The dilemma after an unforeseen aortic arch anomalies at thoracoscopic repair of esophageal atresia: Is curtailing surgery still a necessity?

BACKGROUND AND OBJECTIVE: There are several forms of relevant epi-aortic branching anomalies, and perhaps that is why different views as to the best approach have been reported. To help resolve this dilemma, we examined the unforeseen arch anomalies found at thoracoscopic repair of esophageal atresia and the outcomes. METHODS: In a retrospective cohort, all consecutive patients who were thoracoscopically approached for esophageal atresia over a 5-year period with unforeseen aortic/epi-aortic branching were identified and grouped. Thoracoscopic views, operative interventions, and outcomes were studied. RESULTS: A total of 121 neonates were thoracoscopically approached for EA, of whom 18 cases with aberrant aortic architecture were selected. Four (3%) cases were diagnosed on a preoperative echocardiography as a right-sided aortic arch, whereas unforeseen anomalous anatomies were reported in 14 cases (11.6%): left aortic arch with an aberrant right subclavian artery (ARSA) (n = 10), right-sided aortic arch with an aberrant left subclavian artery (ALSA) (n = 3), and mirror-image right arch (n = 1). Single postoperative mortality was reported among the group with left arch and ARSA (10%), whereas all the cases with right arch and ALSA died. CONCLUSIONS: In all, 11.6% of the studied series exhibited unexpected aberrant aortic architecture, with higher complication rates in comparison to the typical thoracoscopic repairs. For EA with left aortic arch and ARSA, the primary esophageal surgery could safely be completed. Meanwhile, curtailing surgery-after ligating the TEF-to get advanced imaging is still advised for both groups with the right arch due to the significant existence of vascular rings.

Thoracoscopic repair of congenital diaphragmatic hernia: a new anatomical reconstructive concept for tension dispersal at primary closure.

BACKGROUND: Several measures were implemented among authors striving to tail off recurrence rates of thoracoscopic congenital diaphragmatic hernia repair. In the presented study, we extended the use of rib-anchoring stitches to reorient the diaphragmatic muscle leaflets in the types B&C diaphragmatic hernias, to achieve tension dispersal at primary thoracoscopic repair. PATIENTS AND METHODS: Included in this study were early and late-onset lateral congenital diaphragmatic hernia patients, who had been operated upon in the years 2012 through 2018. A preliminary stitch was taken between posterior muscle edge and rib cage to reorient the diaphragmatic defect into a reversed C-shaped line. The lateral portion was closed by additional rib-anchoring stitches, while the medial one necessitated muscle to muscle stitches. Primary outcome being validated was the recurrence rate within a year post repair. RESULTS: In the 7-year inclusion period, 36 congenital diaphragmatic hernia cases were managed using the described approach. The repair was accomplished thoracoscopically in all but two cases, who were excluded from the study. Mean operative time was 76 min. No pledgets or synthetic patches were applied. Mean length of hospital stay was 7.6 days. Early postoperative course was uneventful in all but four cases; two ventilatory barotrauma and two mortalities. After a mean follow-up period of 29 months, five recurrences were reported (16%). Ipsilateral chest wall deformity was noticed in one case 3 years post repair. CONCLUSION: In the presented study, authors adopted thoracoscopic reorientation of diaphragmatic muscle leaflets in lateral congenital diaphragmatic hernia cases to achieve tension dispersal at primary repair. Short and mid-term results supported the efficacy and reproducibility of the described approach. However, long-term comparative studies seemed a necessity to validate this outcome.

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.

Fetus in Fetu: Lessons Learned from a Large Multicenter Cohort Study.

INTRODUCTION: Fetus in fetu (FIF) is an extremely rare condition of abnormal twinning during embryogenesis. Most publications are single case reports. We describe the combined experience of four large tertiary referral centers with FIF which were not previously reported or published, and thereby draw conclusions to establish criteria for the workup, diagnosis, and management including intraoperative risk. MATERIALS AND METHODS: A survey was forwarded to a national pediatric surgery group which includes members from all pediatric surgery centers in the country enquiring about unpublished cases of FIF encountered over a 20-year interval. The cohort was analyzed for age of presentation, type of presentation, diagnostic workup, surgical management, and outcome. RESULTS: From 1998 to 2018, a total of 10 FIF cases were included in the study. Mean age of presentation was 4 months. Computed tomography and ultrasound were the main preoperative diagnostic modality in our cohort. Resection of the mass was curative in nine cases. Two cases in which the FIF was in direct topographic proximity to the biliary tree suffered severe intraoperative or lethal postoperative complications. CONCLUSION: Complete excision of FIF is the treatment of choice and generally results in excellent long-term quality of life. Mortality is rare and may be associated with biliary involvement and retroperitoneal right upper quadrant location of the FIF tends to be associated with increased risk in excision, and there is also a possible association with the presence of immature elements in the pathology report.

