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In Manchester, feminising genitoplasty is offered to children with 46XX Congenital Adrenal Hyperplasia (CAH) when there is a single perineal opening and/or enlarged clitoris. Our aims are to describe the anatomical reconstructive technique and present long-term outcomes. Our hypothesis is that 'the common channel (CC) length and distance to the vagina from perineal skin is mostly due to virilisation and hypertrophy of perineal tissue over the almost normally positioned vaginal introitus (V-I) in relation to the perineal body (PB)'. METHOD AND RESULTS: This is a retrospective notes review of all consecutive 46XX CAH operations from 1976 to December 2021. 99 patients, who had feminising genitoplasty and being followed-up, were included. 15 patients who were lost to follow up were excluded. Median age at surgery was 15 months. In 91, midline division of the labia majora, spongiosum, bulbo-spongiosus muscle (BSM) and CC down to PB was performed. This was sufficient to expose the V-I at the same level or within 5 mm depth of PB in 88. In 78 V-I was adequate taking 10/12fr dilator (Type 1). In 10, CC resembled a male urethra and V-I was narrow (Type 2), requiring widening by 5-10 mm incision at 6 o'clock position. Dartos of labia majora was attached to BSM to reduce the distance to V-I from perineal skin and the gap was lined with inner foreskin to create a vestibule. Out of 70 who were post-pubertal, 75% (53/70) had adequate calibre vaginal openings. 5 had introitoplasty and 2 had dilatation under anaesthesia. 10 needed self dilators only. 29 patients, of one of the three surgeons, had measurements of clitoris, CC, urethra and vagina. A hymen was found in 86% (25/29). There was significant strong, inverse correlation between the CC length and the urethral length (r = -0.708, p 0.001, n = 27) but not between CC and vaginal lengths. After adjusting for age, the urethral length of Type 2 patients was 3.825 mm shorter than those of Type 1 (p = 0.017). CONCLUSION: Our data show that 'high' confluence is mostly due to virilisation of genitalia; and the anatomical technique of reversing the fusion of the urethral folds, spongiosum and bulbo-spongiosus muscle could be performed with all degrees of virilisation with success in early childhood with no need of local flaps or mobilisation of the urethro-vaginal complex. About 10% require surgery to treat narrowing of vaginal opening post puberty.

Adhesions and fibrosis following failed primary surgery for severe gastro-oesophageal reflux (GOR) in neurologically impaired children (NI) can render mobilization of the lower oesophagus and oesophago-jejunal anastomosis a technically demanding exercise both at open surgery and laparoscopy. This paper presents the Modified Oesophago-Gastric Dissociation (M-OGD) as a less complex technical modification of the original Total Oesophago-Gastric Dissociation (TOGD). The stomach is detached from the oesophago-gastric junction with an articulated 5-mm stapler, leaving a 5-mm strip of stomach attached to the oesophagus. An end-to-side isoperistaltic oesophago-jejunostomy is created between the gastric stump and the isoperistaltic jejunal Roux loop. A jejuno-jejunal anastomosis restores bowel continuity. Between May 2018 and February 2020, M-OGD was performed on 3 NI patients with a weight of 9–27.3 kg (median = 14 kg). Median age at surgery was 60 months (18–180), median surgical time 170 min (146–280), median re-feeding time was 3 days (2–5), and median length of stay was 20 days (11–25). All patients healed primarily and after a median follow-up of 3 months, there were no problems related to the oesophago-jejunal anastomosis. M-OGD reduces the difficulties of redo oesophageal surgery following failed anti-reflux procedures, with a safer oesophago-jejunal anastomosis and a good long-term outcome.

Background Esophageal replacement in children is an option that is confined to very few situations including long-gap esophageal atresia and esophageal strictures unresponsive to other therapies (peptic or caustic ingestion). The purpose of our work was to describe the experience of gastric transposition in three Italian centers. Methods This is a retrospective study. The data were extrapolated from a prospective database. We included all patients who had undergone gastric transposition in the last 15 years. Results In the 15-year period, eight infants and children (3 males and 5 females) underwent gastric transposition for esophageal replacement. Six patients had long-gap esophageal atresia, and two had caustic esophageal stenosis. There were no deaths in the series. Three patients had an early postoperative complication: two had a self-limited salivary fistula at three weeks, and one (a patient with jejunostomy) had a jejunal perforation treated surgically. One late complication, anastomotic stricture, was recorded that required two endoscopic dilatations. The median follow-up was 60 months (range: 18-144 months). At final clinical follow-up, six patients had no eating problems, and two patients had some difficulties with eating (jejunostomy in situ), but they underwent logopedic therapy with improved outcomes. All patients had an increase in body weight and height postoperatively. Conclusion Our small study reports the clinical experience of three Italian centers in which gastric transposition was performed with excellent results, both in terms of surgical technique (simplicity, reproducibility, complication rate) and clinical follow-up (good oral feeding of young patients, normal social life and regular growth curves).

