Surgical Dilemma: Exomphalos Major with Accessory Liver Lobe and Congenital Liver Cysts.

The association of exomphalos major, congenital liver cysts, and an accessory lobe of the liver is very rare. There are only two previously reported cases in the literature, both describing surgical excision of the accessory lobe with liver cysts during primary closure of the exomphalos defect. We report a case of this rare association, managed by delayed primary closure, where the cysts underwent spontaneous regression. This case, along with those previously reported, supports the etiopathogenesis theory of a malformative sequence of exomphalos and hepatic trauma within the sac of this rare association. Spontaneous regression of these cysts would favor a delayed primary closure in such cases.

Omphalomesenteric Duct Remnant: A Rare and Unusual Cause of Intestinal Atresia.

Intestinal atresia is generally considered to result from mesenteric vascular disruptions during fetal life. This report describes an unusual case of ileal atresia, resulting from an omphalic ring closure anomaly and an omphalomesenteric duct (OMD) remnant. A 2-day-old male neonate presented with bilious vomiting and abdominal distension. At laparotomy, the distal portion of the atretic ileum was entrapped within the umbilical ring, causing volvulus of the proximal dilated atretic bowel around the fixed distal bowel at the omphalic ring. This case report supports OMD remnants as a rare, potential cause of jejunoileal atresia.

Risk Factors for a Persistent Gastrocutaneous Fistula Following Gastrostomy Device Removal: A Tertiary Center Experience.

AIM: The aim of this study is to identify the risk factors for a persistent gastrocutaneous fistula (GCF) after gastrostomy device (GD) removal in children. MATERIALS AND METHODS: A retrospective analysis of 59 patients that underwent GD insertion and removal over an 11-year period (2005-2015). Patients were divided into two Groups (A and B) according to persistence or closure of the gastrocutaneous tract. Data included patient demographics, comorbidities, age at insertion, gastrostomy site infections, size and type of device, duration of placement, and method of insertion and removal. Statistical analysis was done using Chi-square test and ANOVA test where P < 0.05 was considered statistically significant. RESULTS: A total of 34 patients (Group A) developed a GCF post-GD removal. The gastrostomy tract closed spontaneously in 25 patients (Group B). Underlying comorbidities did not influence spontaneous closure. Younger age at insertion (<2 years), longer duration of device placement, open gastrostomy insertion, upsizing the GD, changing a gastrostomy tube to a button, and site infections were significant risk factors for a persistent GCF. CONCLUSIONS: Risk analysis of persistent GCF is important for patient counseling before removal or replacement of the GD. We have identified a number of potentially reversible risk factors for a persistent GCF and have made recommendations accordingly.

Paediatric robotic surgery: a narrative review.

The benefits of minimally invasive surgery (MIS) compared with traditional open surgery, including reduced postoperative pain and a reduced length of stay, are well recognised. A significant barrier for MIS in paediatric populations has been the technical challenge posed by laparoscopic surgery in small working spaces, where rigid instruments and restrictive working angles act as barriers to safe dissection. Thus, open surgery remains commonplace in paediatrics, particularly for complex major surgery and for surgical oncology. Robotic surgical platforms have been designed to overcome the limitations of laparoscopic surgery by offering a stable 3-dimensional view, improved ergonomics and greater range of motion. Such advantages may be particularly beneficial in paediatric surgery by empowering the surgeon to perform MIS in the smaller working spaces found in children, particularly in cases that may demand intracorporeal suturing and anastomosis. However, some reservations have been raised regarding the utilisation of robotic platforms in children, including elevated cost, an increased operative time and a lack of dedicated paediatric equipment. This article aims to review the current role of robotics within the field of paediatric surgery.

How to establish a successful bowel management programme in children: a tertiary paediatric centre experience.

