Biomedical subjects

Aliza P Cohen

Publications and source records attributed to Aliza P Cohen.

8 recordsLinked to original sources

PubMed2006

Deficient tracheal rings.

Deficient cartilaginous rings are an extremely rare intrinsic tracheal defect. To our knowledge, only two cases have previously been reported in the literature. We describe our experience with three patients presenting with this unusual defect causing severe tracheomalacia who were successfully managed with slide-tracheoplasty. With respect to correction of the stenotic tracheal segment, our clinical outcomes were excellent. Dissimilarities in medical history suggest that the origin of deficient tracheal rings can be either congenital or acquired.

Child, Preschool↗

PubMed2006

Esophageal strictures in children with recessive dystrophic epidermolysis bullosa: an 11-year experience with fluoroscopically guided balloon dilatation.

BACKGROUND: Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited blistering skin disorder that is associated with significant esophageal strictures, resulting in dysphagia and nutritional failure. Although endoscopically guided balloon dilatation is a widely used treatment, the use of an endoscope carries the risk of oropharyngeal trauma. To minimize this risk, we have eliminated its use. METHOD: We reviewed the charts of all RDEB patients who underwent balloon dilatation for esophageal strictures between August 1993 and March 2005. Balloon dilatation procedures were performed under anesthesia and with fluoroscopic control. RESULTS: We performed 92 dilatations on 25 RDEB patients. Most patients reported immediate relief of symptoms, rapid recovery, and resumption of adequate food intake within 1 day. The mean interval between dilatations was 1 year. Six patients (24%) have required only 1 dilatation, and 1 of these 6 has had a dilatation-free interval of 25 months. One patient with a history of multiple dilatations has remained dilatation-free for 5 years. No procedure-related complications have occurred. CONCLUSIONS: Fluoroscopically guided balloon dilatation is a gentle, safe, effective, and repeatable technique that should be considered as a first line of treatment.

Adolescent↗

PubMed2006

Nonoperative management of pectus carinatum.

BACKGROUND: Although surgery has been the mainstay of treatment of chondrogladiolar pectus carinatum (PC), several authors have advocated the benefits of nonoperative approaches to induce chest wall remodeling. Based on our initial success with compression bracing, we have integrated this modality into our treatment algorithm. METHOD: We reviewed the charts of all patients treated for PC at our pediatric hospital between 1997 and 2004. Patients were managed with observation, operative repair, and orthotic bracing that provides continuous anteroposterior sternal compression. The brace was worn for 14 to 16 hours per day until linear growth was complete or for a minimum of 2 years. RESULTS: One hundred patients were diagnosed with PC. Fifty-seven patients had no treatment and were monitored. Twenty-nine patients were fitted with a brace. Of these 29 patients, 3 were noncompliant, resulting in a compliance rate of 90%. Of the remaining brace patients, all have had positive outcomes with no observed complications. Seventeen patients underwent surgical repair. Their outcomes were also positive with no major complications. CONCLUSION: Our findings clearly demonstrate that compression bracing is a safe and effective treatment for children with chondrogladiolar PC. We currently offer this approach as a first-line treatment, reserving surgery for patients who are noncompliant and those who fail the nonoperative modality.

Adolescent↗

PubMed2006

Lymphatic malformations of the tongue base.

PURPOSE: In light of the paucity of literature on lymphatic malformations of the tongue base, our aim was to present our experience and long-term outcomes of patients with this rare and challenging pathologic entity. METHODS: Medical records of 25 patients treated by the 3 senior authors (RGA, MTC, and RTC) between 1974 and 2003 were retrospectively reviewed, and comprehensive clinical data were collected and analyzed. RESULTS: Twenty-one patients (13 female and 12 male infants) were diagnosed either prenatally or at birth. Of these patients, 18 required early airway stabilization; 17 required tracheotomy. Four patients were diagnosed after 1 year of age and had no airway problems. Follow-up ranged from 2 days (owing to death) to 28 years, with a mean of 10 years. In 21 patients, pathology was extensive, involving contiguous anatomical areas such as the anterior tongue, larynx, pharynx, and floor of mouth. Multiple resections and debulking procedures were performed to restore function and improve cosmesis. Four patients died, all with laryngeal involvement. Of the 14 survivors who had tracheotomies, only 5 are decannulated. Normal oral feeding has been achieved in 14 patients and normal speech, in 8 patients. Cosmesis has improved with time. Orthodontic and dental problems are common, and 9 patients have significant macrognathia. CONCLUSIONS: Although most patients with lymphatic malformations of the tongue base achieve normal oral feeding, airway, speech, and cosmesis issues remain problematic throughout life. Laryngeal involvement signifies extensive disease and is the most significant risk factor for serious complications and death.

