A Baby With Complete Androgen Insensitivity Syndrome and the Fortuitous Discovery of 45,X/46,XY Mosaicism.

Disorders of sex development (DSD) are caused by defects in the complex sexual differentiation cascade, resulting in discordance among an individual's genetic, gonadal, and genital sexes. It affects one in 4,500 live births. A wide spectrum of genital phenotypes can be found depending on the underlying pathogenic mechanism and the developmental stage that is affected. We herein report a newborn with female external genitalia but palpable gonads at labia majora with normal testicular function and structure, which is typical of complete androgen insensitivity syndrome (CAIS). The genetic study revealed 45,X/46,XY mosaicism and c.2081A>C missense androgen receptor gene mutation, indicating the likelihood of co-existing CAIS. This case demonstrated the importance of correlating genital phenotype and the underlying pathogenic mechanism, to provide appropriate management of DSD. Important considerations on managing the gonads about the risks of gonadal malignancies are also discussed.

A combined approach of robot-assisted laparoscopic pyeloplasty and flexible endoscopy to treat concomitant ureteropelvic junction obstruction and calyceal stones in children: Technical considerations and review of the literature.

The management of children with concomitant ureteropelvic junction (UPJ) obstruction and calyceal stones remains challenging. The various treatment options available for pediatric nephrolithiasis may require multiple sessions, and the techniques by themselves are not designed for simultaneous correction of UPJ obstruction. Recently, success in combining robot-assisted laparoscopic pyeloplasty (RALP) and flexible endoscopy has been reported by multi-institutional studies to treat children with concomitant UPJ obstruction and renal stones. Given the paucity of technical details of this novel approach in the existing literature, we herein report our techniques to treat two girls aged 6 and 10 years who had concomitant UPJ obstruction and multiple stones in mid- and lower poles calyces. Three robotic ports were used without any assistant ports. A flexible endoscope, either a cystoscope or a single-use ureteroscope, was introduced via the undocked epigastric port to perform nephroscopy and stones removal after the renal pelvis was opened. The rest of the RALP was completed in the usual manner. Technical modifications were employed to facilitate the flexible endoscope to examine the entire calyceal system. Both patients underwent successful surgical procedures by the combined approach without any intra- or post-operative complications. Three and 14 stones were removed from each of the patients respectively. Postoperative investigations demonstrated successful correction of UPJ obstruction and complete stone clearance in both patients. A combined approach of RALP and flexible endoscopy is a safe and effective technique to treat concurrent UPJ obstruction and calyceal stones in children.

Surgery in Chinese children affected by 45,X/46,XY disorders of sex development: A 20-year experience in a single center.

BACKGROUND: Children affected by 45,X/46,XY mosaicism have a wide spectrum in their phenotypes. Although surgery has a role in management, there is no universally agreed strategy. METHODS: We conducted a retrospective review in a tertiary center on the surgical management and its long-term outcomes in children with the karyotype 45,X/46,XY or its variants over a 20-year study period. Patients were divided into 4 groups based on their phenotypes. RESULTS: 22 children(female=13, male=9) were included in the study, and were grouped as I)female phenotype(n = 11), II)ambiguous external genitalia(n = 2), III)undervirilized male with hypospadias(n = 6), and IV)normal male phenotype(n = 3). 19/22(86%) underwent gonadal surgeries(bilateral gonadectomy=15; unilateral gonadectomy=1; testicular biopsy=3) and  8/22(36%) underwent genitalia reconstructive surgeries(hypospadias repair=6; feminizing surgery=2). Gonadal germ cell tumors were detected in 55%, 50% and 50% of Group I, II and III respectively. 3/6 patients required reoperations for complications after hypospadias repairs. None of the patients had recurrence of germ cell tumors after gonadectomy. All the patients who had reached late adolescence or adulthood maintained their genders as their sex of rearing. CONCLUSIONS: Majority of 45,X/46,XY children benefit from timely gonadal surgery while genitalia reconstructive surgery can be considered in selected patients. Surgical management can be strategized by patients' phenotypes.

Comparing Robot-Assisted Laparoscopic Pyeloplasty vs. Laparoscopic Pyeloplasty in Infants Aged 12 Months or Less.

