Adrian Bianchi

• Manchester University NHS Foundation Trust

Objectives: Total Oesophago-gastric Dissociation (TOGD) is an alternative antireflux surgery for neurologically impaired children because of a 16%-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: 30 patients (18...

Premature babies <30 weeks’ gestation with surgical necrotizing enterocolitis (SNEC) and presenting late with bowel perforation, septicaemia, bone marrow depression, and multiorgan failure, carry 30–50% mortality even following ‘last-ditch surgery’ guided by Bell’s criteria, that were designed for staging the disease and not for timing of 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...

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...

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 “hing...

Almost as many different thoracotomy incisions have been described as the number of esophageal repairs that have been proposed. For much of the history of EA repair, the only question in making these incisions was how to obtain the best exposure without considering the functional or cosmetic consequences. While exposure is of major importance, more...

Even for the otherwise normal child, clinically significant gastro-oesophageal reflux may be a cause of major morbidity and possible mortality and effective treatment difficult to achieve. Despite heavy medical management, the patient’s quality of life and indeed that of his family or caregivers is often severely reduced and an outwardly effective...

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 an...

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 cr...

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...

Background: 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). Th...

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)...

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...

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 satis...

Aim: Spiral Intestinal Lengthening and Tailoring (SILT) invented by our team is a new technique that offers minimal mesenteric handling and a more physiological result compared to the STEP procedure. Its feasibility has been tested in animal models and now we report the first successful human application in extreme short bowel syndrome. Materials...

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 Entero...

‘Caudal duplication syndrome’ was coined to describe the apparent duplication of organs derived from the hindgut, the neural tube and the adjacent mesoderm. Review of the anatomy suggests that the word ‘duplication’ may be a misnomer. This paper describes the management of 2 girls with caudal duplication syndrome who underwent multistage reconstruc...

Female epispadias without bladder exstrophy is an extremely rare anomaly occurring in 1:480.000 girls. It presents typical features and can be diagnosed immediately at birth. Early surgical reconstruction of the bladder neck, urethra, and external genitalia within the physiological phase for the development for continence, is relevant towards estab...

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 impr...

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 pati...

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 multidiscip...

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 p...

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. Case notes of all patients treated from 1984 to 2005 were reviewed. Operative procedures,...

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 liabilit...

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. The authors performed a retrospective review of the transcrotal primary orchidopexy car...

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 unde...

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 vaginoplas...

Self-esteem can play an important role in satisfaction with body appearance. The present pilot study was designed to examine the relationship between self-esteem and satisfaction with penis appearance in adolescent males with bladder exstrophy and/or epispadisas.Material and Methods Nine adolescent males aged 11-16 with bladder exstrophy or primary...

Self-esteem can play an important role in satisfaction with body appearance. The present pilot study was designed to examine the relationship between self-esteem and satisfaction with penile appearance in adolescent males with bladder exstrophy and/or epispadisas.Material and Methods Nine adolescent males aged 11-16 with bladder exstrophy or primar...

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 d...

We assessed the results from a single exstrophy center of salvage continence surgery after failed staged reconstruction for bladder exstrophy. A total of 32 patients with bladder exstrophy had undergone salvage continence procedures. Indications for surgery included incontinence due to poor bladder capacity or failed bladder neck repair, and upper...

Various options are available for the surgical treatment of meconium ileus (MI). This paper examines the use of resection and primary anastomosis as the favoured option for the treatment of complicated meconium ileus. This was a retrospective study. All patients (13 children) with MI treated with primary resection and anastomosis (RA) in the 10-yea...

Parenteral nutrition-dependent patients with short bowel can realistically hope for a return to enteral autonomy on their own bowel, and with a full good quality of life. Autologous bowel reconstruction (including expansion, lengthening and reversed segments) enhances the natural adaptive process towards increased absorption.

Since the first report by Bianchi in 1997, TOGD has been found to be safe and effective in patients with gastro-oesophageal reflux (GOR) with neurological impairment. This paper explores the versatility of total gastric dissociation (TGD) in difficult clinical situations. The medical records of 11 neurologically normal children treated with TGD and...

