The Diagnostic Pathway of Hirschsprung's Disease in Paediatric Patients: A Single-Centre Experience.

BACKGROUND: The presenting symptoms of patients with Hirschsprung's disease (HD) are a failure to pass meconium, abdominal distension, and bilious vomiting. The gold standard diagnosis is a rectal biopsy to confirm aganglionosis. The aim of our study was to describe the diagnostic pathway of Hirschsprung's disease at our institution and document the indication for a rectal biopsy. METHODS: We have performed a prospective collection of all patients who underwent a rectal biopsy to exclude HD from December 2022 until September 2023 including. The following data were collected: patient's age, presenting symptoms, type of biopsy, failure rate, complications, and histopathological results. RESULTS: We identified 33 patients who underwent 34 rectal biopsies at 0.6 years of age. A total of 17 patients had a rectal suction biopsy (RSB), and 17 patients underwent a partial thickness under general anaesthesia (GA). 1/17 (6%) patients had an inconclusive RSB and subsequently underwent a biopsy under GA. Constipation and chronic abdominal distension plus vomiting were the most common presenting symptoms throughout all ages. Five patients (15%) had a rectal biopsy that was positive for HD. CONCLUSION: A protocolised approach to the assessment of infants and children with suspected HD ensures the appropriate utilisation of invasive procedures such as biopsy.

Thoracoscopic vs open repair of congenital diaphragmatic hernia after extracorporeal membrane oxygenation: a comparison of intra-operative data.

PURPOSE: ECMO is an escalation treatment for hypoxic respiratory failure in patients with CDH. Open repair has been advocated after ECMO indicating that physiological changes associated to thoracoscopic repair were not well tolerated. METHODS: We have performed a retrospective review of all patients who underwent ECMO prior CDH repair over a 7 year period (2015-2021). Outcome measures were intra-operative Ph, PCO2, PO2 and FiO2 at 30 min, 1 h 30 min, and 2 h 30 min of surgery, operative time and recurrence rate. Data are shown in median (range). RESULTS: Eleven patients required ECMO prior CDH repair. Six of eleven (55%) were done thoracoscopically (Group A) and five of eleven (45%) via laparotomy (Group B). Two of six (33%) patients (Group A) were converted to a laparotomy, one of six (16%) patient developed a recurrence, and there was no recurrence in Group B. Two of five (40%) patients died within the first 60 days of life, whilst there was no death in Group A. Intra-operative values are shown below. CONCLUSION: Whilst this is a preliminary report of a limited number of patients, there is no obvious difference of intra-operative blood gas parameters during surgical repair in patients after ECMO. Thoracoscopic CDH repair may be considered in patients after ECMO.

Definition of efficiency in vacuum therapy--a randomised controlled trial comparing with V.A.C. Therapy.

Redon drains are still used to suction wounds for vacuum sealing. Vacuum-assisted closure (V.A.C.((R)); Kinetic Concepts Inc, San Antonio, TX) is a computer-controlled therapy system for delivering topical negative pressure therapy. The efficiency of V.A.C. in the treatment of pressure ulcers was prospectively studied in a randomised controlled trial in which patients with pressure ulcers were randomly assigned to negative pressure wound therapy (NPWT) using either V.A.C. or Redon bottles. The target parameters were absolute and relative proportion of wound area consists of granulation tissue, fibrin and necrosis. Other outcome measures were the number of dressing changes and time invested using each system. The study was terminated after a post hoc analysis after inclusion of ten patients because of the significantly better results when using V.A.C., and the substantially larger care effort needed in the Redon group compared with the V.A.C. group. An increase in surface granulation tissue of 54% was observed in the V.A.C. group, and a reduction in the Redon group (P = 0.001). The Redon group showed an increase in fibrin tissue at the wound base of 21.8%, whereas in the V.A.C group, a 27% reduction was observed (P = 0.035). Necrosis was reduced in the V.A.C. group, but this difference did not reach significance. Redon bottles are not a good alternative for V.A.C. therapy for delivering NPWT.