Summary Introduction To determine the outcome of distal hypospadias repairs performed using bipolar diathermy (BD) for all the dissection. Methods Retrospective review of 310 patients undergoing distal hypospadias (264 subcoronal/distal penile and 46 coronal/glanular) over a 11-year period. Median age at surgery was 2.0 years (range 9 months–15 years). Caudal anesthesia was performed in all patients. All children underwent an in situ tubularization of the urethral plate, which was combined with midline incision of the plate in 30 (10%). Preputial reconstruction was performed in 303 (98%) patients. BD forceps was used for coagulation and for all the dissection including skin incision, elevation of glans wings, separation of the corpus spongiosum from the corpora cavernosa, and urethral plate incision, when deemed appropriate. The authors assessed surgical complications and cosmetic results. The latter using the hypospadias objective score (HOSE), with a score ≥ 14 considered as acceptable. Results The average operative time was 70 min. There was no postoperative bleeding or hematomas that required surgical intervention. There were no wound infections or necrosis. Complications occurred in 37 patients (11.9%). Urethral fistula formation was the commonest (n = 18). Postoperative persistent preputial swelling occurred in 3.5% of cases. Two hundred twelve patients (90.6%) had a HOSE score ≥ 14, and no patient required revision surgery for skin problems after a median follow-up of 8.1 (range 1.2–13.1) years. Discussion The study is limited by its retrospective nature and by the fact that a number of other pre-operative, intra-operative, and postoperative variables can affect outcome. Conclusions Bipolar diathermy can be safely used for distal hypospadias repairs. It allowed careful control of intra-operative bleeding and also clear visualization of tissue planes. Complication rate overall compares favorably with the literature, and cosmetic results were satisfactory. Table . Results of hypospadias repairs using the bipolar diathermy technique All patients (n = 310) % Fully healed 273 88.1 Urethral fistula 18 6 Partial or total urethroplasty dehiscence 6 2 Complete dehiscence 7 2.2 Preputial fistula 2 0.6 Severe urethral meatal stenosis 2 0.6 Persistent chordee 2 0.6

Premature babies

Bowel dilatation is a common adaptive mechanism after intestinal resection. The symptomatic dilated dysmotile duodenum is difficult to manage, since conventional bowel tailoring and lengthening techniques are potentially hazardous because of the anatomy of the duodenal blood supply, the proximity to the pancreas, and the risk of injury to the common bile duct. A 2-month-old child with short bowel and a symptomatic massively dilated duodenum was treated with a Transverse Flap Duodenoplasty (TFD). The duodenum was opened longitudinally along its antimesenteric border preserving an intact strip of tissue overlying the pancreatic head. Three full thickness vascularized pedicle flaps were cut on both the anterior and posterior walls and were spirally rotated and sutured to create a uniform propulsive duodenum without diverticulae. Healing was complicated by a soft anastomotic duodeno-ileal stenosis that resolved after three elective balloon dilatations. Oral feeding established rapidly. The child is growing, does not vomit, and passes 1–2 semiformed motions daily. TFD is a safe and versatile technique that preserves all duodenal absorptive mucosa and that removes any risk to the pancreas, bile duct, and ampulla of Vater. Our experience, although limited, has been encouraging and leads us to suggest TFD for the management of the difficult symptomatic dysmotile dilated duodenum.

We report a child with post-surgical short bowel state who underwent bowel expansion followed by spiral intestinal lengthening and tailoring (SILT) at 10 months of age. Growth at 1-year follow-up is along the 15-25th centile on 82 % oral calories as normal diet and 18 % as parenteral nutrition, and he is passing 2-3 semisolid motions daily. SILT is a versatile technique for reconstructing dilated bowel towards improved propulsion and absorption, and has a role in the management of the short bowel state.

Introduction Spiral Intestinal Lengthening and Tailoring (SILT) offers a new opportunity for the surgical treatment of short bowel syndrome. SILT requires less manipulation on the mesentery than the Bianchi procedure and does not alter the orientation of the muscle fibers like serial transverse enteroplasty (STEP). This study reports the first SILT results in a surviving animal model. Material and Methods Vietnamese minipigs (n = 6) underwent interposition of a reversed intestinal segment to produce proximal small bowel dilation. Five weeks later the reversed segment was resected, and the wall of the dilated intestine was cut spirally at 45°–60° to its longitudinal axis. The bowel was lengthened longitudinally, and the spiral shaped intestinal wound was sutured. Five weeks later, the animals were explored, and the lengthened segments were measured. Haematoxylin and eosin, picrosirius, neuron specific enolase, S-100, C-kit, and immunohistochemistry were performed. Results Mean lengthening was 74.8% ± 29.5% and mean tailoring (lumen reduction) was 56.25% ± 18.8%. No instances of necrosis, perforation, suture break down, or peritonitis were observed in 6/6 animals. Four of six animals recovered uneventfully with viable lengthened segments. Statistical analysis showed no significant difference in length (p = 0,078) and width (p = 0,182) after 5 weeks. Two animals developed bowel obstruction due to narrowed lumen, adhesion, and strangulation after 14 and 24 days of surgery. In both animals the lumen was tailored by more than 70% to less than 1.5 cm diameter. The mucosa and the muscle layers in the operated segment had become hypertrophic, but the orientation of the circular and longitudinal muscle fibres remained normal after the SILT procedure. There were no signs of chronic ischemia or collagen accumulation after the SILT. The myenteric and submucosal plexuses and the Cajal cell network appeared normal. Conclusion The bowel remained viable macroscopically and microscopically after SILT, such that SILT may be an alternative or an addition to the present technical repertoire of intestinal lengthening. However the limitations of tailoring should be kept in mind.

Background: Around 70% of children with neurodisability (ND) present pharyngeal neuromuscular incoordination and severe gastroesophageal reflux disease (GORD). Methods: This is a pilot study with the Robotic-assisted minimally invasive total esophagogastric dissociation (TOGD). Results: We included 4 patients, 2 males and 2 females, with ND and severe GORD refractory to medical treatment. Conclusions: Pharmacological management of GORD is often unsuccessful and antireflux surgery is common, but it has a high failure rate with symptom recurrence, requiring re-do surgery. TOGD is a good option for these patients.

