Orchidopexy without ligation of the processus vaginalis is not associated with an increased risk of inguinal hernia.

PURPOSE: To report our experience using a modified orchidopexy with division and non-ligation of the processus vaginalis. METHODS: We performed a single-centre retrospective analysis of all patients who underwent orchidopexy between December 2005 and October 2008 at our institution. In the present technique, the processus vaginalis was gently peeled off the spermatic cord structures as high as possible and severed at the level of the internal inguinal ring without its ligation. Postoperative follow-up was routinely offered to all patients. Additionally, we made a special clinical follow-up, ranging from 1 to 69 months (median 34). RESULTS: One hundred and twenty-three patients, aged 1-11 years (median 3), underwent 147 orchidopexies during the study period. Of these, 25 were accomplished using conventional division and ligation of the processus vaginalis, and in the remaining 122 orchidopexies, the processus vaginalis was only divided. Of the 137 testes available at follow-up, 134 were in the scrotum and 3 (2 %) required re-do orchidopexy due to secondary reascent, including 2 treated with division only of the processus vaginalis. None of the patients experienced postoperative hydrocele or inguinal hernia development. CONCLUSIONS: Our findings confirm that division without ligation of a patent processus vaginalis is usually followed by spontaneous peritoneal scarring and complete closure of the internal inguinal ring. Present technique is as effective as traditional orchidopexy and saves extra time spent for meticulous closure of the processus vaginalis or peritoneal tears.

Outcomes after tongue-lip adhesion or mandibular distraction osteogenesis in infants with Pierre Robin sequence and severe airway obstruction.

The objective was to review and compare outcomes after tongue-lip adhesion (TLA) and mandibular distraction osteogenesis (MDO) in infants with severe breathing difficulties related to Pierre Robin sequence (PRS). A single-centre retrospective (2002-2012) study was carried out; 18 infants with severe breathing difficulties related to PRS resistant to conservative treatment, who underwent TLA or MDO to correct airway obstruction, were enrolled. The primary outcome measures were successful weaning from respiratory support and resumption of full oral feeding. Nine underwent TLA and nine MDO. Eight of the nine infants who underwent MDO and all those treated with TLA were successfully weaned from respiratory support. After discharge, residual respiratory distress was diagnosed more commonly after TLA than after MDO (6/9 vs 1/9, P=0.050). Infants resumed oral feeding sooner after MDO than after TLA (mean days after surgery to full oral feeds 44±24 vs 217±134, P<0.003). The length of hospital stay was longer for infants treated with MDO than for those treated with TLA. The rate of complications was similar. Infants with severe airway obstruction related to PRS can benefit safely from either TLA or MDO. Although MDO lengthens the time to discharge, this option stabilizes airway patency of infants with PRS more efficiently and achieves full oral feeding more rapidly than TLA.

[Horizons of endoscopic surgery]. / Orizzonti della chirurgia endoscopica.

Minimally invasive surgery (MIS) has been one of the most important developments in surgery in the last century. By reducing the incision to small puncture wounds, morbidity, pain, adhesions and scarring are reduced. Due to their small size, neonates have not benefited from the advances in endoscopic surgery as rapidly as their adult counterparts. In the last 10 years, miniaturization of instruments and the development of sophisticated new techniques have enabled paediatric surgeons to apply endoscopic surgery to neonates. MIS is now being performed in both the neonatal chest and abdomen. This presentation will review these new developments and discuss the potential for even further improvements in neonatal surgery in the future. Also, a profile of the patient at risk for an insufflation-related incident and selection of neonates who will benefit most from these techniques in conditions of maximal safety will be drawn.

Two unusual cases of pharyngeal hairy polyp causing intermittent neonatal airway obstruction.

Hairy polyp of the pharynx is an uncommon developmental malformation that is most frequently seen as a penduculated tumour in the neonate. The clinical presentation is characterized by the presence of a polypoid mass protruding through the mouth as 'a second tongue' causing respiratory distress. Two patients are presented with this condition.

Compensatory renal growth in children with unilateral renal tumor treated by nephron-sparing surgery or nephrectomy.

