scholarly journals HIRSCHSPRUNG’S DISEASE

2010 ◽  
Vol 17 (02) ◽  
pp. 223-231
Author(s):  
MUHAMMAD ZAFAR IQBAL ◽  
Hammad Azam ◽  
MUHAMMAD JAHANGIR ◽  
Muhammad Tahir ◽  
MUHAMMAD ANWAR ◽  
...  
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Case Series ◽  
Abdominal Distension ◽  
Functional Results ◽  
International Studies ◽  
Stool Incontinence ◽  
Case Series Study ◽  
Duhamel Procedure ◽  
One Year

Introduction: Hirschsprung’s Disease is one of the most common congenital anomalies that Pediatric Surgeons manage. In spite of the various modifications of pull through procedures available, the long term functional results are less than ideal. However, Modified Duhamel Procedure is one which has relatively good functional results and that is the reason we have selected this procedure for Hirschsprung’s disease in Sheikh Zayed Hospital, Rahim Yar Khan. Objectives: To evaluate the outcome of patients after Modified Duhamel Procedure for Hirschsprung’s Disease. Design: This is case series study. Settings: Department of Pediatric Surgery Sheikh Zayed Medical College/ Hospital Rahim Yar Khan. Period: Seventeen cases were studied over a period of two year i.e. from November 2006 to December, 2008. Material and Method: Seventeen (17) cases from both sexes were operated for Hirschsprung’s Disease over a period of one year and Modified Duhamel Procedure was adopted for all these cases. All patients were diagnosed cases of rectosigmoidal aganglionosis and follow up was done over a period of one year according to a comprehensive Proforma. Four parameters like normal stool evacuation, abdominal distension, soiling and stool incontinence were followed and then the results were compared with other national and international studies. Results: Fever 19.4% (n=04), wound infection 19.4% (n=04), vomiting 9.52% (n=02), abdominal distension 4.76% (n=01), and bleeding per rectum 4.76% (n=01), were the immediate post operative complications. Fourteen patients (82.35%), used to pass stool once daily. Abdominal distension was observed occasionally in six patients (28.57%). Soiling was seen in five patients (29.41%). Out of Seventeen, stool incontinence was seen in only three patients (17.64%). Conclusion: Modified Duhamel Procedure with the help of linear cutter stapler device is quite safe, easy and less time consuming. Infact Modified Duhamel is a procedure of choice for Hirschsprung’s Disease. 

scholarly journals Early and long-term complications following transanal pull through Soave technique in infants with Hirschsprung's diseaseric infants with Hirschsprung's disease

2019 ◽  
Author(s):  
Manoochehr Ghorbanpour ◽  
Mohammad Ali Seyfrabie ◽  
Babak Yousefi
Keyword(s):  
Intestinal Obstruction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Statistical Tests ◽  
Case Series ◽  
P Value ◽  
Short Term ◽  
Case Series Study ◽  
The Mean

Objective. Patients undergoing Soave surgery for Hirschsprung's disease are at risk for some complications. The aim of this study was to investigate such short-term and long-term complications and evaluate the outcome of the operation in these patients. Methods. A case series study was carried out during the last 12 years, during 2007 to 2018 in Besat hospital of Hamadan. Data collection conducted using a checklist includes questions about demographic information, clinical features, and short-term and long-term complications, and consequences of post-operative surgery. The findings of the study were analyzed using SPSS software version 20 and appropriate statistical tests. P-value less than 0.05 was considered statistically significant. Results. A total of 55 children underwent Soave surgery during the last 12 years in Besat Hospital Hamadan, Iran. The mean age of the patients was 38±10 days during surgery, of which 56.4% were female. The mean hospital stay was 7.3 days. Also, the mean weight of children at birth was 2970±447 gr. Most of the patients were born as NVD (52.7%) and term (74.5%). The most common comorbidity was congenital heart disease. The most common short-term complication was intestinal obstruction in 14 patients (25.5%) and the most frequent long-term complication was intestinal obstruction and constipation (27.3% each cases). The mortality rate of patients in this study was 14.5% in total. Conclusions. One stage surgical procedure in Hirschsprung's disease is a safe and effective method, but care should be taken in choosing patients and patients should be monitored for possible complications, so that they can be considered and implemented for proper treatment.

scholarly journals Outcomes in Hirschsprung’s disease with coexisting learning disability

2021 ◽  
Author(s):  
Joseph R. Davidson ◽  
Kristiina Kyrklund ◽  
Simon Eaton ◽  
Mikko P. Pakarinen ◽  
David Thompson ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Learning Disability ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Core Outcome Set ◽  
Functional Results ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Related Quality