BACKGROUND: Bowel management is a term used to describe a medical approach to the management of faecal incontinence. AIMS: To present the outcomes of an individualised bowel management programme developed by a tertiary paediatric centre and evaluate contributory factors for successful bowel management in children. METHODS: A retrospective review of children attending a bowel management clinic in a tertiary centre in Dublin, Ireland, over 5 years (2010-2015). The main outcome measure was the ability to achieve a regular bowel pattern and remain socially clean. Multiple linear regression analyses were used to determine the factors contributing to successful bowel management. RESULTS: One hundred ninety-two children attended the clinic over 5 years. The median age at commencement of washouts was 7 years. Underlying diagnosis was spina bifida in 50%, imperforate anus in 17.7%, Hirschsprung's disease in 14.6%, idiopathic constipation in 7.8%, and other conditions in 9.9% of patients. Children with spina bifida and Hirschsprung's disease preferred Peristeen washouts, while those with imperforate anus and idiopathic constipation preferred Willis washouts (p < 0.001). Our programme was successful in 93.7% of cases. Regression analysis showed that the underlying condition (p < 0.001), washout medication (p = 0.016), and individuals administering washouts (p < 0.001) contributed to a successful bowel management programme. CONCLUSION: Treatment protocols should be individualised based on the underlying condition and outcomes. Decision-making must be cognisant of the physical, social, psychological, and developmental needs of the child and family. A partnership approach is advocated, which includes child and parent/carer preferences allowing them to make an informed decision.

Medium-term outcome of endoluminal gastroplication with the EndoCinch device in children.

OBJECTIVE: Endoluminal gastroplication (EG) is emerging as a minimally invasive procedure for the treatment of gastroesophageal reflux disease (GERD). The aim of this study is to evaluate the medium-term outcomes after EG in a pediatric patient population. PATIENTS AND METHODS: Seventeen children with a median age of 12.4 years (range 6.1-15.9 years) with GERD underwent EG using a flexible endoscopic sewing device (EndoCinch) over a period of 3 years. Three plications were placed in the gastric tissue below the lower esophageal sphincter. Drug dose requirement, pH measurements, symptom severity and frequency, and validated Quality of Life in Reflux and Dyspepsia (QOLRAD) scores were compared before EG and 1 and 3 years after EG. Statistical analysis was performed using a Wilcoxon rank-sum test and P < 0.05 was the threshold for significance. RESULTS: All patients showed an immediate posttreatment improvement in symptom severity, symptom frequency, and quality of life scores. Completed 1- and 3-year data were obtained from 16 patients. Four cases (25%) required a repeat procedure as a result of recurrence of symptoms after 2 to 24 months. Fourteen patients (88%) at 1 year and 9 patients (56%) at 3 years remained without a need for any antireflux medication. A sustained improvement in heartburn (P = 0.004), regurgitation (P = 0.017), and vomiting (P = 0.018) was seen at 3 years. The total QOLRAD score (maximum of 175) improved from a median of 87 (range 69-142) to 156 (range 111-175) at 1 year (P < 0.0001) and 153.5 (range 55-174) at 3 years (P = 0.002). CONCLUSIONS: EG is an effective and safe procedure in children. It is a viable option for the treatment of GERD refractory to or dependent on antireflux medications.

H-type tracheoesophageal fistula with type III laryngotracheoesophageal cleft.

H-type tracheoesophageal fistula and laryngotracheoesophageal cleft are both rare anomalies. Laryngotracheoesophageal clefts are identified as a part of Opitz-Frias syndrome. We report a neonate with this combination of rare congenital anomalies. These associated malformations can have major implications in terms of resuscitation, diagnosis and surgical management, which are discussed.

Clinical application and diagnostic yield of wireless capsule endoscopy in children.

OBJECTIVE: The small bowel is anatomically difficult to examine and was investigated by invasive, indirect modalities, such as push enteroscopy and small bowel follow-through. The aim of this study was to assess the efficacy and clinical impact of wireless capsule endoscopy (WCE) in children. MATERIALS AND METHODS: Over the last 3 years (2002-2005), 37 patients with suspected small-bowel disease were investigated with WCE at a median age of 11 years (range, 16 months-16 years). The indications for WCE was suspected Crohn's disease (CD) (18), obscure or occult gastrointestinal bleeding (7), polyposis syndromes (5), protein losing enteropathy (4), recurrent abdominal pain (2), and malabsorption syndrome (1). All patients had preceding upper gastrointestinal endoscopy (OGD), ileocolonoscopy, and 26 cases had a small bowel follow-through (SBFT). These results were compared with the findings on WCE. RESULTS: Thirty-three (33) cases successfully completed the WCE through the small bowel. Four (4) patients were unable to swallow the capsule, 3 of which had to be placed in the duodenum endoscopically. In 3 patients, the capsule remained in the stomach and no small bowel images were obtained. The overall diagnostic yield was 85% (28/33 patients). The diagnostic findings included CD (13), source of gastrointestinal bleeding (7), polyposis syndromes (3), erosive enteropathy and patchy lymphangiectasia (4), and intussusception (1). WCE was found to be more sensitive for small bowel pathology than SBFT (20 vs. 6 [30% sensitivity, compared to WCE]) and endoscopic investigations (28 vs. 12 [43% sensitivity compared to WCE]). As a result of WCE findings, there was a positive alteration in the management in 28 of 33 (85%) cases. CONCLUSIONS: WCE is a novel, noninvasive, and useful tool for the investigation of the small intestine in children. It is superior and more sensitive than other conventional endoscopic and radiologic investigations in the assessment of the small bowel. It can help in guiding surgical decisions and should be routinely integrated as a part of the diagnostic work-up of small bowel pathology.