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. Nineteen neonates with EM were paralyzed and ventilated. The abdominal domain was increased by upward cord traction to assist liver-bow...

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. Twenty-six (14 male, 12 female) patients underwent T...

Traditionally, a stoma is established after resection of perforated or necrotic intestine for isolated intestinal perforation (IIP) and necrotizing enterocolitis (NEC). We compared the outcome of resection and anastomosis (RA) with stoma formation (RS). Sixty-eight neonates undergoing laparotomy for IIP (n = 20), NEC (n = 43), and indeterminate cau...

Clinically significant gastro-oesophageal reflux (GOR) following oesophageal atresia and tracheo-oesophageal fistula (OA/TOF) repair is commonly considered normal sequela after repair. A retrospective review of patients operated on by two consultants was undertaken. All patients underwent oesophageal tailoring and augmentation for reconstruction of...

Neurologically impaired (NI) children have an increased incidence of gastroesophageal reflux and many will require surgery. 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. There was no operative mort...

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...

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), betwee...

Miss LS was born with jejunal atresia. She had lost most of hersmall bowel leaving her with 38 cm of massively dilated prox-imal jejunum distal to the duodenal-jejunal flexure. Sheretained the entire colon but lost her ileocaecal valve. Failureof conventional surgery to establish enteral function, initiallywith a jejunostomy followed by a jeju-nocol...

Gastroesophageal reflux (GER) is a common condition affecting all age groups. It occurs in isolation but is also associated with other conditions such as hiatus hernia, esophageal atresia, and congenital diaphragmatic hernia, which themselves require surgery. GER is not infrequent in children suffering from mental subnormality. In infants and young...

In 1998 Bianchi and Dickson published their proposal for elective delayed midgut reduction without anaesthesia (EDMR-No GA). The study has been prospectively extended to develop "selection and conversion criteria" to ensure safe application of the technique. In a prospective study from 1993 to date, EDMR-No GA was considered as first-line postnatal...

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A Bianchi, SM Bianchi ODA News Review 4:14-16 2002

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 g...

Parenteral nutrition represents standard therapy for children with short bowel syndrome and other causes of intestinal failure. Most infants with short bowel syndrome eventually wean from parenteral nutrition, and most of those who do not wean tolerate parenteral nutrition for protracted periods. However, a subset of children with intestinal failur...

To evaluate the effect of a delay in closure of the abdominal wall (AWC) on outcome in the management of gastroschisis, a retrospective analysis of 91 babies admitted over a 7-year period (1992-1998) to a single neonatal surgical unit with a diagnosis of gastroschisis was carried out. Antenatal diagnosis was made in 89 (98%) cases. Surgical interve...

The aim of this study was to evaluate the contemporary outcome in the management of gastroschisis. A retrospective analysis was conducted of 91 babies admitted over a 7-year period to a single neonatal surgical unit with a diagnosis of gastroschisis. An antenatal diagnosis was made in 89 (98%) cases. Surgical intervention occurred in 90 babies, at...

Tracheomalacia occurs as a primary developmental defect or may be secondary to vascular compression. It is common in association with esophageal atresia and tracheoesophageal fistula. Collapse of the weak trachea is a cause of recurrent respiratory symptoms but may be severe and life threatening. Between 1978 and 1999 at Sheffield Children's Hospit...

Over a 16-year period, 20 neonates and infants with short-bowel syndrome underwent longitudinal intestinal lengthening and tailoring because of a dysfunctional dilated jejunum. There was no operative mortality, and morbidity was limited to 2 hemiloop anastomotic stenoses and 1 spontaneously resolving air and bile leak. Long-term survival was 45%. S...

Female omphalopagus twins underwent laparotomy on the second day of life after an antenatally diagnosed high jejunal bowel obstruction. Bowel resection and choldocho-enterostomies were performed. Despite recovery from laparotomy, the development of severe necrotizing enterocolitis (NEC) in one twin led to rapid deterioration and the death of both i...