AbstractThis study describes functional and health-related quality of life (HRQoL) outcomes in patients with Hirschsprung’s disease (HSCR) with associated learning disability or neurodevelopmental delay (LD), completing a core outcome set for HSCR. This was a cross-sectional study from a tertiary pediatric surgery center. Patients treated between 1977 and 2013 were prospectively contacted to complete an outcomes survey. Children under 12 and older patients with LD were assisted to complete these by a proxy. Bowel and urologic function were assessed (Rintala’s BFS and modified DanPSS) along with HRQoL (PedsQL/GIQLI/SF-36). Thirty-two patients with LD were compared to 186 patients with normal cognition. Patients with LD had 76% survival over the follow-up period, compared to 99% in the remainder of the cohort. Poor functional outcomes were common in the patients with LD, considerably higher than cognitively normal patients: with weekly issues withholding stool, soiling and fecal accidents in over half of patients surveyed (44–60%), and urinary incontinence in 46%. Use of permanent stoma was significantly higher (22% vs. 4%; p = 0.001). HRQoL was worse in domains of physical functioning in adults and children but not for social or emotional domains in adults. Subgroup analysis of patients with Down syndrome suggested similar functional results but better QoL. Multivariate analysis demonstrated a dramatically higher incidence of poor continence outcomes in patients with LD (adjusted OR 9.6 [4.0–23]).Conclusions: We provide LD-specific outcomes showing inferior function but similar HRQoL to other patients with HSCR, this is much needed in the counselling of families of these children. What is Known:• Hirschsprung’s disease is commonly associated with syndromes or other anomalies with resultant cognitive impairments.• The outcomes for these patients specifically have been poorly described in the literature. What is New:• Objective functional and quality of life surveys demonstrate significant differences from patients without cognitive impairment.• Patients with learning disability Patients with associated LD were almost ten times more likely to have an associated poor functional outcome, with very little impact on proxy-reported quality of life.

scholarly journals One-year outcomes of fixed-dosing Aflibercept therapy for pre treated and naive polypoidal choroidal vasculopathy patient

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Hun Gu Choo ◽  
Jin Hae Lee ◽  
Hyun Sub Oh ◽  
Soon Hyun Kim ◽  
Yong Sung You ◽  
...  
Keyword(s):  
Polypoidal Choroidal Vasculopathy ◽  
Vision Loss ◽  
Case Series ◽  
Age Related Macular Degeneration ◽  
Research Information ◽  
Age Related ◽  
Case Series Study ◽  
Treatment Naïve ◽  
One Year ◽  
Choroidal Vasculopathy

Abstract Background Polypoidal choroidal vasculopathy (PCV) is a type of age-related macular degeneration that can cause permanent vision loss. The purpose of this paper was to report the one-year outcomes of fixed-dosing aflibercept therapy for the treatment of PCV. Methods This was a prospective, single-arm, interventional case series study of 25 PCV patients; 12 pre-treated and 13 treatment-naïve patients. The patients were treated and monitored for 12 months. Each patient was administered with an aflibercept (2.0 mg) injection every month for the first 3 months (the loading phase), and thereafter, once every 2 months. At every follow-up visit, best-corrected visual acuity (BCVA) test, fundus examination, and optical coherence tomography for measuring the central subfield macular thickness (CSMT) were performed. Fluorescein and indocyanine green angiography were conducted at baseline and at 4 and 12 months. Results After 12 months of aflibercept therapy, the mean BCVA of the patients significantly improved from 65.48 letters at baseline to 69.91 letters (p=0.001), and the CSMT significantly decreased from 406.92 um at baseline to 276.12 um (p< 0.001). Additionally, ten patients (40%) showed complete polyp regression. The treatment-naïve patients showed a statistically significant improvement in BCVA from 66.58 letters at baseline to 76.36 letters at 12 months, and a significant decrease in CSMT, from 462 to 243 um. In the pre-treated group, there was no change in BCVA (64.46 letters), and the decrease in CSMT from 356.08 to 303.69 um was not statistically significant. Conclusions The fixed-dosing aflibercept regimen is effective for treating patients with PCV and is more effective in treatment-naïve patients than in pre-treated patients. Trial registration Clinical Research Information Service (CRiS), Republic of Korea. Identifer: KCT0005798, Registered: Jan 20, 2021. Retrospectively registered, URL: https://cris.nih.go.kr/cris/en/search/search_result_st01.jsp?seq=18546

Long-term outcome and colonic motility after the Duhamel procedure for Hirschsprung's disease

1999 ◽  
Vol 34 (2) ◽  
pp. 325-329 ◽  
Author(s):  
C.T Baillie ◽  
S.E Kenny ◽  
R.J Rintala ◽  
J.M Booth ◽  
D.A Lloyd
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Colonic Motility ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Duhamel Procedure

Hirschsprung’s Disease in Adults: the Duhamel Procedure

2004 ◽  
Vol 104 (3) ◽  
pp. 304-308 ◽  
Author(s):  
J. Vanoorbeek ◽  
M. Kint ◽  
J.-P. Yvergneaux
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Duhamel Procedure

scholarly journals P0258EVALUATION OF ACUTE - PHASE REACTANTS IN MONITORING DISEASE ACTIVITY AND RESPONSE TO IMMUNOSUPPRESSIVE THERAPY IN IDIOPATHIC RETROPERITONEAL FIBROSIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Katarina Obrencevic ◽  
Marijana Petroviä‡ ◽  
Nemanja Rancic ◽  
Jelena Tadiä‡ ◽  
Neven Vavic ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Case Series ◽  
Immunosuppressive Drugs ◽  
Obstructive Nephropathy ◽  
Mass Reduction ◽  
Retrospective Case ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Acute Phase Reactants ◽  
Case Series Study ◽  
One Year