Prenatal bowel dilatation and the subsequent postnatal management.

The presence of dilated bowel loops antenatally suggests fetal bowel obstruction. Neonatal intestinal obstruction can have different variations in presentation depending on the level and extent of obstruction. Some of these conditions can be diagnosed antenatally. Antenatal detection of surgically correctable anomalies would ideally reduce perinatal morbidity and mortality by allowing a planned delivery with early resuscitation and prompt surgical intervention. Duodenal atresia is the most common intestinal atresia diagnosed in a fetus. Presently there are no significant abnormalities of the fetal gastrointestinal tract that benefit from fetal intervention. However a thorough understanding of the disease processes is necessary for diagnosis and treatment of intestinal obstruction. With advances in neonatal intensive care and management there has been a significant decrease in mortality rates of neonates with intestinal obstruction.

Laparoscopic antegrade continence enema using a two-port technique.

BACKGROUND: The antegrade continence enema is an effective method of treatment of fecal incontinence. We report our experience of a laparoscopic antegrade continence enema procedure and describe a simple approach to this procedure using a two-port technique. MATERIALS AND METHODS: Over a 3-year period, 12 children with intractable constipation and fecal soiling underwent the antegrade continence enema procedure laparoscopically. All cases had full bowel preparation the day before surgery. This procedure was done through one 5-mm camera port and two 5-mm working ports in 8 cases, and using the camera port and only one additional 5-mm working port in 4 cases. The appendix was used in 5 cases and the cecum in 3 cases with the threeport technique while the appendix was used in all 4 cases with the two-port technique. The appendix or cecum was delivered extracorporeally through the 5-mm port site in the right lower quadrant. The mucocutaneous anastomosis was stented using a gastrostomy button. RESULTS: Between 2001 and 2004, 12 children (10 male, 2 female) underwent a laparoscopic antegrade continence enema procedure at a median age of 10.5 years (range, 7-14 years). This procedure was easy to perform and no case required conversion to an open procedure. The wash-outs via the MIC-KEY gastrostomy button (MIC-KEY, Kimberly-Clark) were commenced at a median of 3.5 days (range, 1-5 days). Median postoperative hospital stay was 2 days (range, 1-5 days). This procedure was effective in completely resolving fecal incontinence in 9 cases and partially resolving it in 3 cases. There were no episodes of stomal stenosis, leakage, or herniation. However, one case required a revision of antegrade continence enema due to wound breakdown and leakage of irrigation fluid around the stoma. The median follow-up period was 15.5 months (range, 5-32 months). CONCLUSION: The laparoscopic technique is a simple and effective approach in creating an antegrade continence enema. The use of a gastrostomy button can potentially reduce some of the complications commonly associated with an antegrade continence enema. We describe a procedure that incorporates the advantages of both laparoscopy and a button device, which is simple and easy to perform using just two ports.

Repetitive eructation (belching) in children: is it pathological?

Eructation (belching) is a common symptom encountered in clinical practice. It has a similar mechanism as gastroesophageal reflux disease (GORD), which is transient lower oesophageal relaxation and is generally assumed to be a manifestation of GORD. Repetitive eructation can be distressing and its management can be difficult and challenging. This report highlights this problem in two adolescent children with a comprehensive review and discussion of its etio-pathogenesis.

Congenital Middle Mesocolic Hernia: A Rare Cause of Neonatal Intestinal Obstruction.