A normally positioned aesthetic umbilicus that is patient acceptable is important to the psychological well-being of the child through to adulthood. Following intra-abdominal placement of the extruded edematous bowel, in children born with gastroschisis, closure of the wide 1.5–3.5 cm diameter umbilical port can be achieved in the manner of a “hinged bottle top,” using the splayed cord that is attached to the left margin of the port. The technique uses the natural spontaneous fascial contracture that occurs during drying of the umbilical cord to achieve a midline aesthetic umbilicus and a scarless abdomen. Umbilical hernia has not been a problem. Complications have been minor with early periumbilical cellulitis requiring antibiotics and the occasional “soft” wide umbilicus coming to late surgery. Long-term follow-up of 32 patients, now >20 years, confirms the patient acceptability of a normally placed aesthetic umbilicus and a scarless abdomen.

The pediatric short bowel state is most often secondary to congenital anomalies and is a life-changing event that requires close cooperation between diverse medical specialities, the patient, and the family. It is best guided from a specialist center, working on a “hub and spoke” model that provides medical and nurse specialist support to referral hospitals. Long-term care, including home parenteral nutrition, is delivered by the family and the local carers, thereby retaining the “family unit” within their work, school, and social environment.

Bladder exstrophy and epispadias are severe congenital anomalies associated with an open bladder and urinary sphincter. Despite modern reconstruction, there is a significant incidence of residual or recurrent urinary incontinence that impacts on quality of life (QoL) and self-esteem, which in turn limits social interaction (Figure). The present study involved 14 patients, mainly from a Middle Eastern country, and reported the early findings with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique for both urinary and faecal control.Fourteen children, with a median age of 8.1 years, with poor quality of life and low self-esteem because of urinary incontinence and small polypoidal open bladders of 5-15 ml volume, mostly after bladder exstrophy surgery, were managed with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique keeping an intact anal sphincter. The retrorectal pulled-through colon was anastomosed to the posterior wall of the rectum just above the external sphincter complex, thereby avoiding any possible injury to the anal sphincter. All patients had a normal colon and a competent anal sphincter without lumbosacral spinal or nerve anomalies.Ten children had a 5- to 10-year follow-up, one child had a 15-year follow-up, and three others, that were also continent, were excluded because of a5-year follow-up. There were no postoperative complications, and all were dry and odour-free by day within 2-4 weeks of surgery. Two children still had minor urinary loss at night. There were no UTIs and renal function remained unimpaired. Eleven years after surgery, one child underwent excision of a pedunculated benign inflammatory polyp from the tip of the left ureter because of recurrent torsion and bleeding, there was no recurrence at the 2-year follow-up. None of the rectal or ureteric biopsies from any of the children showed metaplasia or neoplasia; however, in view of the potential long-term risks, all children were placed on a lifelong 'proctoscopy and biopsy' protocol.The ability to be dry and odour-free, and to wear normal clothing had a striking impact on QoL and psychological well-being of the children and their families. This was reflected in their positive overall approach, voluntary school attendance, and enthusiastic participation in communal events. All agreed that their improved genital appearance markedly contributed to their better body image and increased self-esteem.These significant benefits, at a crucial time in the child's life, outweigh the potential risk of long-term neoplasia. Therefore, the Heitz-Boyer-Hovelacque rectal bladder technique is recommended with long-term proctoscopic follow-up.

Short bowel syndrome is a multisystemic disorder that results from the loss of a significant amount of small bowel. The goal of treatment in these patients is to achieve complete enteral autonomy while minimizing complications. Our unit has 30 years of experience in the management of short gut patients. During the past decade, our results have improved significantly, especially in children with severe short bowel syndrome. This brief communication looks at the algorithm presently used in our unit. In this communication, the principles in management of short bowel syndrome in our unit are discussed. In addition, our algorithm is published for the first time. A brief summary of our results is provided. Twenty-seven children were enrolled from 2000 to 2009. In this cohort, two patients died because of significant liver disease: one after having two liver and bowel transplants. Overall, survival stands at 92%. All had autologous gastrointestinal reconstruction, and 19 patients underwent bowel lengthening (longitudinal intestinal lengthening and tailoring). The median residual length of bowel of this subgroup at first operation was 25 cm in those who had their gut measured. Two patients were lost to follow-up. Two patients remain on supplemental total parenteral nutrition (TPN), with an overall 91% of surviving patients off TPN at a median of 6 months after reconstruction. We believe this improvement is related to the development—over many years—of a structured pathway for managing these patients.

The treatment of short gut syndrome has evolved dramatically during the past decade. The combination of surgical techniques and medical management in the context of a multidisciplinary approach has improved the outcomes of these children. The authors describe in detail their technique of controlled tissue expansion of the bowel before lengthening procedures. Monitoring of the child and troubleshooting actions during the controlled tissue expansion program also are discussed.