Objective: To investigate the outcomes of minimally invasive approach to infants with ureteropelvic junction (UPJ) obstruction by comparing the two surgical modalities of robot-assisted laparoscopic pyeloplasty (RALP) and laparoscopic pyeloplasty (LP). Methods: We conducted a retrospective review of all consecutive infants aged ≤12 months who underwent either LP or RALP in a single institution over the period of 2008-Jul 2020. We included primary pyeloplasty cases that were performed by or under the supervision of the same surgeon. Results: Forty-six infants (LP = 22; RALP = 24) were included with medians of age and body weight at 6 months (2-12months) and 8.0 kg (5.4-10 kg), respectively. There was no difference between the two groups in the patients' demographics and pre-operative characteristics. All infants underwent LP or RALP successfully without conversion to open surgery. None had intraoperative complications. Operative time (OT) was 242 min (SD = 59) in LP, compared with 225 min (SD = 39) of RALP (p = 0.25). Linear regression analysis showed a significant trend of decrease in OT with increasing case experience of RALP(p = 0.005). No difference was noted in the post-operative analgesic requirement. RALP was associated with a shorter hospital length of stay than LP (3 vs. 3.8 days; p = 0.009). 4/22(18%) LP and 3/24(13%) RALP developed post-operative complications (p = 0.59), mostly minor and stent-related. The success rates were 20/22 (91%) in LP and 23/24 (96%) in RALP (p = 0.49). Conclusions: Pyeloplasty by minimally invasive approach is safe and effective in the infant population. RALP may have superiority over LP in infants with its faster recovery and a more manageable learning curve to acquire the skills.

Impact of Age of Patient and Experience of Surgeon on the Outcome after Kasai Portoenterostomy: Can We Delay the Surgery?

INTRODUCTION: Age of patient and experience of biliary atresia (BA) center are well-known factors associated with early jaundice clearance (EJC) after Kasai portoenterostomy (KPE) in infants with BA. This study focused on the impact of age and surgeon factor on the short-term outcome after KPE within a single center. MATERIALS AND METHODS: Fifty-four consecutive infants (18 boys and 36 girls) who underwent KPE from January 2010 to January 2020 were reviewed. KPE was performed in the earliest available operative session once the initial work-up was completed. In group A (n = 41), KPE was performed by surgeon A. In group B (n = 13), KPE was performed by specialists under the supervision of surgeon B (who is the mentor of surgeon A) when surgeon A was not available for operation. The demographics of patients, the EJC (total bilirubin <20 µmol/L within 6 months of KPE), and 2-year native liver survival (NLS) between the two groups were studied. RESULTS: The median age at operation was 52 days (range 26-135 days). The overall EJC rate and 2-year NLS were 85.2 and 89.4%, respectively. Group A (p = 0.015) and male gender (p = 0.029) were statistically associated with EJC but not the age at operation (p = 0.101). Group A was also statistically associated with superior 2-year NLS (p = 0.047). CONCLUSION: Balancing between the impact of age at operation and the experience of surgeon on the outcome after KPE, our result suggested that KPE may be deferred until a more experienced surgeon to operate.

Long-Term Results of One-Stage Laparoscopic-Assisted Endorectal Pull-Through for Rectosigmoid Hirschsprung's Disease in Patients Aged Above 5 Years.

Purpose: Despite the fact that the laparoscopic-assisted endorectal pull-through (LAEPT) for Hirschsprung's disease (HD) was introduced over 20 years ago, published outcomes in older children and adolescent are lacking. To address this, we studied the long-term results of LATEP for rectosigmoid HD in patients above 5 years of age. Materials and Methods: A retrospective review was conducted on all nonsyndromic patients above 5 years of age and who underwent one-stage LAEPT for rectosigmoid HD between January 2002 and December 2017. Late Hirschsprung-associated enterocolitis (HAEC) was defined as HAEC occurring 1 year after the pull-through operation. Postoperative bowel function was assessed using the Krickenbeck classification. Results: Forty-one patients (37 males and 4 females) were included in this study with a median follow-up of 9.0 years. The median age at surgery was 55 days. Two patients had anastomotic leakage. No patient had late HAEC, rectal prolapse, anastomotic stricture, or intestinal obstruction. According to the Krickenbeck classification, all patients had voluntary bowel movements without constipation. Overall, 65.8% of patients had no soiling. However, on subgroup analysis, only 45.4% of patients younger than 11 years of age had no soiling compared with 89.5% in patients at or older than 11 years of age (P = .003). Conclusions: Our results showed that LAEPT for rectosigmoid HD was a safe procedure. Nearly 90% of patients had normal bowel function by puberty. Further studies are needed to address the problem of soiling in patients younger than 11 years of age.

