Intestinal malrotation (IM) results from an altered or incomplete rotation of the fetal midgut around the superior mesenteric artery axis. The abnormal anatomy of IM is associated with risk of acute ...midgut volvulus which can lead to catastrophic clinical consequences. The upper gastro-intestinal series (UGI) is addressed as the gold standard diagnosis procedure, but a variable failure degree has been described in literature. The aim of the study was to analyze the UGI exam and describe which features are the most reproducible and reliable in diagnosing IM. Medical records of patients surgically treated for suspected IM between 2007 and 2020 at a single pediatric tertiary care center were retrospectively reviewed. UGI inter-observer agreement and diagnostic accuracy were statistically calculated. Images obtained with antero-posterior (AP) projections were the most significant in terms of IM diagnosis. Duodenal-Jejunal Junction (DJJ) abnormal position resulted to be the most reliable parameter (Se = 0.88; Sp = 0.54) as well as the most readable, with an inter-reader agreement of 83% (
k
= 0.70, CI 0.49–0.90). The First Jejunal Loops (FJL), caecum altered position and duodenal dilatation could be considered additional data. Lateral projections demonstrated an overall low sensitivity (Se = 0.80) and specificity (Sp = 0.33) with a PPV of 0.85 and a NPV of 0.25. UGI on the sole AP projections ensures a good diagnostic accuracy. The position of the third portion of the duodenum on lateral views showed an overall low reliability, therefore it was not helpful but rather deceiving in diagnosing IM.
Data on the epidemiology and risk factors for pouchitis following restorative proctocolectomy and ileal pouch-anal anastomosis (IPAA) in pediatric patients with ulcerative colitis (UC) are scarce.
To ...determine incidence, risk factors and clinical outcome of pouchitis following IPAA in children.
This multicenter, retrospective cohort study, included all pediatric UC patients who underwent colectomy and IPAA from January 2010 to December 2016.
Eighty-five patients were enrolled. During a median post-surgical period of 24.8 (range: 1.0–72.0) months following IPAA, 38 (44.7%) patients developed pouchitis, including 6 (15.8%) who developed chronic pouchitis. Kaplan–Meier survival estimates of the cumulative probability for pouchitis were 14.6% at 1 year and 27.3% and 51.5% at 2 and 5 years, respectively. Multiple Cox regression model showed that older age at colectomy (hazard ratio, HR: 0.89, p = 0.008) was a protective factor, whereas chronic active colitis as indication for surgery (HR: 4.45, p < 0.001), and a 3-stage IPAA (HR: 2.86, p = 0.028) increased the risk for pouchitis.
Long-term risk for pouchitis is significantly high in pediatric-onset UC after IPAA. Younger age at colectomy, chronic active colitis as indication for surgery and 3-stage IPAA may increase the risk for pouchitis.
A 15-year-old girl was admitted to the Pediatric Emergency Department due to abdominal discomfort, anorexia, diarrhea, emesis, and weight loss. An inflammatory bowel disease diagnosis was suspected ...and a complete endoscopic investigation was deemed necessary. Initially, only colonoscopy with ileoscopy was performed, with evidence of widespread ileal ulcers, whereas a subsequent esophagogastroduodenoscopy revealed a huge trichobezoar that was further surgically removed. Trichobezoars are intraluminal conglomerates composed of ingested hair and are typically associated with underlying psychiatric disorders. Bezoars can be asymptomatic or present with various gastrointestinal symptoms, mimicking, among other conditions, inflammatory bowel disease. They may extend through the pylorus into the jejunum leading to Rapunzel Syndrome, which may cause intestinal obstruction. The treatment of trichobezoar depends on its location and volume. An endoscopic approach is possible for small-sized trichobezoars, but to avoid bezoar tail migration along the intestine, causing obstruction, conventional laparotomy is the only valid treatment for patients with Rapunzel Syndrome. Due to the increasing incidence of psychiatric disorders in pediatric age during the SARS-CoV-2 pandemic and their relationship with trichobezoars, an increase in cases of trichobezoars was observed. Thus, interdisciplinary collaboration between psychiatrists, pediatricians, and surgeons is crucial not only during the acute phase but also for long-term management and prevention of recurrence.
The combination of esophageal atresia, congenital duodenal obstruction, and anorectal malformation has seldom been reported. We describe the largest series of patients with such association, which we ...summed up with the mnemonic acronym DATE D-duodenal obstruction, A-anorectal malformation (ARM), and TE-tracheoesophageal fistula with esophageal atresia.
This was a multicenter retrospective review of 13 patients recruited from 8 institutions over a nearly 5-decade period (1968-2017). Information gathered included type of DATE malformations, other associated anomalies, type and timing of surgery, and clinical outcomes.
