45 years' experience with early childhood anatomical technique of feminising genitoplasty for 46 XX Congenital Adrenal Hyperplasia -observations of vaginal introital anatomy and its relationship to the perineal body.

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.

Total Esophagogastric and Cologastric Dissociation in Neurologically Normal Children: Systematic Review.

Total esophagogastric dissociation (TEGD) was first described by Bianchi as a definitive procedure for gastroesophageal reflux disease (GERD) in neurologically impaired children. In the last 20 years, different centers extended the indication to neurologically normal (NN) patients with GERD associated with congenital or acquired esophageal anomalies. The aim of this paper is to analyze the role of TEGD in this cluster of patients. A PubMed and Google Scholar search was conducted. All cases of NN children who underwent TEGD for GERD were collected. Patient characteristics and outcomes were analyzed. Complications were classified according to Clavien-Dindo classification. Forty-eight children were identified. In 56.25%, TEGD was the first anti-reflux procedure, while in 43.75% it was performed after failed fundoplications. Mean follow-up was 5.5 years. Mortality related to surgery was 2.08%. All of the survivors improved their condition, with resolution of GERD and weight gain. In addition, 50% of children weaned off enteral nutrition, with 14.6% having their gastrostomy removed, while 41.67% maintained partial enteral supplementation. Respiratory symptoms almost disappeared in 54.17% of patients. This review suggests that TEGD can also be considered for NN children where conventional methods seem insufficient to control reflux and preserve pulmonary function. Nevertheless, long-term follow-up is still required.

Minimizing Enterostomy Complication in Neonates, Lessons Learnt from Three European Tertiary Centres.

INTRODUCTION: Stoma formation in neonates is often a life-saving procedure across a variety of conditions but is still associated with significant morbidity. Tube stoma technique was originally described for short bowel patients, but in selected cases of neonates this approach could prevent the incidence of stoma-related complications. The aim of the study was to evaluate the safety and utility of tube stomas as an alternative to conventional enterostomy in the neonatal population. MATERIAL AND METHODS: A retrospective multicentre analysis of neonates undergoing emergency laparotomy and tube stoma formation between 2005 and 2017 was performed. Tube stoma complications were analysed. The investigation focused on stricture, skin lesion, enteric fistula and prolapse. RESULTS: Thirty-seven neonates underwent tube stoma fashioning during the study period. Tube-stoma complications were limited to three patients (8.1%), with two children (5.4%) requiring additional stoma surgery during the first 30 days because of an enterocutaneous fistula, and one child (2.7%) for bowel stenosis. CONCLUSIONS: In select neonates, such as those with proximal enteric stomas, the tube stoma avoids some of the commonly encountered complications (prolapse, skin excoriation). Further prospective studies are needed to validate these findings in order for us to recommend this technique as superior.

Modified Oesophago-Gastric Dissociation (M-OGD) - a technical modification.

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.

Total Oesophagogastric Dissociation in Neurologically Impaired Children: 18 Years' Experience and Long-term Follow-up.

OBJECTIVES: Total oesophagogastric dissociation (TOGD) is an alternative antireflux surgery for neurologically impaired children because of a 16% to 38% fundoplication failure rate. This study evaluates TOGD's feasibility and its long-term efficacy both as a Primary and as a "Rescue" procedure after failed fundoplication. METHODS: Thirty patients (18 boys) who underwent TOGD between 2000 and 2018 in 2 Italian Centres were retrospectively reviewed. Twenty-three were Primary procedures and 7 were "Rescue" ones. Inclusion criteria were severe neurodisability, intractable gastroesophageal reflux, and dysphagia. RESULTS: Preoperatively, all children had regurgitation, vomiting or retching, and 93% had unsafe swallowing and aspiration, with recurrent chest infections/aspiration pneumonia. Median relative weight was 77% (48%--118%). All patients were taking antireflux medication before surgery. Median age at TOGD was 6.48 years (0.69--22.18). Median follow-up was 3.5 years (0.6-17.7). No recurrence of gastroesophageal reflux (GER) and vomiting was recorded. The number of chest infections and length of hospital stay showed a significative decrease (P value <0.0001 for both), whereas median relative weight reached 101% (P value 0.002). Parents'/caregivers' perception of outcome showed a significative improvement. Six patients (20%) experienced early complications and 3 required surgical intervention. Three late complications (10%) also required surgery. There was no surgery-related mortality. CONCLUSION: TOGD is an effective procedure with an acceptably low complication rate for children with severe neurological impairment and is followed by a major improvement in general health and quality of life for children and families. There was no substantial difference in outcome between Primary and "Rescue" procedures.

Transverse flap duodenoplasty (TFD): a new technique in autologous bowel reconstructive surgery.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Robotic-Assisted Minimally Invasive Total Esophagogastric Dissociation for Children with Severe Neurodisability.

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.

Gastric transposition as a valid surgical option for esophageal replacement in pediatric patients: experience from three Italian medical centers.

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).

[New technique for intestinal lengthening -- from the idea to the clinical application]. / Új bélhosszabbító mutéti technika -- az ötlettol a klinikumig.

INTRODUCTION: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSION: SILT is a safe and easy technique and not altering the intestinal musculature significantly.

"Spiral intestinal lenghtening and tailoring (SILT)" for a child with severely short bowel.

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.

The parascrotal flap phallo-urethroplasty for aphallia reconstruction in childhood: report of a new technique.

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.

Spiral intestinal lengthening and tailoring - first in vivo study.

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.

Caregiver evaluation and satisfaction with autologous bowel reconstruction in children with short bowel syndrome.

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.

Intestinal rehabilitation and bowel reconstructive surgery: improved outcomes in children with short bowel syndrome.

BACKGROUND AND AIM: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Transanoproctoplasty: a 21-year review.

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.

20 years of transcrotal orchidopexy for undescended testis: results and outcomes.

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.

Versatility of the circumumbilical incision in neonatal surgery.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

An insight into vaginal surgery in a severely masculinized CAH patient.

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.