Comparison of Characteristics and Outcomes in Antenatally and Postnatally Detected Choledochal Cyst in Infants and Young Children in a Laparoscopic Surgery Center.

Purpose: The aim of this study was to compare the characteristics and the outcome between infants and young children with antenatally (AN) and postnatally (PN) detected choledochal cyst (CC) in a laparoscopic surgery center. Methods: A retrospective review was conducted for all children who underwent excision of CC and hepaticojejunostomy (HJ) before 36 months of age between October 2004 and October 2019. Results: Thirty-nine children (28 girls and 11 boys) were included in this study. Twenty-one children had AN detected CC and 18 had PN detected CC. The median age at operation (AN vs. PN; 3 months vs. 15.5 months, P < .001) and body weight (AN vs. PN; 5.6 kg vs. 10.5 kg, P < .001) were significantly different between the two groups. Children in PN group has an increased risk of being symptomatic (AN vs. PN; 6 vs. 18, P < .001) and having intervention before operation (AN vs. PN; 0 vs. 4, P = .037). Laparoscopic excision was performed in all children in AN group and in 12 children (66.7%) in PN group (P = .006). Conversion to open HJ was performed in 4 children in AN group but none in PN group (P = .146). There was no statistical differences in success in laparoscopic operation (P = .257), median operative time (P = .094), postoperative complication (P = .576), and median length of hospital stay (P = .749). Conclusions: Despite younger age at operation, the outcome of laparoscopic excision of AN detected CC was comparable with PN detected CC. Earlier detection and operation decreased the risk of preoperative intervention.

A standardized technique of laparoscopic placement of peritoneal dialysis catheter with omentectomy and closure of patent processus vaginalis: A 3-in-1 minimally invasive surgical approach in children.

BACKGROUND: Omental wrapping is a common cause for catheter failure in children on peritoneal dialysis (PD). Previous studies are conflicting in the benefits of omentectomy. METHODS: We conducted a retrospective study comparing children who underwent PD catheter placement by a standardized laparoscopic three-in-one technique (lap3-in-1) from 2013 to 2018 versus a historical control cohort by open surgery without omentectomy. Lap3-in-1technique combined catheter placement with well-defined indication and extent of omentectomy, and closure of any patent processus vaginalis (PPV). RESULTS: There were 33 and 32 children in the lap3-in-1 and control cohorts respectively. 4/33(12.1%) in lap3-in-1 had reoperations for catheter failures which equated 1 reoperation per 144 catheter months. No reoperations were performed in lap3-in-1 cohort for omental wrapping or inguinal hernia, compared with 13/32 (41%; p < 0.001) and 5/32 (16%; p = 0.02) in the control cohort. Kaplan Meier survival curves showed significantly longer catheter life in the lap3-in-1 cohort (p < 0.001). In multivariate analysis by the COX proportional hazards model, the lap3-in-1 approach had significantly reduced risk of reoperation for catheter failure (HR 0.11; 95% CI: 0.04-0.31; p < 0.001). CONCLUSIONS: The lap3-in-1 technique is effective in selecting those children who would benefit from omentectomy, and avoiding a second operation for inguinal hernia which develops after PD. LEVEL OF EVIDENCE: Treatment study, level III.

The Hypospadias Phenotype With A Distal Meatus In The Presence Of Distal Penile Penoscrotal Angle Fixation.

OBJECTIVE: Hypospadias patients may present with the phenotype that features the migration of scrotum to distal penile shaft below a coronal/subcoronal meatus. Patients with this phenotype differ widely in the severity of the hypospadias and the complexity of the surgical repair. We aimed to investigate the operative findings and the outcomes of consecutive patients who presented with this phenotype. METHODS: We retrospectively reviewed the medical charts of 31 consecutive patients who underwent hypospadias repairs from January 2014 to May 2017, and the hypospadias was characterized by i) the external urethral meatus at coronal/subcoronal region, ii) scrotal skin encroaching distally resulting in fixation of penoscrotal angle at distal penile shaft, and iii) urethral plate below the glans on penile shaft invisible or barely visible. RESULTS: The median age at the time of surgery was 15.5 months (10-63). The division of corpus spongiosum was noted at distal penile shaft (n=2; 6.5%), mid-shaft (n=5; 16.1%) and proximal location (n=24; 77.4%). The median ventral curvature before degloving was 45 degrees (10-90). Twenty-eight and 3 patients underwent tubularized incised plate and 2-stage preputial flap repairs, respectively. Twenty-nine of 31 patients required cutback of the hypoplastic urethra. At the time of urethroplasty, 2 (6.5%), 7 (22.6%) and 22(70.9%) patients underwent distal, mid-shaft and proximal repairs, respectively. At a median follow-up of 30 months (14-50), 6 (19.4%) patients developed one or more complications (fistula=3; meatal stenosis=5). CONCLUSION: Patients affected by this particular phenotype likely require non-distal hypospadias repair with possibly higher complication rate and should be treated by surgeons with expertise in complex hypospadias repair.