Background Transanal anorectoplasty was developed through the 1980s by the senior author (AB) as an alternative approach to posterior sagittal anorectoplasty for the management of imperforate anus. This study evaluates this surgical approach and its longer-term results. Methods Case notes of all patients treated from 1984 to 2005 were reviewed. Operative procedures, colostomy requirement, complications, and long-term outcome were recorded. Patients were grouped according to the status of the pelvic floor, the location of the rectal fistula, and the sacral ratio. Results A total of 245 patients (175 male, 70 female) underwent transanal anorectoplasty. The perineum was well formed in 208 patients (85%), moderately formed in 15 (6%), and poorly formed in 22 (9%) patients. Two hundred three patients (82.8%) had a visible perineal or vulval fistula, 24 (9.8%) had a prostatic urethral fistula, whereas 18 (7.4%) had a bulbourethral fistula. Overall, 6 patients (2.5%) had wound infection or breakdown. As continence is age related, 32 patients were excluded from the study. Of the remaining 212 patients, 182 are continent with no soiling or only minimal staining. Thirty patients born with a poor perineum are incontinent. Conclusion Transanal anorectoplasty is a safe procedure with limited morbidity. It is sphincter sparing and permits accurate placement of the rectum with its internal sphincter within the anal canal. The anus lies accurately placed at the center of the external anal sphincter muscle complex. Given a well-performed surgical intervention, eventual continence relates to the original anatomy and neurology of the pelvic floor. Transanal anorectoplasty should be regarded as an alternative rather than as a substitute to posterior sagittal anorectoplasty for reconstruction of most forms of imperforate anus.

The gastrointestinal tract responds to significant mechanical or functional obstruction by dilatation and hypertrophy of the segment proximal to the obstruction. Excessive dilatation compromises motility, and absorption and is associated with considerable morbidity (intraluminal stasis, sepsis) such that bowel dilatation represents a major liability that predisposes the patient to intestinal failure. The dilated bowel proximal to an obstruction provides valuable autologous material for reconstruction with "tissue appropriate to the part." Bowel elongation and dilatation are integral to the natural intestinal adaptation response to loss of small bowel and can also be induced through a structured "Bowel Expansion" program. The additional absorptive tissue that is progressively generated is essential for reconstruction of the bowel (tailoring and lengthening), to restore gastrointestinal dynamics (effective propulsion and absorption), and to reduce morbidity (intraluminal stasis, sepsis). In enhancing the prospects for enteral autonomy, dilatation and elongation of the residual autologous bowel are crucial to long-term survival and good quality life, and represent a most welcome asset. This paper reviews the impact and management of bowel dilatation along the gastrointestinal tract.

Purpose We present our experience with traction-compression-closure (TCC) for exomphalos major (EM) to achieve a safe and embryologically correct midline supraumbilical aesthetic closure with preservation of the umbilicus. Methods Nineteen neonates with EM were paralyzed and ventilated. The abdominal domain was increased by upward cord traction to assist liver-bowel reduction by gravity and sac ligation, followed by circumferential elastic body binder compression. The supraumbilical abdominal wall anomaly cicatrized spontaneously or was closed surgically as a midline scar, with preservation of the umbilicus. Results Over 7 years (1998-2004), 19 patients with EM were treated by TCC, 18 of whom survived. The patients' median gestational age was 36 weeks (range, 24-40 weeks); their median birth weight was 2312 g (range, 890-3000 g). The median time to reduction was 4 days (range, 3-5 days), whereas that to full enteral feeds was 6 days (range, 4-6 days). Mechanical ventilation for 7 days (range, 6-8 days) was not associated with any morbidity, and the time to home discharge was 11 days (range, 8-12 days). Five patients did not require any surgery. There was no episode of sac rupture or infection. Conclusion Abdominal expansion by vertical cord traction followed by compression reduction (TCC) under muscle relaxation and ventilation is time well spent toward a safe and aesthetic midline abdominal wall closure without tension for EM.

Purpose Neurologically impaired children run a 12% to 45% risk of recurrent gastroesophageal reflux (GER) after fundoplication. Elimination of the reflux by "rescue" total esophagogastric dissociation (TEGD) encouraged us to use it also as a "primary" form of antireflux surgery in this group of patients. Methods Twenty-six (14 male, 12 female) patients underwent TEGD between 1994 and 2004, of which 16 were primary and 10 were rescue procedures for failed fundoplication. Results There was no operative mortality and postoperative complications were limited to one subphrenic collection, one esophagojejunal dehiscence, and one small bowel hernia beneath the jejunal Roux loop. Gastrostomy feeding was usually established by 3 to 5 days and the mean hospital stay was 10.2 days (range, 6-18 days). At follow-up of 7 months to 11 years, there was no recurrence of GER. Four late deaths were unrelated to the surgery. The children's nutritional status improved with the mean weight standard deviation score showing a statistically significant increase from −2.63 preoperatively to −0.96 postoperatively (Wilcoxon's signed rank P value ≤.005). Conclusions Total esophagogastric dissociation is a safe definitive solution for GER because it eliminates all risk of recurrent reflux. We therefore feel that TEGD can be used as a primary treatment of choice for severely neurologically impaired patients who are experiencing GER and are completely dependant on tube feeds.

The short bowel state is treatable, with acceptable long-term quality of life. Management during the first 6-12 months of life is critical but, presently, frequently compromises long-term survival and prospects. At first presentation, primary caregivers, working with specialists at designated intestinal failure centers, should develop a structured individual-oriented management plan. Preservation of venous access, "hepatosparing" parenteral nutrition, and avoidance of liver sepsis are crucial to survival. Early surgery should be limited to conservation of autologous bowel, even short bowel lengths having great potential, and to facilitating natural intestinal adaptation. Bowel expansion may be relevant prior to delayed bowel reconstruction with single or combined techniques that include bowel lengthening and/or tailoring, reversed segments, and colon interposition. Bowel transplantation, as yet not recommended for primary management, offers survival and opportunity to those with no prospect of autologous bowel autonomy or following failed autologous gastrointestinal reconstruction. This paper reviews current surgery for the short bowel state and concludes that it is presently appropriate before bowel transplantation to offer autologous gastrointestinal reconstruction, with its prospect of enteral autonomy with quality life. It emphasizes the need for an individual-oriented management plan, developed jointly at the time of first presentation between the primary caregivers and a designated multidisciplinary intestinal failure center, to enhance the prospects for enteral autonomy preferably on autologous bowel. Autologous gastrointestinal reconstruction is in its infancy and requires resources, commitment, and research from dedicated bowel reconstructive surgeons toward a better opportunity for the child and family with short bowel.

