Systematic review of long term follow-up and transitional care in adolescents and adults with esophageal atresia - why is transitional care mandatory?

PURPOSE:  to review recent literature concerning long-term health issues and transitional care in esophageal atresia (EA) patients. PubMed, Scopus, Embase and Web of Science databases were screened for studies regarding EA patients aged more than or equal to 11 years, published between August 2014 and June 2022. Sixteen studies involving 830 patients were analyzed. Mean age was 27.4 years (range 11-63). EA subtype distribution was: type C (48.8%), A (9.5%), D (1.9%), E (0.5%) and B (0.2%). 55% underwent primary repair, 34.3% delayed repair, 10.5% esophageal substitution. Mean follow-up was 27.2 years (range 11-63). Long-term sequelae were: gastro-esophageal reflux (41.4%), dysphagia (27.6%), esophagitis (12.4%), Barrett esophagus (8.1%), anastomotic stricture (4.8%); persistent cough (8.7%), recurrent infections (4.3%) and chronic respiratory diseases (5.5%). Musculo-skeletal deformities were present in 36 out of 74 reported cases. Reduced weight and height were detected in 13.3% and 6% cases, respectively. Impaired quality of life was reported in 9% of patients; 9.6% had diagnosis or raised risk of mental disorders. 10.3% of adult patients had no care provider. Meta-analysis was conducted on 816 patients. Estimated prevalences are: GERD 42.4%, dysphagia 57.8%, Barrett esophagus 12.4%, respiratory diseases 33.3%, neurological sequelae 11.7%, underweight 19.6%. Heterogeneity was substantial (> 50%).   Conclusion: EA patients must continue follow-up beyond childhood, with a defined transitional-care path by a highly specialized multidisciplinary team due to the multiple long-term sequelae. WHAT IS KNOWN: • Survival rates of esophageal atresia patients is now more than 90% thanks to the improvements in surgical techniques and intensive care, therefore patients' needs throughout adolescence and adulthood must be taken into account. WHAT IS NEW: • This review, by summarizing recent literature concerning long term sequelae of esophageal atresia, may contribute to raise awareness on the importance of defining standardized protocols of transitional and adulthood care for esophageal atresia patients.

Preliminary experience with laparoscopic repair of associated inguinal and umbilical hernias in children.

PURPOSE: The authors report their preliminary experience in laparoscopic repair of associated inguinal and umbilical hernias in children. METHODS: Twenty-six patients affected by the association of inguinal and umbilical hernia with an umbilical defect larger than 5 mm underwent a laparoscopic procedure. A 5-mm trocar was placed through the umbilical defect for the optic. To fix the trocar to avoid loss of carboperitoneum, we fashioned and tightened a purse-string non-absorbable suture with a sliding knot around the defect. In this manner, we ensured the trocar, fixing it and avoiding any loss of CO2, proceeding safely to the laparoscopic IH repair, by means of two additional 3 mm operative trocars. At the end of the inguinal herniorrhaphy, the previously fashioned purse-string suture was tightened to repair the umbilical defect. RESULTS: The mean operative time for the repair of associated inguinal and umbilical hernias was 30.1 ± 7.4 min in cases of unilateral inguinal hernia and 39.5 ± 10.6 for bilateral inguinal hernia. Follow-up ranged from 8 to 32 months. Neither intra- nor post-operative complications nor recurrences were seen. CONCLUSION: This small sample suggests that this simple method is safe, effective and might be useful for pediatric surgeons performing laparoscopic repair for inguinal hernia in presence of an associated UH with a statistically significant decrease of operative time.

Multicentric experience on double dartos flap protection in tubularized incised plate urethroplasty for distal and midpenile hypospadias.

PURPOSE: We report a multicenter experience using double dartos flap to protect the neourethra in TIP urethroplasty for distal and midpenile hypospadias. METHODS: A total of 394 patients underwent tubularized incised plate urethroplasty for primary distal and midpenile hypospadias using double dartos flap protection by ten pediatric surgeons and urologists at five different institutions. RESULTS: Tubularized incised plate urethroplasty protected by a double dartos flap was simple to perform and flaps were easy to obtain. Complications occurred in 23 patients (5.83%): fistulas 1.01% (4 cases), stenosis 0.25% (1 case), mild stenosis 2.53% (10 cases), dehiscence of ventral cutis 0.50% (2 cases) and penile torsion 1.26% (5 cases). All fistulae had a spontaneous resolution. CONCLUSION: Double dartos flap to protect tubularized incised plate urethroplasty is safe with a low complication rate. The neourethra is covered entirely with a double layer of vascularized tissue and the double coverage appears a good choice for preventing urethrocutaneous fistula formation.

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.

[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.

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.

Assessment of trans, trans-muconic acid in human seminal plasma.

Trans, trans-muconic acid (tt-MA) is one of the most important metabolites of benzene, a pollutant ubiquitously distributed in ambient air and classified in 1982 as a group I carcinogen. For its sensitivity and specificity, tt-MA excreted in urine is considered a good biological marker of benzene exposure. In this study, seminal tt-MA levels in occupationally nonexposed subjects (n = 32) have been determined. The seminal fluid of normozoospermic subjects contained an average tt-MA concentration (170 +/- 100 ng ml-1) significantly lower than that of teratozoospermic (310 +/- 180 ng ml-1; P < 0.01), oligozoospermic (400 +/- 180 ng ml-1; P < 0.001), and oligoasthenozoospermic (430 +/- 230 ng ml-1; P < 0.01) subjects. A negative correlation existed between tt-MA levels and sperm concentration (r = - 0.62; P < 0.001), percentage of normal spermatozoa (r = - 0.41; P < 0.05), and percentage of vital spermatozoa (r = - 0.89; P < 0.001). Average tt-MA levels detected in seminal plasma were higher in smokers (350 +/- 160 ng ml-1) than in nonsmokers (280 +/- 210 ng ml-1). These results show that seminal plasma tt-MA content could be an important biological indicator for evaluating the negative effects of benzene on spermatogenesis.

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.

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.