Evaluation of Clinical Outcomes in Children with Intrahepatic Cholestasis Postpartial External Biliary Diversion: A Single-Center Experience.

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.

Evaluating the Effect of Low Power Diode Laser 806†nm on the Healing of Unilateral Cleft Lip Scar: An Open-Label Comparative Study.

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 806 nm and power of 100 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.

Correlation between Common Channel Length and Urethral Length in Cloacal Malformations.

INTRODUCTION: This research aimed to study the relationship between common channel length and urethral length in cloaca patients by different diagnostic modalities and the ability of common channel length to predict the urethral length. MATERIALS AND METHODS: The study was conducted on 30 cases of cloaca managed at Alexandria University Children Hospital and Cairo University Children Hospital from August 2018 to December 2019. Preoperative assessment included cystovaginoscopy and magnetic resonance imaging (MRI) studies with a recording of common channel length and urethral length by each modality. RESULTS: There is substantial similarity between MRI and cystoscopy in measuring common channel length; 11 patients had common channel length 1 to 3 cm by cystoscopy. It is the same number by MRI measurement. Nineteen patients had common channel length 3 to 5 cm by cystoscopy and MRI. In total, 16 had urethral length <1.5 cm by MRI, while 14 patients with urethral length <1.5 cm by cystoscopy. However, there is a moderate negative correlation by MRI between common channel length and urethral length, while by cystovaginoscope, there is no significant correlation between common channel length and urethral length. CONCLUSION: Applying MRI or cystoscopy in the preoperative assessment of cloaca cases is equivalent to measuring common channel length, not measuring urethral length. Besides that, the common channel length cannot predict the urethral length.

Laparoscopic hepaticoduodenostomy for choledochal cysts in children <1 year.

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.

Outcome of thoracoscopic repair of type-C esophageal atresia: a single-center experience from North Africa.

Thoracoscopic repair of esophageal atresia is gaining popularity worldwide attributable to availability and advances in minimally invasive instruments. In this report, we presented our experience with thoracoscopic esophageal atresia/tracheoesophageal fistula (EA/TEF) repair in our tertiary care institute. A prospective study on short-gap type-C EA/TEF was conducted at Cairo University Specialized Pediatric Hospital between April 2016 and 2018. Excluded were cases with birth weight < 1500 gm, inability to stabilize physiologic parameters, or major cardiac anomalies. The technique was standardized in all cases and was carried out by operating team concerned with minimally invasive surgery at our facility. Primary outcome evaluated was successful primary anastomosis. Secondary outcomes included operative time, conversion rate, anastomotic leakage, recurrent fistula, postoperative stricture, and time till discharge. Over the inclusion period of this study, 136 cases of EA/TEF were admitted at our surgical NICU. Thoracoscopic repair was attempted in 76 cases. In total, 30 cases were pure atresia/long gap type-C atresia and were excluded from the study. Remaining 46 cases met the inclusion criteria and were enrolled in the study. Mean age at operation was 8.7 days (range 2-32), and mean weight was 2.6 Kg (range 1.8-3.6). Apart from five cases (10.8%) converted to thoracotomy, the mean operative time was 108.3 minutes (range 80-122 minute). A tension-free primary anastomosis was possible in all thoracoscopically managed cases (n = 41) cases. Survival rate was 85.4% (n = 35). Anastomotic leakage occurred in seven patients (17%). Conservative management was successful in two cases, while esophagostomy and gastrostomy were judged necessary in the other for five. Anastomotic stricture developed in five cases (16.6%) of the 30 surviving patients who kept their native esophagus. Despite the fact that good mid-term presented results may be due to patient selection bias, thoracoscopic approach proved to be feasible for management of short-gap EA/TEF. Authors of this report believe that thoracoscopy should gain wider acceptance and pediatric surgeons should strive to adopt this procedure.

A case report of an asymptomatic male child with multiple entero-enteric fistulae post multiple magnet ingestion.