In severe short bowel syndrome, as a result of the natural adaptation, the bowel becomes overdilated, this interferes with the persitalsis and may lead to stasis, bacterial translocation and sepsis. At present two techniques are used to improve peristalsis. The Bianchi procedure is technically challenging, the Serial Transverse Enteroplasty (STEP) is easy however it results in an aphysiological ultrastructure altering the orientation of the muscle fibres. Our aim was to develop an easy technique, which does not alter intestinal muscular ultrastructure dramatically.The idea, Spiral Intestinal Lengthening and Tailoring (SILT), is based on a spiral shape incision of the intestine and retubularisation in a longer but narrower fashion. The feasibility and the effect on the muscular ultrastructure were tested on bowelsimulator and porcine intestine. The intramural microcirculation was checked with intravital microscopy. The outcome was assessed on minipigs (n = 6) than clinical application was commenced.SILT was feasible, did not change the orientation of muscle fibres significantly, did not compromised microcirculation, no surgical complication was noted when tailoring did not exceed 75%. The first clinical application was successful.SILT is a safe and easy technique and not altering the intestinal musculature significantly.Bevezetés: Súlyos rövidbél-szindrómában, a természetes adaptáció következtében, a megmaradt vékonybél annyira kitágul, hogy a béltartalmat nem képes továbbítani, ez stasishoz, bacterialis translocatióhoz, majd sepsishez vezethet. A passzázs javítására jelenleg alkalmazott műtétek közül a Bianchi-műtét igen bonyolult, a serial transverse enteroplasty (STEP) lényegesen egyszerűbb, de az izomrostok orientációját drámaian megváltoztatva afiziológiás állapotot teremt. Célunk egy új, egyszerű és kevéssé kockázatos bélhosszabbító műtéti technika kifejlesztése volt, amely nem változtatja meg jelentősen a bél mikroszkópos anatómiáját. Anyag és módszer: Az ötlet a bél spirális vonal mentén történő incisióján és kisebb kaliberen történő retubularisatióján alapul. A műtétet spiral intestinal lengthening and tailoringnek (SILT) neveztük el. Elsőként a műtét kivitelezhetőségét és az izomrostok orientációjára gyakorolt hatását vizsgáltuk bélszimulátoron és sertésbélen. Az intramuralis keringésváltozásokat in vivo mikroszkópia segítségével elemeztük, majd műtétet túlélő sertésmodellen (n = 6) teszteltük, csak ezek után került sor a klinikai alkalmazásra. Eredmények: A SILT könnyen kivitelezhetőnek bizonyult, az izomrostok orientációja nem változott lényegesen. A műtét nem okozott kritikus változást a bélfal keringésében, és amennyiben a lumen szűkítése nem haladta meg a 75%-ot, nem észleltünk sebészi komplikációt vagy krónikus szöveti ischaemiát sem. Az első klinikai alkalmazás is sikeres volt. Következtetés: A SILT-műtét nem változtatja meg a bél izomstruktúráját, biztonságos és ajánlható módszer a bélhosszabbításra.

A 6-year-old boy developed respiratory distress, metabolic acidosis, severe esophageal and gastric burns, and a coagulopathy after ingestion of an unknown volume of methyl ethyl ketone peroxide (MEKP) in dimethyl phthalate. He was discharged from the pediatric intensive care unit 19 days postingestion but subsequently developed a stricture of the gastroesophageal junction and complete fibrosis of the middle third of the stomach, necessitating gastric resection and reconstruction. He was discharged 93 days postingestion on a program of dilation for the residual esophageal stricture. MEKP acts by initiating lipid peroxidation via free radical production that results in cellular dysfunction and death. Acetylcysteine, a glutathione precursor and possible free radical scavenger, may be of use in severe MEKP poisoning. This case demonstrates the severe effects that some industrial chemicals can have both systemically and locally at the point of contact with the gastrointestinal tract, as well as the long-term management required to ensure good quality of life.

Following the advent of laparoscopic surgery, cosmesis has become an important factor in surgical decision making. The circumumbilical incision combines the advantages of an open approach with an aesthetically pleasing scar on the abdomen. The aim of this paper is to examine the results of this incision in neonatal laparotomy. All neonates who underwent a supraumbilical circumferential skin incision for an exploratory laparotomy in the period 1997–2007 were reviewed. Gestational age, operative procedure, conversions to standard laparotomy, complications and follow-up were recorded. A total of 55 neonates with a gestational age ranging from 28 to 42 weeks had 57 operative procedures. The indications were: nonrotation of midgut in 18; intestinal atresia in 18; necrotizing enterocolitis/spontaneous perforation in 10; meconium ileus in 5; intestinal duplication in 2; patent vitellointestinal duct (VID) in 2. No conversion to a standard transverse incision was necessary in any case. However, an omega extension was made in four patients. The complications encountered include wound infection in one; caecal perforation in one and incisional hernia in two cases. Subsequent follow-up revealed that all incisions had healed and the scars were almost imperceptible as affirmed by parental satisfaction during outpatient clinic consultation. The circumumbilical approach is a safe, flexible and easily reproducible approach providing adequate exposure for most abdominal surgeries in the neonate. The low complication rate and pleasing aesthetic outcome are much appreciated by parents and operators alike.