The DATE association consisted of type C esophageal atresia (13), complete (9) or incomplete (4) congenital duodenal obstruction (CDO), and high or intermediate (8) or low (5) ARM. Eight patients had at least one additional component feature of VACTERL association. A total of 6 patients died. Overall, 9 patients achieved complete restoration of gastrointestinal continuity, 7 of whom are alive at a median follow-up of 4 y (range, 1 to 9). Survivors received a median of 6 major operations (range, 4 to 14) to overcome their anomalies and surgical complications. Two incomplete duodenal obstructions were initially overlooked. All survivors with high or intermediate ARM defects required some form of bowel management to keep them clean.
The DATE association is a low-frequency entity, often occurring among the wider spectrum of VACTERL association. Functional outcomes largely depend on the severity of ARM or other major associated malformations. Awareness of the DATE association may avoid untoward diagnostic delays of subtler component features of the spectrum, such as an incomplete CDO.
In the last decade, the applicability of robotic surgery has been demonstrated in many interventions, expanding the indications of minimally invasive surgery also to pediatrics. The aim of the study ...is to evaluate postoperative pain to demonstrate better control following robotic procedures compared to thoraco–laparoscopic surgery. An observational, retrospective, multicentre study was performed involving 204 children undergoing robot-assisted surgery and thoraco/laparoscopic surgery at the Istituto Giannina Gaslini in Genoa and the Siena University Hospital (2013–2017): 83 children underwent robotic-assisted surgery and 121 thoracic–laparoscopic surgery. Personal data and type of intervention were assessed, dividing the patients into four categories: thoracic, gastrointestinal, hepatobiliary and urological surgeries. We analyzed the anesthetic risk according to ASA classification by type of intervention, the type of anesthesia used, the anesthetic drugs used during surgery and in the postoperative period. Both the problems that occurred during the procedures and the number of interventions converted into open during robotic surgery and laparoscopic thoracic surgery were analyzed. Pain was measured on the 1st, 2nd and 3rd day (FLACC or NRS scales). By comparing the two groups (robotics–non-robotics), the analysis shows that postoperative pain does not change with the chosen approach, but always maintains very low values, typical of minimally invasive surgery. The pain score is significantly higher in patients undergoing thoracic surgery, either robotic or thoracoscopic, compared to those undergoing gastrointestinal surgery (
P
corrected according to Bonferroni: 0.0006) and those undergoing urological intervention (
P
corrected according to Bonferroni: 0.04). In conclusion, no significant change in the intensity of postoperative pain between the two groups was found, while it is seen that the pain in patients undergoing thoracic interventions (robotic/thoracoscopic) is more intense than that reported for other types of interventions.
Background
Pilonidal disease is a troublesome acquired condition for whom various surgical treatments have been proposed with relatively high recurrence and complication rates. Since EPSiT technique ...has been described in 2013, it became an alternative treatment in adult practice. Our study reports the results of a multicentre series of pediatric patients who underwent EPSiT procedure over a 21-month period.
Methods
Between July 2015 and March 2017, all consecutive patients undergoing EPSiT in four different pediatric surgical units have been enrolled. Details regarding demographic data, detailed surgical procedure, outcome and complications have been recorded.
Results
A total of 43 patients underwent EPSiT procedure. Mean age was 15 years. There was a slight female preponderance. Mean weight and height at surgery were 67 kg and 168 cm, respectively. In 14% of cases a previous ineffective procedure was performed. Mean length of procedure was 34 min and median hospital stay was 24 h (12–72 h). Median length of follow-up was 4 months (range 3–18 months). Complications leading to reoperation were reported in 9% of cases with an overall resolution rate of 88%.
Discussion
EPSiT proved to be feasible and safe even in the pediatric population. The effectiveness and safety of the procedure suggest that this technique can represent a valid alternative for the treatment of pilonidal disease in children.
Surgical treatment of Hirschsprung disease (HD) evolved in the last decades with the introduction of new innovative minimally invasive techniques. The aim of present study is to compare the results ...from two centers, applying similar minimal access approaches, total transanal endorectal pull-through (TEPT) and laparoscopic-assisted transanal pull-through (LA-TAPT).
Data of all patients admitted between January 2011 and December 2016 in two Italian referral Hospitals for HD were retrospectively collected and analyzed. Exclusion criteria were as follows: redo procedure, patients with total colonic aganglionosis (ileostomy), patients lost at follow-up, or missing data. Patients who underwent TEPT and LA-TAPT were compared for gestational age, birth weight, age and weight at surgery, associated malformation, genetic syndrome, length of hospital stay, and early postoperative complications (within 30 days). Statistical analysis: Fisher's test and Mann-Whitney test; statistical significance set at
< .05.
None of the patients who underwent TEPT required laparotomy or laparoscopic assistance. Patients who underwent TEPT had lower age (
= .001), lower body weight (
< .0001), a significant higher rate of associated congenital heart disease (
= .006), and longer postoperative stay. In addition, the prevalence of perianal dermatitis was higher in TEPT patients (17/61 28% versus 2/46 4%,
= .001). The two groups were similar in terms of postoperative enterocolitis, redo surgery, anastomotic stenosis, and other infective complications.