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.

Neo-yoke repair for severe hypospadias: A simple modification for better outcome.

BACKGROUND: Although staged repair for reconstructing severe hypospadias is more popular, various one-stage repairs have been attempted. Koyanagi repair (parameatal-based and fully extended circumferential foreskin flap urethroplasty) has enabled correction of severe hypospadias in one stage. However, its un-acceptably high incidence of complications has initiated a series of technical modifications, including the "yoke" repair. OBJECTIVES: To retrospectively analyze the outcome of a proposed modification of the originally described yoke repair, for patients with severe hypospadias. This modification was developed to reduce complications. STUDY DESIGN: Over 4 years (between Jan 2011 and Jan 2015), all cases of severe hypospadias were included in this study; except those with prior attempts at repair, circumcised cases, and cases with severe hypogonadism - because of partial androgen insensitivity - not responding to hormonal manipulations. The make-up of the neo-urethra in this modification is the urethral plate with its spongiosal tissue proximally, a circum-coronal preputial pedicled flap in the middle, and an incorporated part of the augmented preputial flap and the preserved V-shaped glanular urethra, distally. Close postoperative follow-up was conducted to investigate the outcome. RESULTS: Thirty-one children with a median age of 32.48 months had repair of severe hypospadias using the neo-yoke technique. After a median follow-up of 26.7 months, the overall complication rate was 16.1%. Four children developed urethrocutaneous fistula (12.9%). Meatal drop-back occurred in one case (3.2%). No meatal stenosis or urethral sacculation was detected during follow-up of the studied group. Almost all cases had cosmetically appealing outlook. Single-staged repair of severe hypospadias using parameatal foreskin-based urethroplasty has passed through different modifications, all aimed at optimizing the outcome (Table). CONCLUSION: Neo-yoke repair for severe hypospadias is a natural development of established one-stage techniques, which resulted in better mid-term outcomes. However, an extended study is needed to declare the long-term results.

Major penile injuries as a result of cautery during newborn circumcision.

AIM: To report a grading system for complications of male circumcision, and to represent our experience with salvage surgery for postcircumcision electro-cautery penile ablation injuries. PATIENTS AND METHODS: Circumcision injuries presented to our department between January 2010 and January 2015 were collected and graded into five categories according to the degree of penile injury, and the possibility for surgical reversibility. RESULTS: Salvage surgery was carried out on 5 cases of post circumcision total phallic loss (grade V injuries). One of the cases had a postoperative meatal stenosis that responded to dilation under general anesthesia. No skin graft problems were documented in the other cases. The cosmetic outlook was greatly appreciated by the family and the operating team at follow up of all cases. CONCLUSION: Grading the post circumcision penile injuries is of paramount importance, and reconstructing the phallus using the hidden corporal bodies is considered worthwhile.

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.

Isolated Thoracoschisis: Case Report and Review of Literature.

A full-term male baby presented at day 2 postnatal with Riedel liver lobe, stomach, and much of the bowel herniating through a thoracic wall defect located just above and lateral to the left nipple. Operative conversion into a gastroschisis-like defect with construction of a silo bag was done. On thorough review of literature, it was found that this is only the second male infant reported with isolated thoracoschisis.

Cyclin D1 gene amplification in proliferating haemangioma.

Cyclin D1 gene amplification has been reported to promote abnormal endothelial cell proliferation and angiogenesis; these findings constantly present in proliferating haemangiomas. The present study was conducted to evaluate cyclin D1 gene amplification by fluorescence in situ hybridization analysis in tissue biopsies of 22 proliferating haemangiomas from 20 infants. Two significant correlations of cyclin D1 gene amplification with the early onset and the duplication of proliferating haemangiomas have been observed. Moreover, a significant correlation (P< or =0.05) has been found between the treatment parameters of proliferating haemangiomas with the amplified versus the normal cyclin D1 gene. Proliferating haemangiomas with the amplified cyclin D1 gene required more frequent flashlamp pulsed dye laser treatment sessions at the maximum dosimetry and more frequent intralesional steroid injections at the maximum dose/injection but treatment outcomes were limited. The more frequent post-treatment complications among proliferating haemangiomas with cyclin D1 gene amplification might be attributable not only to the associated more aggressive natural course, but also to the higher treatment parameters needed for effective treatment. Within the limitations of the present study, cyclin D1 gene amplification was seen for the first time in proliferating haemangiomas. We have found that the amplification of the cyclin D1 gene can predict the more aggressive natural course of proliferating haemangiomas and the limited outcome and higher incidence of complications after non-excision treatment modalities. The present findings reflect the possible usefulness of antisense cyclin D1 to improve the therapeutic outcome of proliferating haemangiomas.