Pediatrics.

Methods: The study was conducted at a multidisciplinary center specializing in the care of DS during the period 2004–2006. At their first visit to the Center, caregivers of individuals with DS were questioned about the medical status of their child including previous evaluations. Medical records brought in by the parents were reviewed.

Results: The caregivers of 150 individuals with DS (age ranging from newborn to 48 years old, median age 5 years) were interviewed and medical records were reviewed. The prevalence of specific medical problems differed between our population and the reported prevalence from other surveys. For example, 39.3% of our population had documented auditory deficits while the reported prevalence is 75%. For gastrointestinal and thyroid disease, the prevalence was higher in the studied population than that reported in the literature. In terms of compliance with the AAP[2] recommendations, most children (94%) underwent echocardiography, but only 42.7% and 63.3% had been tested for auditory or visual acuity respectively. Only 36.3% over the age of 3 years had cervical spine films.
Discussion: Many individuals with DS are not receiving appropriate medical follow-up and the implications of inadequate surveillance can be serious

Background: Lung disease in patients with famHiat dys-autonomia is caused mainly by recurrent aspiration of gastric contents, food and liquids swallowed incorrectly.

Objective: To describe chest computed tomography findings in patients with familial dyautonomia.

Methods: A retrospective analysis of chest CT findings was performed for 34 FD patients (15 females, 19 males) with a mean age of 18± 12.8 years.

Results: The CT revealed bronchial wall thickening (in 94% of the patients), atelectasis (in 73%), ground glass opacities (in 53%), focal hyperinflation (in 44%), fibrosis (in 29%) and bronchiectasis (in 26%). The extrapulmonary abnormalities were scoliosis (79%) and esophageal dilatation (35%). Silent fractures were noted in two vertebral bodies and one rib.

Conclusions: Pulmonary changes were consistent with chronic inflammation in the bronchi and interstitial tissues. Ground  glass opacities and fibrosis support the theory that these changes could be due to gastric aspiration. Bronchiectasis is less frequent. Esophageal dilatation with fluid overflow adds to aspiration. Fractures can be asymptomaflc and are often missed.