A 21-month-old child with a previously repaired left congenital diaphragmatic hernia underwent a 360 degrees 'loose-wrap' Nissen fundoplication for gastroesophageal reflux. Failure to replace the dislodged nasogastric tube on the 2nd night led to severe gas bloat and total gastric infarction. A 30-cm retrocolic, N-shaped, isoperistaltic jejunal pou...

Lateral thoracotomy is a relatively common procedure in the neonate. Early reports on the incidence of shoulder deformity, scoliosis, and winged scapula rapidly led to a muscle-sparing approach. Scar cosmesis, however, received less attention. Better education and increasing sophistication have led to a higher aesthetic expectation by the public. S...

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...

In a pilot study of 14 children, born when the authors were on a 1:5 "on take" for neonatal referrals, a policy evolved of elective delayed midgut reduction without anaesthesia or sedation in the incubator on the neonatal surgical unit. There was no other form of selection, and it was fortunate that the authors did not encountered any adverse crite...

Interest in primary one-stage reconstruction for Hirschsprung's disease has been increasing steadily because of the obvious clinical quality issues and resource benefits. This report describes one surgeon's 13-year experience with 52 children who had proven Hirschsprung's disease. The patients were managed in a neonatal surgical unit by a one-stage...

The successful management of faecal incontinence can dramatically improve the quality of life of affected children. The introduction of the non-refluxing, catheterisable appendico-caecostomy provides the opportunity to treat previously resistant patients. Over a 6-year period, 29 children had a Malone antegrade continent stoma for enema administrat...

A 1.8 kg chromosomally normal child was suspected antenatally to have a cloacal anomaly and oligohydramnios. Both parents had a chromosome 9 inversion (inv [9] [p11 q13]). Postnatal study showed a high confluence of the urinary, genital and intestinal tracts. Because of prematurity, a right colostomy was performed on a short colon, but the genitour...

The authors report experience with five children, four of whom underwent esophagogastric dissociation with a Roux-en-Y esophagojejunal anastomosis and jejuno-jejunostomy after recurrent failure of fundoplication to control gastroesophageal reflux. A fifth child had a similar temporary gastric dissociation because of life-threatening aspiration caus...

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-thickn...

Longitudinal intestinal lengthening and tailoring (LILT) is increasingly favoured as a treatment for short-bowel syndrome. In a personal series, 20 children underwent LILT between 1982 and 1997. There was negligible morbidity (hemiloop anastomotic stenosis in 2) and no operative mortality. At a mean follow-up of 6.4 years overall survival was 45%,...

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 ne...

Preservation of the umbilical cord attachment (UCA) in gastroschisis (GS) is still not routine practice. In a prospective series of 36 children with GS, it was always possible to preserve the UCA, even in those undergoing a temporary silo and delayed closure. Reconstruction by 'umbilical cord capping' left no additional scar and achieved a normal a...

A reproducible technique was developed experimentally in pigs for construction of a 'composite bowel tube' (CBT) made up of ileal mucosa that was grafted to a mucosally denuded colonic muscle surface vascularised by the colonic mesentery. Macroscopic and microscopic studies at terminal laparotomy revealed a viable, mucosally lined, patent peristalt...

The patient with short bowel syndrome is essentially unable to absorb sufficient nutrients. This is caused by either short mucosal contact time, insufficient mucosal surface area (enterocyte mass), or a combination of the two. Management consists primarily in sustaining health and growth by intravenous nutrition and in enhancing the natural intesti...

Undescended testis is a common congenital anomaly occurring in approximately 1% of male infants. In approximately 20% of patients with an undescended testicle, the testis is not palpable.1 The management of a child with a palpable testis is not controversial, but management of an infant with an impalpable testis can generate considerable clinical d...

This paper presents results from a case record review of the transcrotal approach to undescended testes, hydrocoecle, and hernia in 457 children at the Royal Manchester Children's Hospital between 1984 and 1991. Our results show that orchidopexy for the palpable undescended testis and routine inguinal herniotomy in children can be safely performed...