INTRODUCTION: In children with an unilateral renal tumor, nephron-sparing surgery (NSS) results in a more adequate renal function adaptation compared to nephrectomy. In the present study, we investigated whether nephron-sparing surgery is followed by a different renal structure adaptation compared to nephrectomy. METHODS: Sixteen patients with unilateral renal tumor treated by nephrectomy (Group 1) and 10 treated by nephron-sparing surgery (Group 2) were enrolled in the study. Kidney volume was estimated by ultrasonography, using the formula for a prolate ellipsoid. Kidney volume was adjusted to the patient's weight and kidney laterality and expressed as a percentage of the expected volume of two kidneys in a healthy child. Total kidney volume (TKV) corresponded to the volume of the contralateral kidney in Group 1 patients, and to the volume of contralateral kidney + kidney remnant in Group 2 patients. Renal function was evaluated by serum creatinine values adjusted for sex and age and expressed as standard deviation scores (SDS). RESULTS: Group 2 patients presented with a greater indexed TKV compared to Group 1 patients, (97.4 +/- 18.8 % vs. 77.0 +/- 17.7 %; p = 0.005). Indexed TKV below the reference range for healthy controls with two kidneys was found in 4 of 10 Group 2 patients vs. 14 of 16 Group 1 patients (p = 0.017). In both patient groups, correlation analysis of indexed TKV and creatinine SDS showed a negative correlation (r = - 0.47; p = 0.01). CONCLUSION: In children with unilateral renal tumor, NSS is followed by a more adequate compensatory restoration of TKV compared with nephrectomy.

Pathogenesis of apparent life-threatening events in infants with esophageal atresia.

Many infants with a repaired esophageal atresia (EA) undergo fundoplication, aortopexy, or glossopexy because the mechanisms most responsible for airway obstruction and/or apparent life-threatening event (AO/ALTE) syndrome are considered to be gastroesophageal reflux (GER), tracheal compression (TC), or obstructive apnea, respectively. In the present study, we investigated whether these mechanisms are independent or interrelated. We developed a database of 120 consecutive patients with EA treated by the senior author between 1967-2002. We studied the clinical manifestations of patients with a cervical esophagostomy and/or blind lower esophageal stump, which ruled out TC and/or proximal esophageal GER as a mechanism for AO/ALTE. Of 25 neonates who underwent section/ligation of lower tracheo-esophageal fistula and/or feeding gastrostomy, 10 critically ill neonates died. Of 15 survivors, 9 infants had a feeding gastrostomy without an esophagostomy. Of these, 6 infants presented one or more episodes of AO, and 8 presented ALTE with or without AO. Subsequently, 5 of the 9 infants underwent an esophagostomy. Eventually, 11 infants had a feeding gastrostomy with an esophagostomy. Of the latter, 5 infants presented one or more episodes of AO, and 6 presented ALTE without AO. In conclusion, oral feeding, proximal esophageal GER, and TC are not essential for AO/ALTE syndrome to occur. They are probably factors which offer evidence of an underlying problem with control of upper airway patency.

Radial artery pseudoaneurysm successfully treated by compression bandage.

In children, surgery for radial artery pseudoaneurysm (PA) may be followed by growth retardation of the hand because of inadequate blood flow. We believe this is the first report of a child with PA of the radial artery cured by compression bandage. Conservative management is a safe and valuable initial treatment option for uncomplicated radial PA.

Pattern of cardiovascular anomalies associated with esophageal atresia: support for a caudal pharyngeal arch neurocristopathy.

Patients with cephalic neurocristopathy (an abnormality of neural crest differentiation) present a striking pattern of associated cardiovascular anomalies (CVA). Therefore, to support the hypothesis that esophageal atresia (EA) may be related to a defective contribution from the cephalic neural crest, we studied the pattern of CVA associated with EA. Medical records of 99 patients with isolated EA, 101 with isolated anorectal malformations (ARM) and 15 with both EA and ARM, consecutively admitted to our unit, were reviewed. The prevalence and pattern of CVA associated with isolated EA or isolated ARM were compared on the assumption that the cranial or caudal location of a major malformation is related to a different regional patterning of associated anomalies. The prevalence of CVA was 39% in patients with isolated EA and 7% in those with isolated ARM (p < 0.01). Neural crest-related CVA (aortic arch anomalies, conotruncal defects, and superior vena cava malformations) accounted for 72% of all CVA in patients with isolated EA versus 14% in those with isolated ARM (p < 0.02). In patients with isolated EA, anomalies of the fourth and sixth aortic arch derivatives accounted for 75% of all neural crest related CVA. The present pattern of CVA in infants with EA supports the concept that EA may be related to an abnormal contribution from caudal portion of cephalic neural crest.