Ten-Year Native Liver Survival Rate After Laparoscopic and Open Kasai Portoenterostomy for Biliary Atresia.

PURPOSE: Laparoscopic Kasai portoenterostomy (LKP) remains controversial in the management of infants with biliary atresia (BA). There are no data reporting the 10-year native liver survival rate after LKP. The study aims to present the 10-year native liver survival rate after LKP and complications in native liver survivors after Kasai portoenterostomy (KP). METHODS: A retrospective review was conducted for 31 consecutive infants with BA who underwent KP by day 75 of life in our institute from January 1993 to December 2007. The demographics and outcomes of patients after LKP and open KP (OKP) were compared. RESULTS: Eleven patients underwent LKP and 20 patients underwent OKP. No statistical difference was observed in the age at operation and the preoperative bilirubin level. The operative time for LKP was significantly longer than that for OKP (mean 314.5 minutes versus 271.5 minutes, P = .03). The 10-year native liver survival rate was 45% (5/11) after LKP and was 85% (17/20) after OKP (P = .03). Forty percent (2/5) of the native liver survivors in the LKP and 71% (12/17) in the OKP developed complications within 10 years after KP (P = .23). CONCLUSIONS: The 10-year native liver survival rate in patients who underwent LKP by 75 days of life was 45%. With superior 10-year liver survival rate and comparable complication rate after OKP, OKP is still the treatment of choice for BA in our institute.

Incidence and diagnoses of disorders of sex development in proximal hypospadias.

BACKGROUND: Evidence-based guidelines on evaluation of boys with proximal hypospadias for the possibility of a disorder of sex development (DSD) have yet to be developed. We aimed to investigate the incidence and diagnoses of DSD in patients with proximal hypospadias. METHODS: We retrospectively reviewed the records of consecutive boys who underwent proximal hypospadias repairs from 2006 to Sept 2017. Data collected included scrotal anomaly, testes position/palpability, micropenis, DSD investigations, and surgical techniques. RESULTS: 165 patients were eligible for the study. 14 (8.5%) were diagnosed to have DSD. The diagnoses were 46,XX testicular DSD [n = 1], 46,XY DSD [n = 7; partial gonadal dysgenesis (PGD) = 3; 5α-reductase type 2 deficiency = 3; 17α-hydroxylase deficiency = 1], Sex Chromosome DSD [n = 6; 45,X/46,XY PGD = 4; Klinefelter = 2]. 3/7 (43%) patients with PGD had gonadal germ cell neoplasms. Of the DSD patients, 6/14 (43%), 11/14 (79%) and 11/14 (79%) had undescended/impalpable testes, micropenis and penoscrotal transposition/bifid scrotum, respectively, significantly higher prevalence rates than those without DSD diagnosis (p-values <0.05). 10/14 (71.4%) DSD patients underwent 2-stage repair compared with 57/151 (37.7%) of others without DSD diagnosis (p = 0.01). CONCLUSIONS: Patients presenting with proximal hypospadias and one or more of the coexisting anomalies of micropenis, undescended/impalpable testes, and penoscrotal transposition/bifid scrotum should warrant DSD evaluation. Presence of bilaterally descended testes in scrotum does not preclude the possibility of DSD. LEVEL OF EVIDENCE: IV.

From Laparoscopic Pyeloplasty to Robot-Assisted Laparoscopic Pyeloplasty in Primary and Reoperative Repairs for Ureteropelvic Junction Obstruction in Children.

