ABSTRACT
Background: Severe pruritus caused by progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (AGS) is refractory to medical treatment. Surgical interruption of the enterohepatic circulation is considered the mainstay of alleviating distressing symptoms and delaying cirrhosis. Aim and Objectives: This study aims to evaluate the short-term effect of partial external biliary diversion (PEBD) on pruritus, liver disease progression, patient's growth, and quality of life. Material and Methods: This prospective cohort study enrolled children with PFIC and AGS from July 2019 to July 2021, whose guardians consented to the PEBD procedure. A standard surgical approach was performed by a single surgeon. Outcomes were measured subjectively and objectively pre- and post-procedure using the pruritus 5-D itching score, Paediatric Quality of Life Inventory scale (PedsQL), growth parameters, bile acids level, and liver function tests. Patients' follow-up period ranged from 6 to 12 months. Results: Seven patients had PEBD procedure; five with PFIC and two with AGS. A significant improvement was detected in the 5-D itching score (p-value < 0.001), PedsQL (p-value < 0.001), and bile acids level (p-value 0.013). The preexisting growth failure was ameliorated. The downward trend in the bilirubin level was not significant. No influential difference in the other liver function tests occurred. No intra-operative complications encountered. Only one case had a post-operative stoma prolapse which was managed surgically. Conclusion: PEBD procedure could be considered as an effective and safe treatment options for intractable pruritus in patients with PFIC or AGS, providing preserved synthetic liver functions.
ABSTRACT
OBJECTIVE: To investigate the effect of laser bio-modulation irradiation therapy on the scar after surgical correction of unilateral cleft lip. DESIGN: a comparative, open-label study. SETTING: we conducted the study in a university based tertiary hospital that recruited early wound healers of unilateral cleft lip correction. PATIENTS: Eighty patients were divided into two groups: In study's group, patients undergo laser bio-modulation irradiation (n = 60); in the control group, patients were followed-up without intervention (n = 20). INTERVENTION: In the study's group, patients underwent low-power diode Laser with wavelength of 806â nm and power of 100â mw. MAIN OUTCOME: The change in the scar of cleft lip patients, which was assessed by clinical examination and ultrasound. RESULTS: The median pigmentation score was significantly lower in the laser group (median = 1; IQR = 1-2) than the control group (median 2; IQR 1-3), with p-value of <0.001. Likewise, the median height score was significantly lower in the laser group (median = 1; IQR = 1-1) than the control group (median 1.5; IQR 1.5-2), with p-value of 0.001. The median pliability score was significantly lower in the laser group (median = 1; IQR = 1-1) than the control group (median 2.5; IQR 1-3), with p-value of <0.001. Finally, the median vascularity score was significantly lower in the laser group (median = 1; IQR = 1-1) than the control group (median 1.5; IQR 1-2), with p-value of <0.001. CONCLUSION: laser bio-modulation irradiation therapy demonstrates a potential efficacy in managing the hypertrophic scars after surgical repair of unilateral cleft lip.
Subject(s)
Cleft Lip , Lasers, Semiconductor , Humans , Lasers, Semiconductor/therapeutic use , Cleft Lip/radiotherapy , Cleft Lip/surgeryABSTRACT
CONTEXT: Choledochal cyst (CHC) is one of the most common causes of surgical jaundice in infants. In 1955, Farello et al. were the first to introduce the laparoscopic approach for treatment of CHC. AIM OF THE STUDY: Minimally invasive approaches to the management of CHC excision have been done in pre-schoolers and above but have not yet been described in toddlers, let alone infants. Herein, we review the results of 10 consecutive children <1 year managed with laparoscopic CHC excision and hepaticoduodenostomy. METHODS: This retrospective study investigated 10 infants who underwent laparoscopic resection of a CHC with creation of a hepaticoduodenostomy. RESULTS: This study was performed on 10 consecutive patients <1 year. Liver fibrosis was found in 4 patients. We had 7 cases with Type 1 CHCs and 3 cases with Type IV A cysts. Total cyst excision was done in all patients, no cases needed blood transfusion and the mean operative time was 200 min. The mean hospital stay was 6 days. Overall, morbidity occurred in 20% of the cases presenting with bouts of cholangitis that resolved without any intervention, once at 6 months, the other at 1-year post-operative. There were neither anastomotic strictures nor biliary fistula formation; magnetic resonance cholangiopancreatography was done to these two cases revealed no stricture and mortality at 30 and 90 days was nil. CONCLUSION: Laparoscopic hepaticoduodenostomy in CHC in children <1 year is safe, with satisfactory short-term results.