Congenital colonic stenosis: a case of late-onset.

PURPOSE: Colonic atresia and stenosis are rare causes of intestinal obstruction in the infant. Only 1.8%-15% of intestinal atresias occur in the colon. Congenital colonic stenosis is even less common than colonic atresia. Only 10 cases have been reported in Literature since 1966 and only one late-onset case has been reported in Literature until now. We describe the case of a 4-month-old baby coming to our attention because of an intestinal subocclusion due to a congenital colonic stenosis of the ascending colon. CASE REPORT: A 4-month-old baby came to our attention for persistent abdominal distension, reduction of bowl function and decaying of overall clinical conditions. A plain abdominal radiograph showed distended intestinal loops with air-fluid levels and no gas in the rectum. During the barium enema the contrast medium appeared to completely fill the lumen of the colon up to the ileo-cecal valve and Cecum appearing higher than normal. Beyond the ileo-cecal valve, the contrast medium showed an abnormal hypotonic dilatation of the small intestinal loops. Suspecting an organic intestinal obstruction, an explorative laparotomy was deemed necessary and at halfway in the ascending colon a stenosis was found. RESULTS: The post-operative course was uneventful and the patient is currently in good clinical conditions, has a normal diet and is thriving. CONCLUSION: Considering both the Literature and our own experience, it is wise to reckon the congenital colonic stenosis as a rare but possible cause of complete or partial intestinal obstruction not only in the newborn but also throughout the first year of life.

One trocar assisted pyeloplasty (OTAP): initial experience and codification of a technique.

BACKGROUND: The Anderson-Hynes pyeloplasty is still the gold standard for the surgical treatment of the ureteropelvic junction obstruction (UPJO) in children. To reduce the surgical invasivity, laparoscopic (RPSc) and retroperitoneoscopic (RPSc) pyeloplasty have been proposed but concerns have been expressed. In the RPSc approach, the small operative space and the technical difficulties have limited the application and diffusion of this techinique while in case of laparoscopy, a transperitoneal technique is used to treat a retroperitoneal disease. We report our initial experience with the "one trocar assisted pyeloplasty" (OTAP) in the treatment of UPJO in children. METHODS: From January 2005 to December 2006 sixteen children (13 males--3 females) from 2 to 18 months of age (mean age 5.6 months), with impaired monolateral renal function underwent surgical treatment for UPJO. In 11 cases the obstruction was on the left side and in one case a crossing vessel was found. The renal pelvis was anteriorly reached using a 10 mm operative telescope via a flank 12 mm incision. The UPJ was exteriorized and a dismembered pyeloplasty performed also for the crossing vessel. In two cases a 6 Fr uretero-pyelo-nephrostomy catheter was used. RESULTS: The patients were discharged after four days without complications. The cosmetic results are excellent. In one case, a small perianastomotic leakage was seen. The ultrasonographic follow-up demonstrated the spontaneous resolution of the leakage. The post operative US follow-up showed the reduction of the dilatation in all cases. DISCUSSION: This is an initial experience and a longer follow-up is needed. However, in our opinion the OTAP could be considered as a safe and feasible alternative to the laparoscopic or retroperitoneoscopic pyeloplasty, especially in very small children and also in presence of crossing vessels.

[Laparoscopic surgery in Hirschsprung's disease]. / Trattamento laparoscopico nel megacolon agangliare.

The laparoscopic treatment of benign conditions of the colon represents only one of the numerous application fields of the modern laparoscopic techniques in pediatric surgery. Until the half of the 1990's, the surgical treatment of choice for Hirschsprung's disease has been the procedure ideated by Soave in the early 1960's. Nowadays, the optimal treatment in the classic forms of aganglionic megacolon consists in performing, in neonatal age or in the first months of life, a transanal extramucous pull-through. This approach can be integrated by a laparoscopic step. From September 2000, at the Department of Pediatric Surgery, Bologna University, we have operated 21 cases of congenital aganglionic megacolon with this technique.

[Thoracoscopic treatment of oesophageal atresia]. / Correzione toracoscopica dell'atresia esofagea.