PURPOSE: Robot-assisted laparoscopic pyeloplasty (RALP) and laparoscopic pyeloplasty (LP) are both minimally invasive surgical options to correct ureteropelvic junction obstruction in children. There are limited data reporting surgeons' experience of switching from LP to RALP and comparing the outcomes of the two procedures. METHODS: We conducted a retrospective study on 63 consecutive children who underwent either LP or RALP by the same surgeon in a tertiary center from January 2008 to November 2016. LP had been the standard practice until January 2014 when it was replaced by RALP in short transition. RESULTS: Thirty-seven LP and 26 RALP were successfully performed in children aged 2 months to 16 years. There was no difference between the two groups in age, body weight, laterality, clinical presentations, preoperative imagings, primary/reoperative repairs. The overall success rates were 34/37 (91.9%) and 25/26 (96.2%) for LP and RALP, respectively (P > .05). RALP was associated with shorter length of stay (3.1 days versus 4.0 days; P = .03). Surgeon-in-training participation was greater in RALP group (P < .001). There was no difference in operative time, complication rates, and analgesic requirement between the two groups. CONCLUSIONS: Surgeons proficient in LP can adapt quickly to RALP, achieving comparable outcomes. Robotic technology may facilitate training on minimally invasive pyeloplasty.

Are all patients with short segment Hirschsprung's disease equal? A retrospective multicenter study.

BACKGROUND/PURPOSE: Short segment Hirschsprung's disease (HSCR) carries a better prognosis than long segment disease, but the definition of short is controversial. The objective of this study is to determine anatomically the extent of disease involvement that would be associated with a better functional outcome. METHODS: This is a retrospective multicenter (n = 3) study with patients (≥ 3 years) who had transanal pullthrough operation done for aganglionosis limited to the recto-sigmoid colon were reviewed. The extent of disease involvement and bowel resection was retrieved by reviewing the operative records as well as histopathological reports of the resected specimens. Clinical assessment was performed according to the criteria of a seven-itemed bowel function score (BFS) (maximum score = 20). Manometric assessment was performed with anorectal manometry. RESULTS: The study period started from 2003 to 45 patients were studied with median age at assessment = 52.0 months and operation = 3.0 months. The disease involvement was categorized into upper sigmoid-descending colon (DC) (n = 8), sigmoid colon (SC) (n = 12), upper rectum (UR) (n = 14) and lower rectum (LR) (n = 11) according to the level of normal biopsy result. There was no significant difference in the age of assessment between the four groups. The median BFSs in the DC, SC, UR and LR were 13, 15, 17 and 17, respectively (p = 0.01). Nine patients from the DC and SC groups reported soiling for more than twice per week. Sub-group analysis comparing patients with and without the entire sigmoid colon resected revealed worse functional outcomes in terms of the incidence of soiling (40.7 vs 22.2%, p = 0.05) and the BFS (14 vs 18, p = 0.04) in the former group. Anorectal manometry did not reveal any significant difference between the four groups, but a higher proportion of patients in the UR and LR groups appeared to have a normal sphincter resting pressure (DC vs SC vs UR vs LR = 62.5 vs 75.0 vs 85.7 vs 80.0%, p = 0.10). CONCLUSION: Patients with short segment HSCR are not equal at all. HSCR patients with aganglionosis limited to the rectum without the need of removing the entire sigmoid colon have a better bowel control and overall functional score. Less bowel loss and colonic dissection maybe the underlying reasons. Although future studies with a larger sample size and a longer follow-up period are required to validate the results of this study, it has provided a new insight to the current understanding of short segment disease in HSCR.

Clinical pattern and prevalence of upper gastrointestinal toxicity in patients abusing ketamine.

OBJECTIVE: Evaluations of upper gastrointestinal toxicity from ketamine abuse are uncommon. This study investigated the clinical pattern of upper gastrointestinal symptoms in patients inhaling ketamine. METHODS: In a cross-sectional study of 611 consecutive patients who were seeking treatment for ketamine uropathy in a tertiary hospital setting between August 2008 and June 2016, their clinical pattern of upper gastrointestinal symptoms was evaluated and compared with a control population of 804 non-users. RESULTS: A total of 168 (27.5%) patients abusing ketamine (mean age 26.3 years, 58.9% female) reported the presence of upper gastrointestinal symptoms. These symptoms were significantly more prevalent in patients inhaling ketamine than in those who were not (27.5% vs 5.2%, P < 0.001). Their mean duration of ketamine abuse before symptom presentation was 5.0 ± 3.1 years. The presenting symptoms included epigastric pain (n = 155, 25.4%), recurrent vomiting (n = 48, 7.9%), anemia (n = 36, 5.9%) and gastrointestinal bleeding (n = 20, 3.3%). Uropathy symptoms were preceded by upper gastrointestinal symptoms for 4.4 ± 3.0 years in 141 (83.9%) patients. Logistic regression showed that elder age (odds ratio [OR] 1.06, P = 0.04), active abuser status (OR 1.60, P = 0.04) and longer duration of ketamine abuse (OR 1.00, P = 0.04) were independent factors associated with upper gastrointestinal toxicity. CONCLUSIONS: Although epigastric symptoms are unusual in the young population, upper gastrointestinal toxicity was highly prevalent in those inhaling ketamine. Enquiries about ketamine abuse are recommended when assessing young patients with epigastric symptoms.