Abstract Background and Aims Idiopathic retroperitoneal fibrosis (IRF) is a rare disease characterized by fibroinflammatory periaortic tissue that affects the ureters, causing obstructive nephropathy and impairment of renal function. Findings strongly suggest an autoimmune etiology. Medical treatment includes immunosuppressive drugs, but the optimal treatment has not been established. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) represent markers of active inflammation in the early phase of the disease, and hence could be sensitive predictors of the treatment response. Method This retrospective case series study includes patients with IRF treated with two different protocols: corticosteroids in combination with mycophenolate mofetil (MMF) and corticosteroids combined with azathioprine (AZA). The total number of 28 patients (13 in the first group and 15 in the second group) were treated and followed in the Clinic of nephrology in Military Medical Academy. Results At presentation 92.3% of patients had elevated levels of ESR and CRP, and 67.8% of them had acute renal insufficiency. Systemic symptoms resolved after four weeks in all patients. The kidney function improved significantly in both groups after first moth of treatment, although the creatinine level was higher in the MMF group because of the disease recurrence in 23% of the patients. In MMF group was observed faster reduction in the periaortic mass after six months and 53.8% of patients achieve 100% of the mass reduction at the end of the treatment while 33.3% in AZA group. The levels of ESR and CRP after one month of treatment in AZA group correlated with the fibrotic mass reduction after six months and after one year. In MMF group, only the CRP level after one month correlated with the fibrotic mass reduction achieved after one year. Conclusion MMF had more rapid and complete effect in hydrnephrosis withdrawal and total reduction of the fibrotic tissue, comparing with AZA whose effect was slower and without complete mass reduction. CRP strongly correlated with the remission of the disease in both therapeutic protocols.

Chiari pelvic osteotomy does affect hip survival: a long-term follow-up study

2020 ◽  
pp. 112070002090183
Author(s):  
Josko Jelicic ◽  
Antea Buterin ◽  
Goran Vrgoc ◽  
Zeljko Butorac ◽  
Anton Tudor ◽  
...  
Keyword(s):  
Case Series ◽  
Hip Osteoarthritis ◽  
Pelvic Osteotomy ◽  
Functional Results ◽  
Retrospective Case ◽  
Case Series Study ◽  
Median Period ◽  
Chiari Pelvic Osteotomy

Background: The modern indications for Chiari pelvic osteotomy (CPO) have narrowed and the number of such operations performed has diminished markedly. So far published long-term experience with CPO indicate the fact that in selected patients satisfactory functional results can be achieved. The aim of this study was to investigate the parameters which influence CPO survival and its conversion into total hip arthroplasty (THA). Methods: This is a single-centre retrospective case-series study. In the period from 1976 to 2012, it included 172 CPO in 158 patients. Of those, in 43 patients (48 hips) the follow-up was discontinued, leaving 115 patients (124 hips) for final analyses. The median age at the time of the surgery was 34 years, and the mean duration of the follow-up was 19 years. Results: In 115 patients (124 CPO) included in the study, a total of 51 THA was performed in 49 patients. The median period between CPO and THA was 14 years. Conclusion: Supporting our results, we suggest that CPO should still be indicated in a selected group of younger patients even with advanced stage of hip osteoarthritis who prefer a joint-conserving procedure and consent to a predicted less optimal outcome.

Outcome of laparoscopic modified Duhamel procedure with Z-shaped anastomosis for Hirschsprung’s disease

2011 ◽  
Vol 26 (5) ◽  
pp. 1325-1331 ◽  
Author(s):  
Naoto Urushihara ◽  
Koji Fukumoto ◽  
Hiroaki Fukuzawa ◽  
Akihide Sugiyama ◽  
Kentaro Watanabe ◽  
...  
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Duhamel Procedure

Use of a new stapling device in the performance of the duhamel procedure for Hirschsprung's disease

1990 ◽  
Vol 25 (2) ◽  
pp. 253-254
Author(s):  
Donald B. Shaul ◽  
Marshall Z. Schwartz ◽  
Clifford C. Marr ◽  
Kenneth R.T. Tyson
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Stapling Device ◽  
Duhamel Procedure

Laparoscopic Duhamel Procedure with Ex-Anal Rectal Transection for Right-Sided Hirschsprung's Disease

2017 ◽  
Vol 27 (9) ◽  
pp. 972-978 ◽  
Author(s):  
Xi Zhang ◽  
Li Yang ◽  
Shao-tao Tang ◽  
Guo-qing Cao ◽  
Shuai Li ◽  
...  
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Rectal Transection ◽  
Duhamel Procedure
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