Ten neonates ranging in gestational age from 27 to 40 weeks and weighing 800–3,000 g presented, nine with peritonitis and pneumoperitoneum and one with gastroschisis. At surgery, perforation and rupture of the stomach was discovered. One had a tracheo-oesophageal fistula, one had gastroschisis, and in nine cases there had either been endotracheal i...

Three hundred twenty infants underwent a Ramstedt pyloromyotomy for hypertrophic pyloric stenosis, 182 via a transverse right hypochondrial incision and 138 via a circumumbilical incision. The overall rate of wound infections was 16% for operations performed via the umbilical approach compared with 5.5% for operations via a transverse approach desp...

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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-St...

Paper read at the British Association of Paediatric Surgeons Congress, July 1991.

The long term neurodevelopmental outcome was assessed in 23 survivors born with congenital diaphragmatic hernia who had been managed by an elective delay in surgical repair after a period of stabilisation. This cohort was treated in one neonatal surgical unit between 1983 and 1989 by a single team of surgeons and anaesthetists. All children underwe...

Tracheomalacia involving the whole of the trachea is uncommon and difficult to manage. We report a child with oesophageal atresia and tracheo-oesophageal fistula who presented in the immediate postnatal phase with severe respiratory embarrassment that was relieved by endotracheal intubation with a long tube. The trachea, which was totally devoid of...

Two procedures to increase mucosal surface area in patients with short bowel syndrome have been evaluated in pigs. The colonic serosal patch technique of Binnington, Gaton, and Thompson for growth of new small-bowel mucosa revealed little if any neomucosal growth along the colonic serosal surface. Similarly, using the colonic muscle patch technique...

Congenital hepatic artery-portal vein arteriovenous (AV) malformation is a rare anomaly. We report a 5-week-old infant with this lesion localised within the medial segment of the left lobe of the liver. Hepatic segmentectomy with excision of the aneurysmal AV malformation and maximal preservation of normal liver appears to be a safe and effective t...

Surgery for infants with intestinal atresia has evolved along with the development of specialized neonatal surgical units. This once fatal condition now carries a better than 85% chance of survival and an excellent long-term prognosis. Recent advances in bowel preservation techniques have reduced morbidity and improved gut function in both the long...

Over an 8-year period at the Royal Manchester Children's Hospital, 38 intra-abdominal testes in 32 children aged 2–15 years have been transferred to the scrotum with full revascularisation by microvascular anastomosis of the testicular to the inferior epigastric vessels. Eight children had bilateral microvascular orchidopexy. Follow-up has extended...

During the period 1984–1989, 25 infants with oesophageal atresia and tracheo-oesophageal fistula underwent primary oesophageal anastomosis incorporating a full-thickness pedicle oesophageal flap from the dilated upper oesophageal pouch. Use of the flap creates a wide, oblique anastomosis in all instances. Where a large “gap” is present after full m...

Between 1954 and 1987, 43 children with sacrococcygeal teratoma have been seen in our institution. There was 1 stillbirth, 4 neonates (9%) died during transfer, and there were 2 operative deaths (4.5%). Median age at operation was 4 days (range 1 day to 3 years). The survivors have been followed up for a median of 5.5 years (range 5 months to 32 ye...

The management of subglottic stenosis in children remains a controversial issue. This study aimed to develop a reproducible, single-stage, curative procedure for primary reconstruction of the trachea in subglottic stenosis that would be applicable in the newborn period. A method of resection of the subglottic portion of the trachea with reconstruct...

Eleven cases of colonic atresia presenting in a 13-year period are described. The incidence of this condition is estimated at 1 per 66,000 live births in the north-west of England. A higher incidence of involvement of the transverse colon has been noted in comparison with previously reported cases. Survival was unaffected by the type of surgical pr...

Over a 5-year period (1984–1989), 25 infants underwent a primary neonatal Swenson resection and pull-through procedure for Hirschsprung's disease. There was no mortality and morbidity was limited to 3 infants, 1 of whom developed a rectourethral fistula. We conclude that with modern anaesthesia and monitoring, a primary reparative operation is a sa...