The Risk of Upper Urinary Tract Involvement in Patients With Ketamine-Associated Uropathy.

PURPOSE: The aims of this study were to investigate the prevalence of upper tract involvement in ketamine-associated uropathy, and to determine the predictors of hydronephrosis in patients with a history of ketamine abuse. METHODS: This was a cross-sectional study of a prospective cohort of patients with ketamine-associated uropathy. Data including demographics, pattern of ketamine abuse, pelvic pain and urgency or frequency (PUF) symptom score, uroflowmetry (UFM) parameters, serum renal function, and liver function tests were collected. Upon consultation, ultrasonography was performed to assess the function of the urinary system. RESULTS: From December 2011 to October 2015, we treated 572 patients with ketamine-associated uropathy. Of these patients, 207 (36.2%) had managed to achieve abstinence at the time of their first consultation. Ninety-six patients (16.8%) in the cohort were found to have hydronephrosis on ultrasonography. Univariate analysis identified age, duration of ketamine abuse, PUF symptom score, voided volume on UFM, serum creatinine levels >100 µmol/L, and an abnormal serum liver enzyme profile as factors associated with hydronephrosis. Logistic regression revealed the following parameters to be statistically related to hydronephrosis: age (adjusted odds ratio [OR], 1.090; 95% confidence interval [CI], 1.020-1.166; P=0.012), functional bladder capacity (adjusted OR, 0.997; 95% CI, 0.995-0.999; P=0.029), serum creatinine >100 µmol/L (adjusted OR, 3.107; 95% CI, 1.238-7.794; P=0.016, and an abnormal serum liver enzyme profile (adjusted OR, 1.967; 95% CI, 1.213-3.187; P=0.006). CONCLUSIONS: Ketamine-associated uropathy can involve the upper urinary tract. Patient demographics as well as investigations of UFM, renal function tests, and liver function tests may allow us to identify at-risk patients.

Cystic meconium peritonitis with jejunoileal atresia: Is it associated with unfavorable outcome?

AIM: To compare the outcome between patients with jejunoileal atresia (JIA) associated with cystic meconium peritonitis (CMP) and patients with isolated JIA (JIA without CMP). METHODS: A retrospective study was conducted for all neonates with JIA operated in our institute from January 2005 to January 2016. Demographics including the gestation age, sex, birth weight, age at operation, the presence of associated syndrome was recorded. Clinical outcome including the type of operation performed, operative time, the need for reoperation and mortality were studied. The demographics and the outcome between the 2 groups were compared. RESULTS: During the study period, 53 neonates had JIA underwent operation in our institute. Seventeen neonates (32%) were associated with CMP. There was no statistical difference on the demographics in the two groups. Patients with CMP had earlier operation than patients with isolated JIA (mean 1.4 d vs 3 d, P = 0.038). Primary anastomosis was performed in 16 patients (94%) with CMP and 30 patients (83%) with isolated JIA (P = 0.269). Patients with CMP had longer operation (mean 190 min vs 154 min, P = 0.004). There were no statistical difference the need for reoperation (3 vs 6, P = 0.606) and mortality (2 vs 1, P = 0.269) between the two groups. CONCLUSION: Primary intestinal anastomosis can be performed in 94% of patients with JIA associated with CMP. Although patients with CMP had longer operative time, the mortality and reoperation rates were low and were comparable to patients with isolated JIA.

Spontaneous Intestinal Perforation: The Long-Term Outcome.