Respiratory status of infants with esophageal atresia.

In infants with esophageal atresia (EA), lung opacities on a chest radiograph (CXR) are usually considered the cause of respiratory distress (RD). However, in some instances signs of RD and CXR changes show no correlation. The aim of this study was to investigate the pathogenesis of RD in EA patients with a normal CXR. In 41 infants with EA, CXR findings were correlated with clinical manifestations and blood-gas analysis data. The degree of abnormal gas exchange was quantitated by the arterial/alveolar oxygen tension ratio (a-ARO2). Of the 41 infants, 39(95%) presented with RD. No lung opacities were found in 130 of 294 CXRs examined (44%). An a-ARO2 below 0.75 (lower limit of normal) was calculated in 215 of 247 arterial blood samples analyzed (87%). When a temporal correlation was established, RD with a clear CXR was characterized by signs of extra- and intrathoracic airway obstruction, often associated with an a-ARO2 below 0.75. The degree of hypoxemia was greater than the degree of hypercapnia. We conclude that in infants with EA, RD with a clear CXR is related to both tracheomalacia and upper-airway obstruction that may cause miliary atelectasis not detected by conventional CXR with intrapulmonary shunting and hypoxemia.

Nephron-sparing surgery for unilateral primary renal tumor in children.

PURPOSE: Definition of the role of nephron-sparing surgery (NSS) in the treatment of children with primary unilateral renal tumor (URT). METHODS: Between January 1992 and June 2000, 28 children with URT were admitted to our surgical unit. Criteria for selection of patients eligible for NSS were at least 50% of affected kidney preservable and stage I at surgery (frozen section biopsies from regional lymph nodes, perirenal fat, and surrounding renal parenchyma). Preoperative 2-drug chemotherapy was given to all patients more than 6 months of age. Between 1992 and 1995, 3-drug chemotherapy was used after NSS. Thereafter, following NSS, 2-drug chemotherapy was given if no microscopic residual disease was found on final histologic examination. RESULTS: NSS was feasible in 10 of 28 children (35%). Enucleation of 6 tumors (1 metachronous) was performed in 5 patients. NSS was elective in 5 patients, mandatory in 3 patients (1 with aniridia and genitourinary anomalies, 1 with chronic glomerulonephritis, 1 with bilateral hyperplastic nephroblastomatosis), and advisable in 2 patients (1 with familial vesicoureteric reflux and 1 with cystic nephroma). Seven children had standard histology nephroblastoma, 1 highly differentiated epithelial type nephroblastoma, 1 oncocytoma, and 1 cystic nephroma. The only post-NSS complication was macroscopic hematuria in 1 patient. None of the patients had a relapse. All children are alive and disease free with good functioning of the affected kidney after NSS, at a mean follow-up of 40.7 months (range, 2 to 100 months). CONCLUSION: NSS should be considered in selected children with URT, especially in patients with increased risk for metachronous tumor or renal disease, and in patients with benign or low-grade malignant URT.

Persistent esophagotrachea: description of a case.

Esophagotrachea is the most severe form of laryngo-tracheo-esophageal cleft. This congenital anomaly is due to the anomalous differentiation of the primitive cephalic gut into trachea and esophagus. We present a case of a new born female with a common tracheoesophageal canal up to the carina. Atresia ani, a vulvo-vestibular fistula, sacral ipoplasia and others associated anomalies were also present. The baby underwent surgery after a laringo-tracheoscopy and a barium study of the esophagus. The prognosis of this extremely rare malformation is generally poor and the baby died on the fifth day after surgery for a serious ipertensive pneumothorax.

Recurrent apparent life-threatening event relieved by glossopexy.

