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.

[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 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.

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.

[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.

[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 antireflux surgery in neurologically impaired children.

From May 1996 to April 2002, 48 laparoscopic fundoplications were performed after failure of medical treatment in 47 neurologically impaired infants and children affected by gastroesophageal reflux. Indications for surgery included vomiting, recurrent upper airway infections, failure of medical therapy, feeding difficulties with failure to gain weight, and instrumental (barium swallow and pHmetry) diagnosis of gastroesophageal reflux. A standard approach was adopted, with minimal access modifications according to the patients' characteristics. In two patients, laparoscopic surgery had to be converted to open surgery because of severe kyphoscoliosis and accidental left emidiaphragm perforation. In another patient undergoing a laparoscopic Nissen fundoplication, a re-do laparoscopic operation was performed. Postoperative analgesia was administered during the first 12 h, and fluid intake and feeding were begun on days 1 and 2, respectively. All patients clinically improved except two; a paraesophageal hernia developed in one, and a stenosis developed in the other. We strongly believe that laparoscopic fundoplication can be successfully adopted in neurologically impaired children as well as in pediatric patients as a whole, with the same advantages and far fewer drawbacks than are expected in adults.

[Thoracoscopic management of suspected thoraco-pulmonary malignant diseases in pediatric age]. / Management toracoscopico delle patologie toraco-polmonari di sospetta natura tumorale in età pediatrica.

Recent improvements and miniaturization of instruments have encouraged a wider use of thoracoscopy and laparoscopy as a modality for diagnostic and operative procedures in pediatric age. The utility of thoracoscopy in pediatric patients with suspected thoracopulmonary oncological diseases is shown by diagnostic accuracy and, if necessary, the possibility to perform at the meantime a mininvasive surgery. We report the experience of our Institution in 16 patients with suspected thoraco-pulmonary oncological diseases and treated for this reason with thoracoscopy. Thoracoscopic is indicated in cases of suspected oncological diseases in children both for diagnosis and treatment.

Sub-inguinal interruption of dilated veins in adolescent varicocele: should it be considered a gold standard technique?

AIM: Surgeons of varicocele are at present still searching for a gold standard technique, which can correct varicocele without any recurrences, maintaining optimal testicular function, having got minimal current and future morbidity and being cost effective. We evaluated the presence of these criteria in the technique of sub-inguinal dilated vein interruption. METHODS: Between 1994 and 2001, 142 youngsters and adolescents underwent surgery for varicocele repair at our hospital. Average patient age was 12.4 years (range 8 to 15). One-hundred-six cases (74.7%) were grade III varicocele, while 36 (25.5 ) were grade II. Grade II varicoceles underwent surgery only if associated with scrotal discomfort, testicular softness or hypotrophy of the affected testis (differential volume between the 2 testicles more than 20% or more than 2 ml ). Varicoceles were repaired using a subinguinal ligation of intrafunicular and extrafunicolar dilated veins. The testicular vaginalis was not touched in 46 children (Group A) but it was reversed in 42 and resected in the other 54 cases to prevent postoperative hydrocele. RESULTS: In 126 cases (88.7%) varicocele disappeared after surgery, in 12 (8.4%) a mild residual vein dilatation persisted but without any sign of reflux at color-Doppler ultrasound, in 4 patients a postoperative venous reflux was found. Thus, our recurrence rate is nowadays 2.8%. Average postoperative follow-up was 2.3 years (range 1 to 5 years). No testicular atrophy was observed. Based on our last series, at 1 year follow-up control (26 cases throughout year 2000), mean testicular volume, assessed by ultrasound, increased not significantly after surgery from ml 4.69 (SD+/-1.46) preoperative volume to ml 5.19 (SD+/-1.36) postoperative (p=0.2). CONCLUSION: First of all, we found a recurrence rate of 2.9% similar to the lowest of the other procedures. Regarding morbidity, the main inconvenience consists in postoperative hydrocele. It occurred in 13% of our 1st series (group A), but only in 4.1% of patients after reversion or resection of the vaginalis tunica. Average postoperative testicular volume increases after varicocelectomy in our patients, even if not significantly. About sparing the testicular artery or not it has been demonstrated that ligation of this artery doesn't impair testicular growth up and our own observations confirm this evidence. Thus we believe it to be more useful and safe to interrupt this artery to avoid recurrences due to a periarterial venous network. Finally we can conclude that sub-inguinal ligature of dilated veins, when approached with rigorous understanding of the pathophysiology of varicocele is a very safe procedure and low cost effectiveness.

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.

[Cystic dysplasia of rete testis associated with ipsilateral renal agenesis. Case report]. / Displasia cistica della rete testis associata ad agenesia renale omolaterale. Descrizione di un caso clinico.

Cystic dysplasia of the rete testis is a rare abnormality often associated with the ipsilateral agenesis of kidney. This malformation is due to a development defect of the mesonephric duct which is the cause of both the dilation of the testicular rete testis and renal agenesis. A case of this rare malformation, showing all the peculiarities described in the medical literature, is presented. A 3 years-4 months boy was examined for an asymptomatic left scrotal mass; thus, he underwent ultrasonography, which showed a multiple tubular and cystic dilatation of left rete testis, associated with the absence of left kidney, afterward confirmed by MAG3-radionuclide scan. Diagnosis was also validated by testicular biopsy. No surgery was required. The child is nowadays under observation and at 2-years follow-up he doesn't show any symptom. According to many authors, a conservative treatment of this benign congenital abnormality is suggested as well as serial ultrasonography to monitor the growth of the testicular mass, which in a longest follow-up, could require surgery. Malignant transformation nor infertility have never been described.

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.

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.

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).

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.

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.

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.