BACKGROUND: Advancements in minimally invasive surgery in newborns have allowed even the most complex neonatal procedures to be approached using these techniques. Other authors have demonstrated its efficacy in the treatment of the esophageal atresia with distal fistula. METHODS: We report our experience based on the thoracoscopic repair of esophageal atresia with distal fistula in two newborns. Birth weights were 2.800 g and 2.300 g respectively. The patients were intubated endotracheally and placed in a left prone position. Four trocars were inserted: the first one of 5 mm was positioned in the fifth intercostal space for the camera, the other two of 3 mm were positioned in the fourth intercostal space on the anterior and posterior axillary line respectively for the operative instruments. The last trocar of 3 mm was inserted in the third intercostal space on the anterior axillary line for the lung retractor. CO2 was insufflated at a pressure of 8 mm Hg and a flow of 0.5 L/min. The fistula was first isolated then ligated and cut with scissors. The proximal esophagus was opened and an anastomosis was made over a 6F or 8F nasogastric tube with interrupted 4-0 Vicryl sutures. A tube chest was placed through the lower trocar site with the tip near the anastomosis. RESULTS: These two procedures were free of neither intraoperative nor post-operative complications. Feeding by nasogastric tube was started after a mean of 4 days. Barium swallow made on day 7 demonstrated no leakage and no stenosis of the anastomosis. Total oral feeding was possible after 8 days. Mean hospitalization was 14 days. CONCLUSION: This initial report shows, as demonstrated by the experience since 1999 by other authors, that the thoracoscopic esophageal repair in the newborns is technically feasible and, thanks to a magnified vision, it allows to abtain a good isolation of the esophagus and of the tracheo-esophageal fistula respecting the anatomical structures. Moreover the advantages are in terms of exposure and esophageal length, avoiding the significant short and long-term morbidity associated with thoracotomy.

[Video-assisted treatment for biliary atresia]. / Trattamento videoassistito dell'atresia delle vie biliari.

BACKGROUND: The surgical treatment of biliary atresia is still a great challenge for pediatric surgeons. Kasai's operation usually needs a wide, painful, muscle-cutting laparotomies that quite often are followed by pain and peritoneal adhesion. These possible complications may disturb the post-operative course and humper liver transplantation. Advancements in minimally invasive surgery have allowed even the most complex procedures to be approached using these techniques. METHODS: The authors present a case of successful Roux-en-Y laparoscopic portoenterostomy for the treatment of biliary atresia. We report a case of a 3-month-old patient with biliary atresia who weighted Kg 5,300 at the operation. The patient was placed in supine position. The procedure was performed with 4 trocars of 3 mm and 1 of 10 mm. The umbilical site was used for extracorporeal Roux-en-Y enteroenterostomy. CO2 was insufflated at a pressure of 8 mmHg and a flow of 0.5 L/min. A drain was placed through the lower trocar site with the tip near the anastomosis. RESULTS: The procedure was free of neither intraoperative nor post-operative complications. Feeding by nasogastric tube was started after 2 days. Total oral feeding was possible after 8 days. CONCLUSION: Laparoscopic approach to perform Kasai's operation is technically feasible and thanks to a magnified vision, it allows to abtain a good visualization of the portal structures with an adequate retraction of the liver. This procedure can avoid or decrease the post-operative complications such as pain, breathing difficulty, adhesions and resulting in very small scars. Anyway laparoscopic Kasaiportoenterostomy should be done by a surgeon with a good experience in laparoscopic hand-suturing and neonatal experience and with the support of an experienced in neonatal and infantile videosurgery anaesthesiologist.

[Laparoscopic surgical treatment of anorectal malformations]. / Trattamento laparoscopico delle malformazioni ano-rettali.

Anorectal Malformations (ARM) include a wide spectrum of anomalies, ranging from anal and rectal agenesis (usually associated with a recto-urethral, rectovaginal or recto-vestibular fistula) to ano-cutaneous fistula. On the basis of studies on the anatomy of the muscular structures contained in the pelvic cavity and on the physiology of the mechanisms which regulate the continence, many different techniques have been performed to allow a surgical treatment for the ARMs and to obtain post-operative results as nearer as possible to normality, that is to an adequate colic motility and a defecation control. In 2000, a new surgical technique for high and intermediate ARMs has been introduced, the laparoscopically assisted ano-rectal pull-through (LAARP) according to Georgeson. At the Department of Pediatric Surgery, Bologna University, from 2000 to nowadays, we have treated with LAARP 7 cases of high ARMs, all male patients and with colostomy. The average age at operation was between 2 and 10 months.

The role of emergency thoracoscopy in the management of congenital cystic adenomatoid malformation of the lung associated with oesophageal atresia.

