Rectal prolapse following posterior sagittal anorectoplasty for anorectal malformations

Purpose

Rectal prolapse is a known postoperative problem in children with anorectal malformations. The aims of this study were to determine the incidence of significant rectal prolapse (>5 mm), to objectively quantify its predisposing factors, and to offer recommendations as to its prevention and surgical treatment.

Methods

The authors reviewed their series of 1619 patients with anorectal malformations; 1169 underwent primary posterior sagittal anorectoplasty (PSARP) at their institution between 1980 and 2002, and complete records were available for 833. The series was analyzed for incidence of prolapse, type of anorectal malformation, status of the sacrum, muscle quality, associated vertebral and spinal anomalies, and postoperative constipation. A specific technique for prolapse repair was used.

Results

Of 833 patients, 45 developed significant rectal prolapse (3.8%). The mean age at the time of PSARP was 0.73 years (range, 0.19-5 years). The average time to recognition of prolapse following PSARP was 13.1 months. Of these 45 patients, 32 required surgical repair and of those, 3 required a second surgical repair. The incidence of prolapse varied by complexity of anorectal defect: cloaca (6.2%), rectobladder neck fistula (6.8%), rectourethral fistula (5.4%), rectovestibular fistula (1.2%), rectal atresia (0%), and rectoperineal fistula (0%). There was a significantly increased incidence of prolapse in patients with a low muscle quality score and in patients with vertebral anomalies (20% vs 3.2%). The presence of a tethered cord and an abnormal sacral ratio did not correlate with an increased incidence of prolapse. Twenty-two patients developed prolapse following colostomy closure, and of these, 12 (55%) suffered from constipation.

Conclusions

The overall incidence of significant rectal prolapse following PSARP is low. Prevention of prolapse with the PSARP technique may be because of key technical steps. Patients with higher anorectal malformations, poorer muscle quality, and vertebral anomalies had a greater risk of developing postoperative rectal prolapse. The presence of tethered cord and quality of the sacrum were not predictive of postoperative prolapse. Constipation seems to be a factor in the development of prolapse.     

A Minimally Invasive Solution for Necrotic Fundus following Slipped Adjustable Gastric Band

The laparoscopic adjustable gastric band (LAGB) is widely used for treatment of morbid obesity. Band slippage is a well known long-term complication. Herein, we present a patient with band slippage who developed strangulation of the fundus with irreversible necrosis. The chosen surgical solution was minimally invasive. A diagnostic laparoscopy and consequently resection of the necrotic fundus was performed. A postoperative symptomatic left pleural effusion resolved after chest tube insertion. One week later, the patient was discharged with satisfactory results.     

Can external signs of trauma guide management?: Lessons learned from suicide bombing attacks in Israel

BACKGROUND: Following a suicide bombing attack, scores of victims suffering from a combination of blast injury, penetrating injury, and burns are brought to local hospitals. OBJECTIVE: To identify external signs of trauma that would assist medical crews in recognizing blast lung injury (BLI) and effectively triaging salvageable and nonsalvageable victims. DESIGN: Retrospective analysis of all 15 suicide bombing attacks that occurred in Israel from April 1994 to August 1997. SETTING: National survey. PATIENTS: One hundred fifty-three victims died and 798 were injured as a result of 15 attacks. Medical records were reviewed for external signs of trauma, such as burns and penetrating injuries, and the presence of BLI.Main Outcome Measure The odds ratio for BLI and death. RESULTS: Three settings were targeted: buses, semiconfined spaces, and open spaces. Sixty survivors (7.5%) suffered from BLI, which was more common in buses (37 of 260) than semiconfined spaces (14 of 279) and open spaces (9 of 259) (P<.001). Victims with BLI were more likely to suffer from penetrating injury to the head or torso, burns covering more than 10% of the body surface area, and skull fractures (odds ratios, 4, 11.6, and 55.8, respectively; P<.001). Victims who died at the scene were more likely to suffer from burns, open fractures, and amputations in comparison with survivors (odds ratios, 6.5, 18.6, and 50.1, respectively; P<.001). CONCLUSIONS: Following a suicide bombing attack, external signs of trauma should be used to triage victims to the appropriate level of care both at the scene and in the hospital. Triage of salvageable and nonsalvageable victims should take into account the presence of amputations, burns, and open fractures.     

Unusual presentation of inferior thyroid artery aneurysm

A rare case is reported of an aneurysm of the inferior thyroid artery with hoarseness as the only presenting symptom. The aneurysm was excised. Six years after the operation the patient is well and free of symptoms.     

Functional and genomic approaches reveal an ancient CHEK2 allele associated with breast cancer in the Ashkenazi Jewish population