Reconstruction and exteriorization of the "high" vagina in girls with congenital adrenal hyperplasia (CAH) is technically challenging. Although current surgical thinking favors correction in infancy the evidence from several long-term studies indicates that many patients will require further surgery in adolescence or adult life. Deferred vaginoplasty has been advocated because the procedure may be technically easier as a consequence of changes in the characteristics of the genital tissues and vaginal anatomy. The role of urogenital mobilization is as yet undetermined but carries promise for the short, high vagina. We describe a patient where the radiological and clinical findings support the argument for deferred vaginoplasty.

Three children with tracheomalacia had tracheal reinforcement with free three-quarter circumference ring grafts of autologous cartilage taken from the costal margin. A low cervical manubrium-splitting approach gave excellent access to the anterior mediastinum and the intrathoracic trachea in two children. The first child, a neonate with oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF), had 11 grafts to support the whole of the trachea from the cricoid to the carina and never required a tracheostomy. For the first 5 years she had frequent pneumonic episodes and on one occasion bilateral pneumothoraces. These episodes and radiographic lung hyperinflation, attributed to distal bronchomalacia, have reduced spontaneously in frequency and severity. At 9 years of age she has a well-supported trachea with palpable cartilage rings in the cervical segment. The trachea has grown to approximately 75% of expected normal size for her age. Another child with tracheomalacia related to innominate-artery compression and who presented with 'dying episodes' was completely relieved and resumed a normal life without a tracheostomy following insertion of four grafts to the intrathoracic trachea. He remains well and symptom-free 8 months postoperatively. A third child had cartilage-graft reinforcement of the lower cervical trachea, including the tracheostomy site, to achieve tracheostomy closure at 16 months of age. Five years later he continues to have a well-supported trachea showing acceptable growth. However, he has ongoing evidence of tracheo-bronchomalacia presenting as expiratory wheezing, lung hyperinflation, and pneumonic episodes that are diminishing spontaneously with growth. Our experience, limited to three children, recommends primary tracheal reinforcement with autologous free costal-cartilage grafts for tracheomalacia in the neonate and young infant. This procedure and the anterior mediastinal approach are well-tolerated, providing instant tracheal support, removing the need for a tracheostomy, and allowing the child's rapid return to the family. Long-term follow-up, presently 9 and 5 years in two children originally presenting with OA and TOF, indicates adequate tracheal growth and an aesthetically acceptable appearance. It is relevant to prognosis that relief of the life-threatening tracheal component exposed the full extent of the bronchial cartilaginous weakness, which has significantly detracted from the quality of life for these two children with OA and TOF-related tracheomalacia.

Introduction : Traditionally Hirschsprung's disease has been treated by a three-stage procedure. This approach has been associated with stoma problems, three episodes of hospitalization, and a theoretical disadvantage, because of the delayed passage of feces via the rectum, in controlling defecation. The aim of this study was to assess the results of one-stage neonatal pull-through for the treatment of Hirschsprung's disease. Methods : This was a 10-year retrospective review of all patients treated with a neonatal pull-through for Hirschsprung's disease. Information was obtained from the patients' records. All descriptive data are expressed as mean ± standard deviation. Results : Fifty-one patients were treated in the 10-year period studied. The mean gestational age was 39.6 ± 1.7 weeks, weight was 3.3 ± 0.54 kg, with 76% being boys. The surgery was performed at 10.3 ± 5.8 days of age. A Swenson procedure was carried out in 38 patients and a Soave in 13. The extent of the disease was rectosigmoid in 39, and the remaining were sigmoid, descending colon, and total colonic. The mean length of the surgery was 181 ± 40 minutes. Blood transfusions were required in 13 patients with a mean volume of 55 mL given. The complications observed were: an anastomotic leak in two cases, requiring laparotomy and colostomy (both in patients with Down's syndrome), and wound infection in three cases. There were two delayed deaths both associated with other congenital anomalies. In total, three patients required further surgery; two had colostomies, one requiring a redo pull-through and one had an ileostomy for enterocolitis. The total length of hospital stay was 20.4 ± 11.2 days. Mean follow-up was 3.73 ± 2.9 years. In 21 patients the follow-up was greater than 4 years and in this group none were totally incontinent. Four patients complained of soiling at least once a week, and 17 had normal bowel control. Conclusion : Hirschsprung's disease can be successfully treated in the neonatal period with a one-stage pull-through. The short- and long-term results are as good as those with the three-stage procedure, with the child usually benefitting by not having a stoma and a shorter hospital stay.

Aphallia is best managed by early penile and urethral reconstruction. We describe parascrotal flap phallo-urethroplasty, with a 6-year follow-up and a psychological profile, that constructs a ‘penis’ of realistic shape, colour, and position, and having skin sensation, with an integral urethra that allows a free concentrated urinary stream and satisfies the psychological needs of family and child during childhood. The lack of corpora cavernosa only becomes significant at puberty.

We report our series of selected patients with complete incontinence in whom the appendix was divided and utilized for creating two continent catheterizable stomas. All patients were treated for urinary and fecal incontinence by split appendix technique. The appendix was divided into two different parts preserving adequate perfusion and used for creating an appendicocecostomy (ACE) and an appendicovesicostomy at the same time. After a clinical and radiological follow up, our patients referred a good acceptance and an easily management of the stomas in order to stay dry for all day from urine and feces with improving of their quality of life. The combination of ACE and Mitrofanoff principle have revolutionized the management of urinary and fecal incontinence in patients who are unable to utilize their urethra to keep themselves dry.