Background/Purpose Evaluating the long-term outcome of spontaneous intestinal perforation (SIP). Methods We studied all patients treated for SIP at our institution between January 1, 2005 and December 31, 2014. Results Twenty-three infants (13 males) with a median gestational age of 26 (range: 23-32) weeks and a median weight of 825 (range: 560-1,965) g composed this cohort. Seventeen (74%) infants had an extremely low birth weight (ELBW); nine (39%) infants were the result of multiple pregnancies.Patent ductus arteriosus (PDA) was present in 16 (70%) infants. Cyclooxygenase inhibitors were administered in 12 (52%) infants.Ten infants (seven males, 44%) were diagnosed with intraventricular hemorrhage (IVH), which was identified in the majority (8/10) at a median of 9 (range: 1-11) days prior to the perforation.All patients presented with pneumoperitoneum and underwent a laparotomy at a median age of 9 (range: 2-16) days. Twenty-one patients had an ileal perforation. A temporary stoma was placed in 21 patients, whereas two got primary anastomosis. Two (8.7%) male infants died. During the long-term follow-up period (median 6 years), six (five males) (26%) infants developed moderate to severe disabilities in combination with cerebral palsy. No surgical complications were observed. Conclusion The most important risk factor for SIP is ELBW (75%). The distal ileum is the most frequent site of perforation (88%). Approximately 40% develop IVH most often prior to the SIP. Moderate to severe neurologic disabilities are seen in more than a quarter of the children. Disability and mortality affect mostly the male sex. Long-term risks of surgical complications are very low.

Early Reoperations after Primary Repair of Jejunoileal Atresia in Newborns.

Aim: To review nine-year experience in managing jejuno-ileal atresia (JIA) by primary resection and anastomosis and identify factors associated with reoperations. Methods: From April 2006 to May 2015, all consecutive neonates who underwent bowel resection and primary anastomosis for JIA were analyzed retrospectively. Patients with temporary enterostomy were excluded. Patient demographics, types of atresia, surgical techniques, need for reoperations, and long-term outcomes were investigated. Results: A total of forty-three neonates were included, in which nineteen (44.2%) of them were preterm and fourteen (32.6%) were of low birth weight. Thirteen patients (30.2%) had jejunal atresia whereas thirty patients (69.8%) had ileal atresia. Volvulus, intussusception and meconium peritonitis were noted in 12, 8, and13 patients, respectively. Eight patients (18.6%) had short bowel syndrome after operation. Ten patients (23.3%) required reoperations from 18 days to 4 months after the initial surgery due to anastomotic stricture (n=1), adhesive intestinal obstruction (n=1), small bowel perforation (n=2) and functional obstruction (n=6). Prematurity and low birth weight were associated with functional obstruction leading to reoperation (p=0.04 and 0.01 respectively). The overall long-term survival was 97.7%. All surviving patients achieved enteral autonomy and catch-up growth at a median follow-up of 4.7 years. Conclusion: Long-term survival of JIA after primary resection and anastomosis are excellent. However, patients have substantial risk of early reoperations to tackle intraabdominal complications.

Attitudes of Parents and Health Care Workers to Major Surgery for High-Risk Preterm Infants.

OBJECTIVES: To assess preferences of health care workers (HCWs) and parents of term and preterm infants to adverse health outcomes, and how perceived surgical mortality influences decision-making. STUDY DESIGN: A total of 536 participants (157 HCWs, 201 parents of term infants, and 178 parents of preterm infants) were recruited to take part in a structured interview. Preferences related to treatment of a critically ill preterm infant with necrotizing enterocolitis were measured by health state rank permutation analysis and pivotal risk analysis. Between-group and subgroup comparisons were performed. RESULTS: HCWs rank adverse health states less favorably than parents of preterm infants, consistently ranking 2 of the most adverse health states worse than death. Pivotal risk values of HCWs for all health states were consistently the lowest of the 3 groups. High operative mortality was associated uniformly with reduction in pivotal risks for all groups both in favorable and adverse health states. Subgroup analyses revealed significant discrepancies in preferences between fathers and mothers as well as doctors and nurses. Regular religious practice was significantly associated with increased pivotal risks in parental subgroups. CONCLUSIONS: As discrepancies in health state preferences existed between subgroups (ie, doctors vs nurses, mothers vs fathers) and perceived operative mortality consistently biased parental and HCW health state preferences, we recommend that HCWs should first identify differences regarding patient management before interviewing the parents together. HCWs should be aware of inadvertently biasing parents when discussing the risks and outcomes of surgery in conjunction with the overall long-term prognosis of the underlying condition.