INTRODUCTION: Foreign body ingestion cases are very common in children. They usually present with obvious symptoms allowing proper diagnosis and management; however, magnet ingestion can be asymptomatic. This disorder is considered uncommon as well as challenging to diagnose before complications of intestinal obstruction or peritonitis occur due to entrapment of intestinal walls in the magnetic field. CASE PRESENTATION: An asymptomatic four-year-old male child was admitted to our centre after passing two bullet-shaped magnets in stool that were noticed by his mother. The child was asymptomatic. An abdominal X-ray revealed five bullet-shaped magnets in the lower abdomen. Laboratory investigations were normal except for a mild leukocytosis. Surgery was performed with excision of seven entero-enteric fistulae and two intestinal anastomoses. DISCUSSION: Between 2003 and 2006, the Centers for Disease Control and Prevention reported 20 complicated cases of magnet ingestion in children aged 10 months to 11 years among whom 75% had bowel perforations, and 20% suffered from generalized peritonitis. Half that number of patients required emergency laparotomy. As a result, the Consumer Product Safety Commission (CPSC) issued the first warning announcing the hazards of high-powered magnets used in children's toys, which had been increasing exponentially. It is clear that the diagnosis of magnet ingestion is made commonly due to complications, such as peritonitis or death. However, the patient in this case was completely asymptomatic and had no complications. CONCLUSION: A high index of suspicion regarding complications is recommended in asymptomatic cases of suspected magnet ingestion. Avoid delaying intervention once a diagnosis has been made.

Laparoscopic Diamond Antroduodenostomy for Postcorrosive Pyloric Cicatrization: A Novel Approach.

BACKGROUND: Traditionally, Billroth I procedure or bypass gastrojejunostomy were the recommended approaches for management of postcorrosive complete gastric outlet obstruction (GOO), whereas Heineke Mickulicz pyloroplasty was recommended for moderate mucosal injury with partial cicatrization. In this study, laparoscopic diamond antroduodenostomy was carried out as an alternative minimally invasive approach for cases with pyloric cicatricial obstruction. PATIENTS AND METHODS: Between January and December 2017, children who were referred to Pediatric Surgery Department, Cairo University Hospital, with GOO as a consequence of caustic liquid ingestions were included in this study. Laparoscopic diamond antroduodenostomy was performed for the presented cases. RESULTS: Through the year 2017, 5 cases were approached with laparoscopic diamond antroduodenostomy. Isolated pyloric cicatrization was evident in 4 cases, whereas synchronous insult to thoracic esophagus and pylorus was manifest in the fifth case. Laparoscopic feeding jejunostomy completed the procedure for the case with esophageal stricture. Contrast study-24 hour postoperatively-assured no radiological leaks in the presented cases, where enteral feeding was gradually commenced, and patients discharged home a day later. After a mean follow-up of 13.5 months, neither recurrence of obstructive symptoms nor dumping was displayed. Cosmetic outlook inherent to the minimally invasive approach was appreciated by the parents. CONCLUSION: Laparoscopic diamond antroduodenostomy is a feasible approach for management of postcorrosive pyloric obstruction. It allowed early enteral feeding, with no dumping symptoms, in addition to the fundamental advantages of minimally invasive surgery. A bigger series and longer follow-up is recommended to verify the reported results.

Timing and Outcome Concerns regarding Feminizing Genitoplasty from the Perspective of Egyptian Families of Girls with Virilized External Genitalia.

BACKGROUND: Congenital adrenal hyperplasia in females leads to virilization of external genitalia and persistent urogenital sinus. There are controversies regarding the timing and outcomes of surgery. Deferring surgeries beyond childhood is difficult to implement in conservative societies, and may result in stigmatization and distress to individuals with disorders of sexual differentiation and their families. METHODS: Thirty girls with virilization due to congenital adrenal hyperplasia were admitted for single-stage feminizing genitoplasty, between 2011 and 2014. We prospectively studied the concerns and input of the families represented by the mothers. After comprehensive counselling, the mothers completed a questionnaire to clarify their priorities and concerns related to surgery. RESULTS: Surgeries were performed at a mean age of 22 months. Most cases ranged between Prader's degrees III and IV. Egyptian families believe that early surgical reconstruction is in the best interest of their girls. They are marginally more concerned about functional outcomes and future child bearing than external appearance and cosmetic outcomes. CONCLUSIONS: Social difficulties noticeably add challenges to the management plan within conservative societies. Early genital reconstructive surgery, when reasonably indicated, needs to remain a viable option. Comprehensive psychosocial support within a multidisciplinary approach is needed to defer feminizing genitoplasty in selected cases to adolescence.