Congenital cystic adenomatoid malformation of the lung associated with oesophageal atresia is exceptional. The authors describe a case of a mixed type I - II congenital cystic adenomatoid malformation of the left lung associated with oesophageal atresia and tracheooesophageal fistula in a male infant. The interesting aspect of this case is not only the extremely rare association - only two reports in the literature - but the surgical choices. In fact, two weeks after surgical repair of the oesophageal atresia, the growth of the cystic volume of the congenital adenomatoid malformation made respiratory weaning very difficult, and it was therefore decided to solve the respiratory distress by opening the tensional cysts using a thoracoscopic access. The advantage of this treatment was that it decompressed the underlying healthy lung tissue and permitted the expansion of the normal lobar parenchyma. This is a palliative approach that allows the mandatory definitive resection of the affected lung lobe to be postponed until a later time.

Laparoscopic repair of Morgagni-Larrey hernia in a child.

Primary laparoscopic repair of Morgagni-Larrey hernia has been described in adult patients but not in children. This is the first report of primary laparoscopic correction in the pediatric age group without using a prosthesis. A Morgagni-Larrey hernia was found incidentally in a 3-year-old-girl. Laparoscopic correction of the defect was performed. After 6 months the patient is doing well. The chest radiograph shows complete resolution of the hernia. The laparoscopic approach allowed repair the hernia with minimal invasiveness. Laparoscopic correction is not difficult except for those hernias in which dense adhesions are present.

The varicocele in pediatric age: 207 cases treated with microsurgical technique.

Between 1984 and 1995, 207 boys afflicted by varicocele have undergone surgery. They were all treated employing a venous microvascular termino-terminal anastomosis between spermatic and inferior epigastric veins. The recurrence rate with this technique (3%) is much lower than Ivanissevich's one (nearby 15%). The clinical results are optimal, with disappearance of varices in the majority of cases and improvement of the testicular trophism in the remainder. This technique enables the establishment of an immediate and physiological testicular venous outflow essential for testicular growth and development.

Persistent Mullerian Duct Syndrome associated with transverse testicular ectopia: a case report.

The authors describe their experience in the treatment of a child, male phenotype, with a Transverse Testicular Ectopia (TTE), associated with a rudimentary uterus and two Fallopian tubes (Persistent Mullerian Duct Syndrome = PMDS). The boy was transferred to the Pediatric Surgical Unit of the University of Bologna from another hospital where at operation for bilateral cryptorchidism the diagnosis had become apparent. In order to exclude the presence of PMDS it is very important that an abdominal exploration should be performed in all cases of TTE where the testes are undescended. The diagnosis was made by an occasional detection: it is very important, when a TTE in which the testes are undescended is found, to perform the abdominal exploration, in order to exclude the presence of PMDS.

Laparoscopic Nissen fundoplication with fibrin glue: experimental study on pigs.

Nowadays laparoscopy shows a great development in the pediatric age group. Pediatric surgeons are now able to study the possible applications in pediatric surgery of this new and fascinating procedure. A mandatory laparoscopic training to perform any important operation is clearly essential. Only a few cases of Nissen fundoplication have been reported in children. We suggest this experimental study in which we add a technical modification by using fibrin glue to tighten the gastric wrap on the esophagus positioning only two stitches at the extremes of the suture. The results were interesting and we think it an excellent starting point for a future clinical application of this new technique.

Laparoscopic surgery of diaphragmatic diseases in children: our experience with five cases.

AIMS: In paediatric surgery the laparoscopic approach can be used to repair diaphragmatic anomalies originating from the abdomen or containing abdominal viscera. Candidates for laparoscopic correction are children with mild symptoms and good respiratory and haemodynamic conditions. The authors present their experience with 5 patients treated successfully for different types of diaphragmatic lesions. PATIENTS AND METHODS: Five children were treated laparoscopically since 1998. Two true Morgagni-Larrey hernias, one recurrent left Bochdalek hernia, one diaphragmatic dysontogenetic cyst and one huge congenital sliding and rolling hiatal hernia. All the herniated viscera were repositioned in the abdomen and the defects--including the diaphragmatic hole at the level of the dysontogenetic cyst--were directly sutured without the use of a mesh. RESULTS: All patients are healthy without signs of recurrence observed at chest X-ray after a follow-up of 3 months to 1 year. DISCUSSION: Under specific conditions the laparoscopic approach can be an effective and more advantageous alternative to laparotomy for diaphragmatic congenital diseases in a paediatric population.

Antenatal diagnosis and early laparoscopic treatment of a rare variation of androgen-insensitivity syndrome.

Androgen-insensitivity syndrome (AIS) (Testicular feminisation or Morris syndrome) is characterised by external female genitalia and bilateral testes with a normal male karyotype. This kind of syndrome is transmitted recessively. Presence of hypoplastic and short "pseudo-vagina" is one of the characteristics of this syndrome. We report here a case of AIS in which there was a well-formed vagina (6 cm). The case was diagnosed antenatally and managed laparoscopically.