Both minimally invasive techniques allow surgical advantages and outcomes; TEPT avoids pneumoperitoneum and the transperitoneal approach, with no need for laparoscopic instrumentation. TEPT group had longer postoperative stay, probably due to the higher prevalence of associated cardiac anomalies, and higher prevalence of perianal dermatitis, probably due to the lower age at operation.
Restorative proctocolectomy and ileal pouch-anal anastomosis (IPAA) is the recommended elective surgery for children with ulcerative colitis (UC). The aim of this study was to evaluate functional and ...long-term health-related Quality of Life (HRQoL) outcomes of surgery in pediatric patients with UC.
We reviewed the hospital records of all pediatric patients who had undergone surgery for UC between January 2009 and December 2016 in the Units of Pediatric Gastroenterology and Surgery, of both the University Hospital of Messina, and the Gaslini Children's Hospital of Genoa. Surgical treatment was represented by restorative proctocolectomy and laparoscopic IPAA. Patients and parents were interviewed by telephone before and after surgery and responded to the modified IMPACT III questionnaire about health outcomes and HRQoL. The questionnaire was scored on a five-point scale with higher scores indicating a better HRQoL. The total score ranged from 35 (worst HRQoL) to 175 (best HRQoL).
Data were obtained in 30 patients (16 males), with a median age of 12 (range 3-16). The median amount of time elapsed after the operation was 3 years (range 1-4.5). Preoperative scores were very low in all 4 domains of the questionnaire. Postoperatively, HRQoL measures improved significantly (P<0.05) on symptoms, school attendance, social activities, and emotional aspects. Overall, nearly all were completely satisfied with the outcome of surgery.
Our data confirmed that surgical treatment improves the overall HRQoL in pediatric patients with UC.
Congenital anomalies of the kidney and urinary tract (CAKUT) can be associated with Hirschsprung disease (HSCR). Based on the common genetic background of enteric nervous system and kidney ...development, the reported association of CAKUT and HSCR seems underestimated. Therefore, we designed a prospective study aimed at determining the prevalence of CAKUT in HSCR patients and at identifying RET, glial cell line-derived neurotrophic factor (GDNF), and GDNF family receptor alpha1 (GFRalpha1) mutations or haplotypes associated with this subset of HSCR patients. Eighty-four HSCR patients consecutively admitted to our department between July 2006 and July 2007 underwent interviews, notes review, ultrasound screening (further investigation according to detected anomaly), urinalysis, and DNA extraction for molecular genetics study. Another 27 patients with isolated CAKUT were included as a control group for the molecular genetics study. Twenty-one patients (25%) with HSCR had associated CAKUT, with hydronephrosis and hypoplasia being the most frequent diagnoses. Nine of 21 CAKUT were symptomatic. Six additional patients had other non-CAKUT anomalies (for example, stones, Barter syndrome) that were excluded from association and molecular genetics analysis to avoid bias of inclusion criteria. RET mutations were found in 5 patients (4 HSCR, 1 HSCR + CAKUT, 0 CAKUT) and GDNF mutations in 3 (2 HSCR, 1 CAKUT, 0 HSCR + CAKUT). No GFRalpha1 mutations were found. Finally, the HSCR-predisposing T haplotype of RET proto-oncogene was found in 64% of HSCR, 50% of HSCR + CAKUT, and in 24% of CAKUT patients. The incidence of CAKUT in HSCR patients is 4- to 6-fold higher than expected. Therefore, a patient with HSCR has a 3- to 18-fold higher risk of developing a CAKUT, particularly hydronephrosis or hypoplasia. If we consider that the proportion of predisposing haplotype in HSCR + CAKUT patients resembles that of other syndromic HSCR, we can conclude that HSCR + CAKUT has to be considered a novel syndromic association. These results need to be confirmed in a larger series. At present, we strongly suggest considering ultrasound screening of the urinary tract in every patient with a diagnosis of HSCR.
Fire in the operating theater is a potential source of important morbidity for the patient. Laser surgery of the head and neck district presents a particularly high risk of fire due to the presence ...of all three elements of the 'fire triad,' necessary to cause combustive or explosive events: an oxidiser, a fuel, and a heat source. The aim of the present study is to emphasise the need of new prevention tools and greater adherence to the recommendations available in the literature.
The sudden occurrence of combustion within the airway of an infant undergoing laryngeal laser surgery was presented along with his management.
An infant underwent CO2 laser surgery for the treatment of the laryngeal stenosis. Unfortunately, the endoscopic procedure was complicated by a fire of the tracheal tube. The tube was immediately removed, the saline was flushed down the trachea and the ventilation was maintained through a face mask. Subsequently, a fiberoscopy was performed and showed a vocal cord burn.
Since operating room fires are still an underreported occurrence, we believe that this present work might raise awareness about this potential complication and give useful suggestions for the management of airway fires in paediatric anaesthesia.
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