Congenital mesocolic hernia is an extremely rare, but serious cause of intestinal obstruction in children. Given the rarity of this condition, delays in diagnosis and management can have catastrophic consequences. Congenital mesocolic hernias are usually caused by an abnormal rotation of primitive mid-gut and are divided into left and right congenital mesocolic hernias. We report and discuss the clinical and radiological features and management of a neonate with an extremely rare variant, congenital middle mesocolic hernia along with a literature review of this rare condition.

Rare Association of Extended Total Colonic Aganglionosis and Intestinal Malrotation.

Total colonic aganglionosis occurring together with malrotation is a rare occurrence and may pose diagnostic and management dilemmas for the paediatric surgeon. We report a case of a neonate that presented with extended total colonic aganglionosis and malrotation, along with a spectrum of central nervous system and renal abnormalities. The clinical and radiological features and potential diagnostic and management pitfalls are discussed along with a literature review of this exceptionally infrequent association.

Gonadoblastoma in patients with 45,X/46,XY mosaicism: A 16-year experience.

BACKGROUND: It is recognised that individuals with a 45,X/46,XY karyotype, known as Turner mosaic syndrome with Y chromosome material (TMSY), have an increased risk of developing gonadoblastoma (GB), which may then devolve into one of a number of germ cell malignancies. Hence, children with TMSY are usually recommended to undergo prophylactic gonadectomy. OBJECTIVE: We designed this study to describe the phenotypic features of our series of children with TMSY who underwent prophylactic gonadectomy in order to evaluate the prevalence of GB and germ cell malignancies in their resected specimens. STUDY DESIGN: This is a retrospective case series wherein we comprehensively reviewed the clinical, histological, and cytogenetic features of all patients who underwent prophylactic gonadectomy at three tertiary paediatric referral centres over 16 years. Cases were identified from surgical logbooks and through the institutional histopathology database. Data were collected with particular reference to clinical phenotype, predominant karyotype cell line, operative management, anatomical findings and the presence of neoplastic changes. RESULTS: Fourteen children ranging in age at the time of surgery from 2 weeks to 17 years were included in the series. Eleven children were reared as females. The three children who were reared as males had severe penoscrotal hypospadias. The 46,XY cell line was the predominant cell line in seven (50%) cases in blood lymphocytes. The resected specimens from four patients (28.6%) contained GB, with three patients having bilateral GB. This sub-group of patients with GB were aged 5 months, 48 months, 71 months, and 13 years. GB arose in one patient with and three patients without genital virilisation. There was no focus of invasive germ cell tumour in any specimen. DISCUSSION: GB may be present in infants with TMSY as young as 5 months, even with low levels of Y chromosome material. The prevalence of GB in prophylactic gonadectomy specimens is similar to many previously reported series, although the absence of dysgerminoma in our series is reassuring. The exclusive presence of GB in intra-abdominal gonads is in keeping with the findings of several other series. CONCLUSION: Owing to the presence of gonadoblastoma in the gonads of children with TMSY as young as 5 months, we recommend that all patients with intra-abdominal gonads in the context of TMSY should duly undergo prophylactic gonadectomy, although the timing of such surgery can be discussed with parents during counselling regarding the risk of malignancy.

Laparoscopic-assisted transanal endorectal coloanal anastomosis for Hirschsprung's disease.

BACKGROUND: There has been a recent trend in the use of laparoscopic-assisted one-stage pull-through in the management of Hirschsprung's disease (HD). We describe our initial experience using laparoscopy with a transanal coloanal anastomosis as described by Rintala and Lindhal for HD. METHODS: Six children with biopsy-confirmed HD underwent laparoscopic-assisted pull-through using Rintala's transanal endorectal coloanal anastomosis. The procedure was done through one 5-mm camera port and two 5-mm working ports. The transition zone was identified by seromuscular biopsies obtained laparoscopically. The sigmoid colon and proximal rectum were mobilized laparoscopically. A transanal endorectal mucosal dissection and a coloanal anastomosis were done, using an absorbable monofilament 5/0 polyglyconate suture. RESULTS: Six children aged 4 weeks to 36 months underwent this procedure laparoscopically. Two cases had to be converted to an open procedure as a result of dense pelvic adhesions. The entire mobilization of the bowel as well as biopsy confirmation of the transition zone was done laparoscopically in all 6 cases. The median operative time was 135 minutes (range, 120-240 minutes). All 6 children tolerated full enteral feeds after 48 hours and the median hospital stay was 7 days (range, 6-10 days). There were no early postoperative complications. Two cases developed mild enterocolitis that resolved with conservative management. The overall functional outcome was good in all cases with no soiling, stool incontinence, or constipation at a median follow-up period of 12 months (range, 4-27 months). CONCLUSION: Laparoscopic-assisted pull-through, apart from being cosmetically superior, permits obtaining biopsies as well as an adequate mobilization of the bowel. The transanal endorectal coloanal anastomosis technique is simple and easy to perform, with a minimal dissection which causes less damage to the internal sphincter and pelvic nerves.