BACKGROUND AND AIMS: Short bowel syndrome (SBS) describes the malabsorptive state seen following extensive bowel resection. Management aims to optimise nutritional intake, promote intestinal adaptation, and prevent the development of complications. Surgical options such as autologous gastrointestinal reconstruction (AGIR) attempt to restore enteral autonomy to the patient. Although the literature focuses on the objective measurements of success following AGIR techniques, subjective assessment of its effect on the quality of life (QoL) should also be sought. Because children with SBS are reliant on caregivers, caregivers' satisfaction with surgery and their perception of the effect of AGIR on their daily lives should be assessed. This is the first report of caregiver satisfaction following AGIR in children. METHODS: All children with SBS who had AGIR between January 1999 and June 2010 were identified. A questionnaire was developed. Caregivers were asked to complete the questionnaire to rate their satisfaction with surgery and the pre- and postoperative daily care, medical needs, and subjective interpretation of QoL for their child. Data were analysed using SPSS version 18, using the Friedman nonparametric test and 2-way analysis of variance by rank. Statistical significance was set at ≤0.05. RESULTS: Thirty-two children were identified. Seven assessments were done outside our centre (5 outside the UK) and were not included in the study. One patient moved to another city and was not contacted. Two children died. Twenty patients were contactable and were included in the present study. Parents were satisfied with the AGIR and saw improvements in their child's physical condition, bowel habit, and subjective interpretation of QoL postoperatively. Parents perceived significant improvements in the ease of caring and in aspects of their relationship with their child, including the quality of time spent, their enjoyment, and the level of their frustration with them. CONCLUSIONS: The present study found that AGIR improved the physical well-being of the child and gave the impression of improvements on specific QoL aspects for both the child and the parents.

Short bowel syndrome poses a great challenge to pediatric teams. Several innovative techniques in the management of total parenteral nutrition (TPN) and bowel reconstructive surgery have improved the outcomes of these children. The authors present their experience during the last decade as a specialist unit using improved techniques and multidisciplinary approaches in the management of this condition.All of the children presenting with short bowel syndrome between 2000 and 2009 were identified. Diagnosis, length of residual gut, age at definitive surgery, length of gut prelengthening, length of gut postlengthening, TPN status, and survival were recorded. Median values were calculated.Twenty-seven children were identified (14 boys, 13 girls). Overall survival was 92%. Two children died. Nineteen children required bowel lengthening and 8 children had simple bowel reconstruction while on our protocol. Overall median age at definitive surgery was 12 months. Overall median residual gut length for these was 35.5 cm, whereas the median residual gut length for patients undergoing bowel lengthening was 25 cm. Postbowel lengthening, the median gut length was 90 cm. TPN data were unavailable for 2 patients. Overall, excluding the 2 patients who died and the 2 we have no TPN data on, of 23 patients, 21 (91%) are now off TPN.Our series shows improved results not only with survival but also in the number of patients that are off TPN. Multidisciplinary approach consisting of both medical and surgical expertise is necessary in the management of these patients. The authors advocate centralisation of short gut services to experienced centers with multidisciplinary expertise.

The effect of testicular ischaemia on fertility as assessed by paternity rate was studied in seven groups of male Sprague-Dawley pubertal rats. Transient unilateral occlusion of the testicular artery and venous outflow was performed for 30, 60, 90, and 120 min using non-crushing microvascular clamps in groups 1–4 (n = 6). Group 5 (n = 12, Fowler-Stephens) had division of both the testicular artery and vein, whereas group 6 (n = 12, Refluo) had division of the testicular artery only. Group 7 (n = 6) consisted of controls upon whom a sham operation was performed. Testicular biopsy 6 weeks after transient testicular ischaemia of 30, 60, and 90 min showed tubular atrophy in 20%–33% of the testes. Ischaemia of 120 min led to tubular atrophy in 84% of testes. Division of only the testicular artery caused tubular atrophy in 58% of testes. Division of both testicular artery and venous outflow was associated with total tubular destruction and necrosis in 78% of testes and 22% had variable tubular atrophy. A paternity rate of 50%–80% was noted in groups 1–4 following transient unilateral testicular ischaemia and contralateral vasectomy. This compared well with the expected 75% paternity rate for a control rat population. Chronic ischaemia (group 5) together with testicular venous interruption was associated with observably lower fertility and a paternity rate of 18%. However, when venous outflow was preserved (group 6), chronic ischaemia after testicular artery division did not affect the paternity rate (50%) significantly.

Background The role of the transcrotal approach to the undescended testis remains controversial despite its increasing popularity. The authors update their previous published series and review the literature on this subject, aiming to delineate the value of this technique. Methods The authors performed a retrospective review of the transcrotal primary orchidopexy carried out to treat palpable undescended testis at Royal Manchester Children's Hospital between 1993 and 2005. A structured review of literature published since the proposal of this technique was also performed. Results 122 procedures were included. The transcrotal approach was successfully completed in 119 (97.5%). Additional groin incision was needed in three (2.5%) to further mobilize the spermatic cord. No immediate complications were recorded and 8.4% required a reoperative procedure. On review of the literature, a total of 16 articles were discovered spanning 1695 transcrotal procedures, including the previously published authors' experience. On combining the data, the transcrotal approach required an additional groin incision in 4.4% of cases, 1.6% experienced immediate and/or early complications, and the overall recurrence rate was 2.0%. Conclusions Transcrotal orchidopexy for the treatment of palpable undescended testes is a safe procedure with a long-term success rate comparable to the two-incision approach.

