Laparoscopic-assisted pull-through for Hirschsprung's disease,a prospective repeated evaluation of functional outcome

Aim of the study

To evaluate the functional outcome of laparoscopic-assisted endorectal pull-through (LAP) for Hirschsprung's disease (HSCR) over time.

Methods

Thirty-five children with HSCR underwent laparoscopic-assisted pull-through at our institution between 1998 and 2009. The diagnosis was histologically confirmed in all cases. Clinical data was extracted from the case records. A prospective assessment of the functional outcome was performed in 2009 and 2012. Exclusion criteria were a follow-up of less than 6 months after treatment (1 case) and total colonic aganglionosis (1 case). An independent examiner, not involved in the clinical care of the patients, performed interviews using a semi-structured questionnaire. Four patients could not be traced for the first interview. Two cases were lost for the second interview. Altogether twenty-seven patients completed the study. Data from the two interviews were compared. The regional ethical review board approved the study.

Main Results

The median patient age was 4 years old (range 2–16) at the time of the first interview and 7 years old (range 5–19) at the time of the second interview . There were 23 males and 4 females in the study group. The median age at laparoscopic-assisted pull-through was 104 days old (range 29 days–8 years). In the first interview 11 patients reported constipation, 18 patients reported soiling more frequently than once per week when they had loose stools and 16 patients when they had solid stools. Laxatives or irrigations were used by 13 of the patients. In the second interview 4 patients reported constipation, 16 patients reported soiling when they had loose stools and 15 patients reported soiling when they had solid stools. Eight patients used laxatives or irrigations. The decrease in constipation was statistically significant (p = 0,023).

Conclusions

Our study shows a statistically significant reduction of constipation over time. There is a high risk of incontinence after laparoscopic-assisted pull-through, with few signs of short-term improvement.     

Pulmonary arterial hypertension associated with systemic sclerosis in the Czech Republic

Background  

Systemic sclerosis (SSc) is an important cause of pulmonary arterial hypertension (PAH), with an estimated prevalence of 7.85–26.7%.     

Radiological Evaluation of Acetabular Erosion After Antibiotic-Impregnated Polymethylmethacrylate Spacer (Spacer-G)

Different types of hip spacers have been described (hand-made, custom-molded or prefabricated) for treatment of a chronic hip infection. A potential disadvantage of monoblock prefabricated spacer is that it may cause acetabular bone loss. This study assesses the radiological acetabular erosion using an antibiotic-impregnated pre-fabricated polymethylmethacrylate Spacer-G. We retrospectively reviewed the radiographs of thirty five patients who were managed with Spacer-G to treat chronic hip infection. No acetabular erosion were observed in thirty two patients with a mean time from the first to second stage and from the first to the last radiograph of 5.09 and 3.77 months respectively. In three patients the time between the radiographs was more than one year and the second stage was not performed; two developed a protrusion acetabuli whereas the other one a destruction of the acetabular roof. Using a Spacer-G in chronic hip infection treatment for less than one year is not associated with radiological acetabular erosion if the patient is maintained at partial weight bearing.     

Circumoral complications in hereditary sensory and autonomic neuropathy — a case of simple lip reconstruction?

We report a case of a 7-year-old Bangladeshi boy who caused himself oral incontinence by self-mutilation. The patient was known to suffer from hereditary sensory and autonomic neuropathy type V. As definitive management, a full dental clearance was performed along with reconstruction of the lower lip with a good functional and aesthetic outcome. He did not experience any adverse effects from the full dental clearance with regard to feeding, nutrition or development. We discuss the dilemma and challenges raised in the management of this patient and highlight the need for a multi-disciplinary specialist input for what appeared to be a simple case of lip reconstruction for a plastic surgeon.     

Nephrectomy for the failed renal allograft in children: predictors and outcomes

Background

There are no guidelines for the removal of a failed renal allograft, and its impact on subsequent dialysis and retransplantation has not yet been described.

Methods

We performed a 10-year review of allograft failure to study the factors that determined an outcome of transplant nephrectomy and choice of subsequent renal replacement therapy in children with or without nephrectomy.

Results

A total of 34 children developed graft failure over the 10-year study period, of whom 18 (53 %) required transplant nephrectomy. The median graft survival was 1.1 (range 0.2–10.6) versus 7.5 (1.5–15.0) years in the nephrectomy and non-nephrectomy groups, respectively (p?=?0.011). Children with graft failure within 1 year of transplantation were four-fold more likely to require transplant nephrectomy than those with graft failure after 1 year (p?=?0.04). Renal biopsy performed at ≤8 weeks prior to graft loss showed Banff grade II acute rejection in 13 of the 18 children who required subsequent nephrectomy versus three of the 13 children who did not need nephrectomy (p?=?0.01). Inflammation (fever, graft tenderness and raised C-reactive protein (CRP) in the 2 weeks preceding graft failure) was seen in 66 % of nephrectomized children, but not in any in the non-nephrectomy group (p?=?0.0003 for CRP between groups). Banff II rejection, an inflammatory response and the time post-transplantation significantly and independently predicted the outcome of nephrectomy (p?=?0.008, R ²?=?67 %). Human leukocyte antigen (HLA) antibody levels after graft failure were higher in the nephrectomy group (p?=?0.0003), but there was no difference between groups in terms of the presence or class of donor-specific antibodies. Of the children with graft failure, 82 % required dialysis (61 % hemodialysis) and 35 % have to date been successfully retransplanted.

Conclusions

Children with Banff II rejection, an inflammatory response and early graft loss are more likely to require transplant nephrectomy. Nephrectomy may be associated with higher circulating HLA antibody levels.     

The immediate effects of deep breathing exercises on atelectasis and oxygenation after cardiac surgery

Objective—To investigate the effects of deep breathing performed on the second postoperative day after coronary artery bypass graft surgery.

Design—The immediate effects of 30 deep breaths performed without a mechanical device (n?=?21), with a blow bottle device (n?=?20) and with an inspiratory resistance‐positive expiratory pressure mask (n?=?20) were studied. Spiral computed tomography and arterial blood gas analyses were performed immediately before and after the intervention.

Results—Deep breathing caused a significant decrease in atelectatic area from 12.3?±?7.3% to 10.2?±?6.7% (p?<?0.0001) of total lung area 1?cm above the diaphragm and from 3.9?±?3.5% to 3.3?±?3.1% (p?<?0.05) 5?cm above the diaphragm. No difference between the breathing techniques was found. The aerated lung area increased by 5% (p?<?0.001). The PaO ₂ increased by 0.2?kPa (p?<?0.05), while PaCO ₂ was unchanged in the three groups.

Conclusion—A significant decrease of atelectatic area, increase in aerated lung area and a small increase in PaO ₂ were found after performance of 30 deep breaths. No difference between the three breathing techniques was found.