Laparoscopic Excision of Large Intra-Abdominal Cysts in Children: Needle Hitch Technique.

Laparoscopic surgery has both diagnostic and therapeutic advantages in the management of intra-abdominal cysts in children. Large cysts in small children pose technical challenges during laparoscopic surgery, requiring multiple incisions and advanced laparoscopic skills. This paper describes a novel laparoscopic technique using minimal manipulation for both aspiration and excision of the cyst. This simple, safe, and effective approach was used to achieve traction and facilitate excision of a large intra-abdominal cyst in a neonate and a young child.

Pyeloplasty for pelvi-ureteric junction obstruction in malrotated kidneys in children.

PURPOSE: Pelvi-ureteric junction (PUJ) obstruction associated with malrotated kidney is very rare. In such cases, dismembered pyeloplasty poses technical difficulties. We present our experience with management of PUJ obstruction in malrotated kidneys in children and their outcomes. METHODS: Retrospective review of case notes of all children who had pyeloplasty for PUJ obstruction associated with malrotated kidneys, over a period of 7 years between January 2003 and December 2009 at our institution. RESULTS: We identified four patients, and all four patients had malrotated kidneys with a lateral and slightly inferior facing renal pelvis. Anderson and Hynes dismembered pyeloplasty with inferior pelvi-ureteric anastamosis was performed in two patients (one patient with nephropexy and one patient without nephropexy), but failed to improve the drainage across the PUJ in both the patients. The remaining two patients underwent a dismembered pyeloplasty with anterior pelvi-ureteric anastamosis that resulted in a good drainage. One of the patients who had pyeloplasty with inferior pelvi-ureteric anastamosis had a redo pyeloplasty with anterior pelvi-ureteric anastamosis that restored the drainage adequately. CONCLUSION: In our experience dismembered pyeloplasty with inferior pelvi-ureteric anastamosis along with or without nephropexy has not been successful in cases of PUJ obstruction in laterally malrotated kidneys. Although the number of cases in our study is small, we have had a 100% success with dismembered pyeloplasty with anterior pelvi-ureteric anastamosis. The authors are of the opinion that this modified approach is recommended as a viable option both as a primary and salvage procedure for PUJ obstruction in laterally malrotated kidneys.

Factors affecting the outcome of foreskin reconstruction in hypospadias surgery.

OBJECTIVE: Despite ongoing refinement of numerous techniques, the incidence of complications following hypospadias repair is still significant. The aim of this study is to evaluate the factors that affect the success in childhood of foreskin reconstruction with hypospadias repair. MATERIALS AND METHODS: A retrospective study was carried out of all primary hypospadias repairs with foreskin reconstruction (n=408) over the last 23 years. The hypospadias was coronal in 160 (39%), glanular in 114 (28%), subcoronal in 78 (19%) and distal penile in 56 (14%) cases. Foreskin reconstruction was included in 362 cases suitable for a meatal advancement (191) or distal urethral tubularization (171), and 46 cases for a flip-flap procedure (37 Mathieu, nine Barcat). Outcome analysis was of foreskin-related complications post surgery. RESULTS: Foreskin repair was successful in 333 cases (92%) that underwent meatal advancement/distal urethral tubularization, and 33 (72%) that underwent a flip-flap operation. Complications related to the foreskin occurred in 10% of the whole group with a urethral fistula rate of 8%. The median age at surgery was 13 months (2-120 months), and the median follow-up period was 11 months (1-100 months). CONCLUSIONS: A good cosmetic and functional outcome can be achieved with foreskin reconstruction combined with a variety of hypospadias repairs. The outcome in this series was better in cases of distal hypospadias using interrupted polyglactin sutures.