scholarly journals Treatment and Patient Reported Outcome in Children with Hirschsprung Disease and Concomitant Congenital Heart Disease

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Johan Hasserius ◽  
Josefine Hedbys ◽  
Christina Graneli ◽  
Kristine Hagelsteen ◽  
Pernilla Stenström

Purpose. Congenital heart disease (CHD) is reported to be associated with Hirschsprung disease (HD). The aim was to evaluate any differences between children with HD with and without CHD, respectively, with regard to patient characteristics, medical care, and patient reported bowel function. Method. This is a retrospective chart study and a cross-sectional long-term follow-up of patients older than 4 years old, including all children with HD operated on with transanal endorectal pull-through (TERPT) at a tertiary center of pediatric surgery. Information about patient characteristics, diagnostics, surgery, and medical care was compiled. At long-term follow-up, bowel function was assessed by Bowel Function Score. Results. Included were 53 HD-patients, 13 with CHD and 40 without CHD. Children with CHD more commonly presented with failure to thrive; 4 (23%) compared to those without CHD (0%) (p<0.01). In the long-term follow-up, including 32 patients (6 with CHD), constipation was more commonly reported by children with CHD 5 (83%) than by children without CHD 4 (27%) (p=0.01). No differences were shown in the other parameters such as fecal control and incontinence. Conclusion. HD-patients with CHD more commonly presented with failure to thrive and more frequently reported constipation than HD-patients without CHD. The findings indicate that HD-patients with CHD might need special consideration in their initial care and long-term follow-up.

2018 ◽  
Vol 4 (6) ◽  
pp. 771-780 ◽  
Author(s):  
Ivo Roca-Luque ◽  
Nuria Rivas-Gándara ◽  
Laura Dos Subirà ◽  
Jaume Francisco Pascual ◽  
Antònia Pijuan-Domenech ◽  
...  

2019 ◽  
Vol 14 (4) ◽  
pp. 525-533 ◽  
Author(s):  
Madalena Coutinho Cruz ◽  
André Viveiros Monteiro ◽  
Guilherme Portugal ◽  
Sérgio Laranjo ◽  
Ana Lousinha ◽  
...  

2019 ◽  
Vol 7 (7_suppl5) ◽  
pp. 2325967119S0037
Author(s):  
Erin Fynan Ransom ◽  
Heather Minton ◽  
Bradley Young ◽  
Brent Ponce ◽  
Gerald McGwin ◽  
...  

Objectives: Thoracic outlet syndrome represents a complex combination of symptoms in the upper extremity that occurs due to compression of the neurovascular structures of the thoracic outlet or subcoracoid space. It can be seen in overhead athletes and is commonly misdiagnosed as other shoulder pathology. This study seeks to highlight patient characteristics, intraoperative findings, and both short term and long term outcomes of thoracic outlet decompression in the adolescent population as well as a comparison of outcomes by mechanism of injury including athletes. Methods: A retrospective chart review was performed of patients between the ages of 13 and 21 with a clinical diagnosis of TOS that were treated surgically by a single surgeon between 2000 and 2015. Data points including preoperative patient characteristics and intraoperative findings were collected. In addition, patient reported outcome scores including quickDASH, CBSQ, VAS, and SANE were obtained for a cohort of patients with long-term follow up ranging from 2 to 15 years. Results: The study population consisted of 54 adolescents (61 extremities) aged 13-21 at the time of presentation. There was a 3:1 female to male ratio. Patients saw an average of 2.08 other healthcare providers before referral to our institution. The most common surgical procedures included neurolysis of the brachial plexus (60; 98.4%), anterior scalenectomy (59; 96.7%), middle scalenectomy (54; 88.5%), excision of the first rib (28; 45.9%), excision of cervical rib (5; 8.2%), and subclavian artery manipulation (50, 81.9%). A second incision in the subcoracoid space was utilized in 28 (45.9%) extremities for exploration of the infraclavicular brachial plexus with release of the coracocostal ligament in 26 of these 28 patients (92.9%). Long-term follow-up data, including patient reported outcomes, was collected for 24/54 patients (44%). In this patient subset, the average follow-up was 69.5 months and ranged from 24 months to 180 months. The average preoperative VAS was recorded at 8.2 with an average postoperative VAS of 2.0, showing an improvement of 6.2 points. The average preoperative SANE score was 28.9 and the average postoperative SANE score was 85.4 with an improvement in 56.5. The average postoperative quickDASH score was 11.4. The average postoperative CBSQ score was 27.4 . The average postoperative NTOS Index score was 17.2. Subgroup analysis was performed analyzing patients having a first rib excision versus patients where their first rib was left intact. There was no difference regarding clinical outcome measures in these groups including CBSQ, VAS, SANE score, quick DASH and NTOS index. An additional subgroup analysis was performed comparing mechanism of injury including overuse from sports, trauma, and idiopathic causes. There was also no difference regarding clinical outcome measures in these groups including CBSQ, VAS, SANE score, quick DASH and NTOS index. Conclusion: We found no difference in clinical outcome scores in patients treated with rib resection versus patients with the first rib left intact. We also found no difference in outcomes with respect to mechanism of injury including overhead sports athletes. Overall, patients did well long-term after decompression of the thoracic outlet. Thoracic outlet syndrome should be considered in the differential diagnosis of athletes with upper extremity pain especially if they have neurologic findings.


Heart ◽  
2014 ◽  
Vol 100 (Suppl 3) ◽  
pp. A3.2-A4 ◽  
Author(s):  
Sandhya Santharam ◽  
Maria Theodosiou ◽  
Sara Thorne ◽  
Paul Clift ◽  
Lucy Hudsmith ◽  
...  

PEDIATRICS ◽  
1982 ◽  
Vol 70 (4) ◽  
pp. 638-642
Author(s):  
Åke Lundberg

A follow-up study of 49 individuals with paroxysmal atrial tachycardia in infancy is reported. Forty subjects had supraventricular tachycardia and nine had atrial flutter/fibrillation. The mean follow-up period was 24 years. Males whose ECGs showed preexcitation displayed the highest rate of recurrences (approximately 60%) in the long-term follow-up. Approximately 30% of the subjects without preexcitation in the ECG displayed episodes of supraventricular tachycardia in their third decade. Eighty-six percent of the children of the subjects in the present series were males. The prognosis is good in the absence of congenital heart disease.


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