Functional and genomic approaches can be integrated to screen efficiently for pathogenic alleles in founder populations. We applied such approaches to analysis of the cancer-associated cell cycle regulator CHEK2 in the Ashkenazi Jewish population. We first identified two extended haplotypes at CHEK2 that co-segregated with breast cancer in high-risk families. We sequenced CHEK2 in a case representing each haplotype and discovered two novel amino acid substitutions, CHEK2.S428F in the kinase domain and CHEK2.P85L in the N-terminal region. To assay these alleles for loss of CHEK2 function, we tested their capacity to complement Rad53 deletion in Saccharomyces cerevisiae. CHEK2.S428F failed to complement Rad53 and thus largely abrogates normal CHEK2 function, whereas CHEK2.P85L complemented Rad53 as well as did wild-type CHEK2. Epidemiologic analyses were concordant with the functional tests. Frequencies of CHEK2.S428F heterozygotes were 2.88% (47/1632) among female breast cancer patients not selected for family history or age at diagnosis and 1.37% (23/1673) among controls (OR=2.13, 95% CI [1.26, 3.69], P=0.004), whereas frequencies of CHEK2.P85L were 0.92% among cases and 0.83% among controls. On the basis of the experience of mothers, sisters and daughters of probands, breast cancer risk due to CHEK2.S428F was estimated as 0.17 (+/-0.08) by age 60. We conclude that CHEK2.S428F increases breast cancer risk approximately 2-fold among Ashkenazi Jewish women, whereas CHEK2.P85L is a neutral allele. In general, these results suggest that selecting probands with extended haplotypes that co-segregate with disease can improve the efficiency of resequencing efforts and that quantitative complementation tests in yeast can be used to evaluate variants in genes with highly conserved function.     

Poly-lactic-glycolic acid microspheres: a biodegradable marker for the diagnosis of aspiration in hamsters

Aspiration is a major cause of lung disease in infants and young children. As the symptoms and signs of aspiration are not specific, the diagnosis is delayed due to a low index of suspicion and low sensitivity and specificity of the available diagnostic tests. In the present study, we evaluated the utility of microspheres composed of a degradable polymer, polylactic glycolic acid (PLGA), as a marker to diagnose aspiration in hamsters. Thirty hamsters underwent direct tracheal instillation of 0.1 mL of a suspension of PLGA. Eighteen other animals served as controls and underwent tracheal instillation of 0.1 mL of saline. Three animals served as naive controls and had no tracheal instillation. Five animals from the PLGA group and three from the saline group underwent whole-lung lavage (WLL) on days 1, 8, 15, 29, 43, and 58. PLGA microspheres were easily identified under light microscopy inside the alveolar macrophages obtained from WLL in all PLGA-instilled animals during all studied days. The number and size of PLGA microspheres within the alveolar macrophages decreased gradually with time with a 90% rate of disappearance of about 36 d. There was a marked neutrophilic response in lung lavage and a mild peribronchial neutrophil infiltration on the first day after tracheal instillation of PLGA which subsequently disappeared. We conclude that PLGA microspheres are a sensitive and specific marker for aspiration in hamsters. The usefulness of this test in diagnosing aspiration in humans should be further evaluated in clinical studies.     

Endocrine profile of children with intrauterine growth retardation

BACKGROUND: Intrauterine growth retardation (IUGR) is a major cause of short stature in childhood. Most but not all children experience catch-up growth by 2 years of age. METHODS: We investigated the endocrine profile (thyroid function, prolactin, cortisol, C-peptide and insulin-like growth factor-I [IGF-IJ levels) of 57 children with IUGR, aged 2-10 years, and compared it with 30 controls whose birth weight was appropriate-for-gestational-age. RESULTS: The hormonal profile for both groups was similar for thyroid hormones, prolactin, C-peptide and IGF-I. Cortisol levels were significantly lower in the IUGR group compared to controls (p <0,05). When the IUGR group was divided into 'catch-up' growth and 'non-catch-up' subgroups, the latter had significantly lower IGF-I levels (p <0.001). CONCLUSIONS: Lower cortisol levels in children born with IUGR may reflect impaired function of the hypothalamic-pituitary-adrenal axis associated with this condition. The significantly lower IGF-I levels of the 'non-catch-up' subgroup may be involved in their failure to grow.     

CT of hemangiomas of the upper airways in children

OBJECTIVE: Hemangiomas of the airways in infants are commonly diagnosed at bronchoscopy performed for the investigation of stridor or other respiratory symptoms. Occasionally on bronchoscopy the appearances are atypical or the entire extent of the suspected hemangioma cannot be appreciated. We report on the clinical usefulness of dynamic contrast-enhanced CT in the evaluation of suspected hemangiomas of the airways in infants. CT findings of 11 infants who underwent investigation for a suspected airway hemangioma were correlated with bronchoscopic findings. CONCLUSION: Dynamic contrast-enhanced CT is a valuable noninvasive method for the evaluation of airway hemangiomas. Although it can be used to confirm the diagnosis in patients with equivocal findings on bronchoscopy, we believe that CT findings are specific enough that CT can be recommended as the primary method of establishing the diagnosis. Multiplanar reconstructions illustrate the location, extent, and degree of luminal narrowing and any involvement of adjacent tissues.     

Proteases, extracellular matrix, and cancer: a workshop of the path B study section

The role of the extracellular matrix (ECM) in the tumor microenvironment is not limited to being a barrier against tumor invasion. The ECM is a reservoir of cell binding proteins and growth factors that affect tumor cell behavior. It is also substantially modified by proteases produced by tumor cells or stroma cells. As a result of the activity of these proteases, cell-cell and cell-ECM interactions are altered, new biologically active ECM molecules are generated, and the bioavailability and activity of many growth factors, growth factor receptors, and cytokines are modified. ECM-degrading proteases also play a critical role in angiogenesis, where they can act as positive as well as negative regulators of endothelial cell proliferation and vascular morphogenesis. This review article summarizes some of the most relevant findings made over the recent years that were discussed at a workshop organized by the Path B Study Section of the National Institutes of Health in October 2002.