Ectopic cervical thymus: case report.

Ectopic cervical thymus is rarely considered in the differential diagnosis of cervical masses: this lesion is essentially asymptomatic and generally occupies a position in the neck along the carotid sheath, underneath the sterno-cleido-mastoid muscle. It is supposed that most of these masses arise as a consequence of migration defects during glandular embryogenesis. Ectopic thymus rarely invades contiguous structures but in the literature some cases of malignant transformation of aberrant cervical thymus have been reported. Some non-invasive investigations (MRI, ultrasonography) are useful but accurate diagnosis depends eventually on surgical excision and histologic examination.

Laparoscopic removal of a persistent Müllerian duct in a male: case report.

The authors refer to the use of the laparoscopic approach in Persistent Mullerian Duct Syndrome, starting from a case of "male vagina" with an anomaly inserted left ductus deferens inside the mullerian persistent duct. This patient was operated on using the laparoscopic technique.

Gastric perforation in a newborn.

Gastric perforation in neonates is an uncommon condition. In most cases, it is attributed to peptic ulceration and/or hemorrhagic gastritis. The high mortality rate in such patients can be improved by early diagnosis and prompt resuscitation, followed by surgery. We report a full-term female newborn, who developed a gastric perforation in the first day of life. The possible aetiology and the perioperative management are discussed.

The nonpalpable testis: an experience of 132 consecutive videolaparoscopic explorations in 6 years.

From may 1995 to may 2001, 114 children with nonpalpable testis (NPT) were evaluated at our institution (18 babies had bilateral cryptorchidism). The age range was 1-11 years. When ultrasonography and nuclear magnetic resonance cannot show the position of the NPT along the normal pathway, video laparoscopy is essential for diagnostic accuracy. 20 cases were observed to have blind-ending was deferens and testicular vessels; therefore, no other procedure was done. 4 had residual nonfunctional intra-abdominal tissue; in 52 cases, an intra-abdominal testis was found, and 17 microvascular and 26 traditional orchidopexies were performed. The remaining 50 patients (six with bilateral cryptorchidism) had normal vas and spermatic vessels entering the inguinal canal. In 15 cases a normal testis was present, and it was positioned into the scrotum with the standard technique; in 41 cases an atrophic testis was found and was removed through an inguinal approach. Diagnostic laparoscopy permits not only localisation of the testis but also planning for a better therapeutic program with a minimally invasive procedure, thus avoiding the knife in 18% of cases (in our experience 15% of blind-ending and 3% of abdominal vanishing testis).

Agenesis of the appendix vermiformis.

Agenesis of the appendix vermiformis represents a rare condition that accounts in approximately 1 in 100.000 laparotomies performed for suspected acute appendicitis. L.B., female, born at term after a normal pregnancy. Shortly after birth she presented respiratory distress; chest x-ray showed a left diaphragmatic hernia (CDH). At operation was noted the absence of the appendix and of the mesenteriolum as well together with the presence of a mesenterium commune. Other associated anomalies were detected: dextroposition of the heart, hypoplasia cnemis, hexadactylism of right foot, congenital hip displacement and bilateral congenital cataract. Etiopathogenesis of the agenesis of the appendix can be easily understood from an embryological point of view, following the cecal pole development. Diagnosis of this malformation is possible only after an accurate laparotomic or laparoscopic exploration around the ileocecal and retrocecal zone.

The role of endoscopic surgery in paediatric oncological diseases.

Recent improvements in video imaging and instrumentation have encouraged a wider use of endoscopic surgery as a modality for diagnostic and operative procedures. To asses the utility and diagnostic accuracy of endoscopic surgery in children with oncological diseases, we reviewed our experience about thirty-eight patients affected by oncological diseases, referred to our Department since 1995. We performed laparoscopy in 22 cases, thoracoscopy in 14, 1 combinated procedure (laparoscopy + thoracoscopy) and retroperitoneoscopy in 1 case. Endoscopic surgery is indicated in cases of paediatric oncological diseases both for diagnosis and treatment.

Retroperitoneoscopic adrenalectomy in a residual of neuroblastoma.

The authors report a case of retroperitoneoscopic adrenalectomy in a child with neuroblastoma previously treated by chemotherapy. Usually the presence of malignant tumours is a contraindication to retroperitoneoscopic surgery. A restaging of the tumor was done by retroperitoneoscopy. The residual adrenal gland was excised by mini-invasive surgery of the retroperitoneal space. The histopathologic examination confirmed the presence of a residual neuroblastoma in the adrenal gland. This case report shows a possible of a new indication for the retroperitoneoscopy.