Airway Obstruction↗

PubMed2005

Managing bariatric patients in a children's hospital: radiologic considerations and limitations.

As the increasing prevalence of pediatric obesity and its associated risks gain national attention, adolescent bariatric surgery is gradually gaining acceptance as a viable weight loss tool for some severely obese adolescents. Obese patients often exceed the capacity limits of most radiological devices used for imaging. Because the radiological evaluation often plays a crucial role in the management of these patients, it is important for pediatric surgeons and radiologists to be aware of the special challenges and alternative algorithms for radiological evaluation. This review outlines and illustrates these considerations and limitations. Herein the authors discuss these topics within the context of characteristic bariatric patient profiles and the Roux-en-Y gastric bypass procedure, currently the authors' standard operative approach.

Adolescent↗

PubMed2004

Association of anterior glottic webs with velocardiofacial syndrome (chromosome 22q11.2 deletion).

OBJECTIVE: An association between anterior glottic webs and velocardiofacial syndrome (chromosome 22q11.2 deletion) has previously been noted in a number of case reports. Our objective was to determine if the presence of such webs warrants a high index of suspicion for this chromosome deletion. Study design and setting This study was carried out in the Division of Pediatric Otolaryngology-Head and Neck Surgery at Cincinnati Children's Hospital Medical Center. Chromosome 22q11.2 deletion status was determined for all patients endoscopically diagnosed with anterior glottic webs between July 1998 and December 2000. Families of patients who tested positive for the deletion were referred to the Cincinnati Children's Division of Human Genetics for additional evaluation and counseling. RESULTS: Eleven of 17 patients (65%) with anterior glottic webs were positive for chromosome 22q11.2 deletion. Of these 11 patients, 5 showed subtle clinical manifestations of velocardiofacial syndrome and underwent genetic testing due only to the presence of a web. All 11 patients were diagnosed with velocardiofacial syndrome. CONCLUSION: We strongly recommend that all patients diagnosed with anterior glottic webs undergo fluorescence in situ hybridization evaluation for this chromosome deletion.

Child↗

PubMed2004

Complications of metallic stents in the pediatric airway.

OBJECTIVE: Our aim was to present our experience with complications caused by placement of metallic stents in the pediatric airway. DESIGN AND SETTING: We conducted a retrospective study of the medical records of patients with complications resulting from metallic stent placement, managed by the senior authors between 1993 and 2002. RESULTS: Nine children had complications associated with the placement of metallic airway stents. Of these, 8 children required stent removal. Granulation tissue and tracheal stenosis were seen in all 7 children with long standing stent placement. There was 1 stent death in this series. CONCLUSIONS: Metallic airway stents can cause significant complications in the pediatric airway. These complications may supersede the airway compromise that necessitated their initial placement. As such, metallic stent placement should be approached with caution. The likelihood and severity of complications increase with time, as do the difficulties encountered upon removal. The proportion of patients in whom metallic stents may be placed "permanently" without complications is not known. Therefore we recommend that metallic airway stents be considered a temporizing measure of limited duration.

Child, Preschool↗

PubMed2004

A diagnostic paradigm for childhood idiopathic sensorineural hearing loss.

OBJECTIVE: Our objective was to determine the diagnostic yield of laboratory testing, radiological imaging, and GJB2 mutation screening in a large cohort of patients with differing severities of idiopathic sensorineural hearing loss (SNHL). DESIGN AND SETTING: We undertook a retrospective study of patients presenting with SNHL at our institution from 1993 to 2002. RESULTS: Laboratory testing had an extremely low yield. Patients with unilateral SNHL had a significantly higher imaging yield than those with bilateral. The diagnostic yield of GJB2 screening was significantly higher in patients with severe to profound SNHL than in those with less severe SNHL. However, a relatively large number of patients with mild to moderate SNHL had positive GJB2 screens. CONCLUSIONS: Based on diagnostic yields, we propose a cost-effective stepwise diagnostic paradigm to replace the more commonly used and costly simultaneous testing approach. EBM RATING: C.

Adolescent↗