An infant with repaired esophageal atresia presented with several apparent life-threatening events (ALTEs). He had upper airway instability, gastroesophageal reflux (GER), and tracheomalacia. Oxygen breathing test results showed a modest increase in arterial Po2 consistent with the development of an intrapulmonary shunt from absorption collapse of some hypoventilated areas of the lung. Glossopexy was followed by improvement in upper airway stability, normal oxygen test, and disappearance of ALTE. These findings support the concept that upper airway instability, obstructive apnea, lower airway instability, absorption collapse, massive intrapulmonary shunt, and ALTE are the result of a cascade reaction. The authors conclude that infants with ALTE associated with obstructive apnea and O2 shunting require glossopexy to reduce the risk of sudden death.

Enucleative surgery for stage I nephroblastoma with a normal contralateral kidney.

PURPOSE: Tumor enucleation is not recommended for children with nephroblastoma and a normal contralateral kidney. However, in adults with unilateral low stage renal cell carcinoma tumor enucleation may offer an alternative to radical nephrectomy, since functioning renal tissue is preserved without a greater risk of residual microscopic disease. Enucleative surgery may be more reasonable in children with nephroblastoma, because the risk of relapse can be reduced with chemotherapy. Therefore, we prospectively evaluated the feasibility of enucleative surgery in children with stage I unilateral nephroblastoma. MATERIALS AND METHODS: Between 1992 and 1995, 13 children with nephroblastoma and a normal contralateral kidney were consecutively admitted to our surgical unit. Possible candidates for tumor enucleation were evaluated according to certain criteria, including stage I disease at diagnosis, well-defined margins on post-contrast computerized tomography and at least 50% of the functioning kidney could be preserved. Preoperative and postoperative chemotherapy was given in all cases. RESULTS: Of 4 children with preservation of more than 50% of the functioning kidney 3 were considered eligible for enucleation. The tumors, which were confined to the mid kidney in 2 children and upper renal pole in 1, were successfully enucleated without hypothermia or vascular occlusion. All 3 children are disease-free at 49, 48 and 26 months of followup, respectively. Renal function has been almost completely restored postoperatively. CONCLUSIONS: These preliminary data suggest that enucleative surgery may be a reasonable option in select children with stage I nephroblastoma and a normal contralateral kidney.

Assessment of pulmonary mechanics and breathing patterns during posturally induced glossoptosis in infants.

Respiratory mechanics were studied in nine infants with glossoptosis-apnoea syndrome to determine whether glossoptosis may account for signs of both inspiratory and expiratory airway obstruction. Airflow, oesophageal pressure, inspiratory and expiratory time (Ti and Te), and inspiratory and expiratory resistance (Ri and Re) were measured before and during ventilatory phases characterised by glossoptotic pharyngeal obstruction, induced by turning the infants onto their backs. In addition, an attempt was made to correlate the abnormalities in pulmonary mechanics with the clinical features. During partial glossoptotic pharyngeal obstruction, a significant increase was observed in Te and Re and variable changes in Ti and Ri. During severe obstruction, the infants displayed obstructed inspiratory efforts often associated with stridor, as well as obstructed expiratory efforts often associated with audible grunting and retarded expiratory flow pattern. The expiratory grunt was loudest over the neck and mimicked bronchospasm over the chest. These findings indicate that glossoptotic pharyngeal obstruction induces functional airway obstruction which may affect both inspiration and expiration. Expiratory airway obstruction seems, at least in part, to be due to active braking of expiratory flow.

Maturational dysautonomia and facial anomalies associated with esophageal atresia: support for neural crest involvement.

Patients with esophageal atresia (EA) or choanal atresia/stenosis (CA) present with many clinical features of maturational dysautonomia (DY). Since CA and DY are considered manifestations of cephalic neurocristopathy, we tested the hypothesis that EA may also be related to faulty development of cephalic neural crest. Forty-eight patients with EA and 53 with CA were followed up to study the frequency of the facial anomalies which are regarded as the phenotypic expression of an abnormal cephalic neural crest contribution to facial embryogenesis. Forty-eight patients with EA and 51 with CA had clinical manifestations of DY. Forty-four patients with EA (91%) and 49 with CA (92%) had one or more facial anomalies. Comparing the groups, patients with EA had an increased frequency of unilateral facial anomalies of branchial arch derivatives (P < .01); those with CA had an increased frequency of anomalies of frontonasal process derivatives (P < .01). These findings support the hypothesis that EA may be related to an abnormal contribution from the cephalic neural crest. The presence of facial anomalies may facilitate the diagnosis of subclinical DY.