Uro-genital bleeding in pre-menarcheal girls always raises concerns regarding child abuse. There are serious social issues involved; however, before labeling it as sexual abuse, all efforts must be made to rule out an organic pathology. We recently encountered two young girls of Afro-Caribbean origin who presented with vaginal bleeding with concerns from referring physicians about child/sexual abuse. On examination both had a rare condition of urethral prolapse. One of them underwent four-quadrant excision and the other was treated conservatively with urethral catherization.

This study was designed to assess the natural history of subclinical varicocele in the pediatric age group.We conducted a school screening for varicocele in 2,107 children 10 to 16 years old, performing clinical examination and venous Doppler study. A total of 354 boys (16.8%) without clinically detectable varicocele had venous testicular reflux during a Valsalva maneuver. Of these children 36 (a 10% sample) were selected randomly and followed annually for 4 years, with assessment of testicular volume (ultrasound values), degree of varicocele according to the Dubin and Amelar classification, and Doppler findings according to the Hirsh testicular Doppler classification. The control group consisted of 20 children selected randomly from the same population, who did not have a varicocele and had a normal Doppler study. Fisher's exact test was used with a significance value at p0.05.Mean age of the 36 children at the first assessment was 12.8 years (SD 1.7). Two boys (5.5%) had spontaneous resolution of testicular venous reflux within 2 years, and in 24 (67%) the subclinical varicocele did not change. The remaining 10 children (28%) had a clinically detectable varicocele, which was grade I in 1 patient, grade II in 7 and grade III in 2. Of these patients 1 had associated left testicular hypoplasia greater than 20%. During the 4-year period there were no clinically detectable varicoceles in the control group (p = 0.01).The proportion of children with subclinical varicocele progressing to a clinically detectable form of the condition was 28% (95% CI 14 to 45) during a 4-year period. We suggest that children with subclinical varicocele require long-term followup.

Background/Purpose Neurologically impaired (NI) children have an increased incidence of gastroesophageal reflux and many will require surgery. Methods The case notes of 50 NI children who underwent total oesophagogastric dissociation (TOGD) were reviewed. Thirty-four were done as a primary procedure, and 16 were rescues for failed fundoplications. Results There was no operative mortality. Morbidity consisted of 1 subphrenic collection, 1 oesophagojejunal dehiscence and 2 stenoses that responded to dilatation, and 2 bowel obstructions. In 1 case, partial gastric resection was needed because of transhiatal herniation of stomach. Gastrostomy feeding was established by 3 to 5 days. The mean hospital stay was 10.9 days. At 4 months to11 years of follow-up, there was no recurrence of reflux. Children who could swallow enjoyed oral feeds. Their general health and weight SD scores improved. Food aspiration, chest infections, and hospitalizations were reduced, with an improvement in quality of life. There were 5 late deaths in the "primary" and 7 in the "rescue" group from deterioration in their original condition. Conclusion Total oesophagogastric dissociation is a safe and versatile procedure without immediate mortality and limited surgery-related morbidity. Review of our practice suggests TOGD should be considered as a primary procedure in severely NI children with gastroesophageal reflux and significant oropharyngeal incoordination and dependence on enteral tube feeding. Rescue TOGD carries a greater morbidity because of previous surgery with consequent difficult dissection, poor oesophageal tissue, and higher incidence of vagal nerve injury.

A gastrostomy is often essential to deliver adequate and safe nutrition. Various types are now available such that the technique can be tailored to the specific needs of the patient. This paper explores the non-refluxing gastrostomy for long-term intermittent gastrostomy feeding, avoiding the need for a permanent indwelling appliance. A full-thickness vascularized flap based on the right gastroepiploic vessels is raised from the greater curve of the stomach. The proximal half of the tubularized flap is buried in a submucosal tunnel and the free distal end is brought to the skin surface as a catheterizable stoma. Fifteen children with varied mental and physical disabilities formed the cohort of the study. There were 3 stomal stenoses and 3 mild mucosal eversions requiring minor surgical adjustments. One child had a wound dehiscence 10 days postoperatively. Once the stoma had healed, the majority fed by intermittent catheterization and bolus feeds at conventional feed times during the day. Intermittent catheterization was painless and easy and was well accepted by caregivers and patients. Perhaps the most important advantages were the increased patient and caregiver confidence and independence, as well as the reduction in anxiety and hospital attendance.

Hirschsprung’s disease (HD) in Down’s syndrome (DS) patients is stated to have a worse outcome than HD alone. In our study we reviewed the immediate and long-term outcomes of these children and questioned whether DS should influence the operative management. Data were collected on all children with HD (including total colonic aganglionosis), between January 1990 and December 2000. They were divided into two groups based on the presence or absence of Trisomy 21 and compared retrospectively. In this time period we treated 173 children with HD; 17 of these had DS. Both the groups were comparable in their mean gestational age, birth weight and presentation except that the DS group had a significantly higher overall incidence of pre and/or postoperative enterocolitis. A tota1 of 164 children underwent a Swenson pull-through and 9 had a Soave’s procedure. Follow-up ranged from 1 to 10 years. Continence assessed using the Wingspread scoring system in children over the age of 4 years showed no significant difference. Although children with both HD and DS are predisposed to complications and required a more cautious management, long-term outcome in terms of continence was not significantly worse than in HD alone. Thus the co-existence of DS should not influence the decision to offer these children and their parents the choice of definitive repair.