Antonio Messineo

Congenital diaphragmatic hernia—A tale of two cities: The Toronto experience☆

PURPOSE The optimal therapy for congenital diaphragmatic hernia (CDH) is evolving. This study analyzes the results of treatment of CDH in a large tertiary care pediatric center using conventional and high-frequency oscillatory ventilation (HFOV) without extracorporeal membrane oxygenation (ECMO) contrasting these with a parallel study from a similar large urban center using conventional ventilation with ECMO. METHODS Between 1981 and 1994, 223 consecutive neonates who had CDH diagnosed in the first 12 hours of life were referred for treatment before repair. Conventional ventilation was used with conversion to HFOV for refractory hypoxemia or hypercapnia, and a predicted near 100% mortality rate. ECMO was used in only three patients, all of whom died. A retrospective database was collected. Thirty-one clinical variables were tested for their association with the outcome. Common ventilatory and oxygenation indices were tested for their prognostic capability. RESULTS Apgar scores, birth weight, right-sided defects, pneumothorax, total ventilatory time, and the use of high frequency oscillatory ventilation were the only variables associated with outcome. A modified ventilatory index and postductal A-aDo2 were strong prognostic indicators. From 1981 to 1984 surgery was performed on an emergency basis. Since 1985 surgery was deferred until stabilization had been achieved. This resulted in a shift in the mortality from postoperative to preoperative with no change in total survival. HFOV did not alter the overall survival. Results of autopsies performed (70%) showed significant pulmonary hypoplasia and barotrauma as the primary causes of death. The survival was 54.7%. CONCLUSION Conventional ventilation with HFOV produced equal survival to conventional ventilation with ECMO in two comparable series. Pulmonary hypoplasia was the principle cause of death. This continued high mortality at both centers suggests that new therapies are required to improve outcomes.

Pulmonary barotrauma in congenital diaphragmatic hernia: A clinicopathological correlation

BACKGROUND/PURPOSE The high mortality rate in congenital diaphragmatic hernia (CDH) has been ascribed to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn (PPHN). One of the principal treatment strategies has been the use of hyperventilation to reverse ductal shunting, but the wisdom of this approach is being questioned because of parenchymal lung injury from high inflation pressures. The authors hypothesize that the use of hyperventilation to reverse or prevent ductal shunting would result in ventilator-induced lung injury, which would be evident on postmortem examination. A retrospective review of clinical and autopsy information was conducted. METHODS Clinical and autopsy information gathered for a previously published series of 223 infants with CDH presenting in the first 24 hours of life was reviewed. Autopsy and clinical data were analyzed from 68 of 101 nonsurvivors who died with severe hypoxemia. RESULTS Sixty-two of 68 cases (91%) had evidence of diffuse alveolar damage and hyaline membrane formation, which was more evident in the ipsilateral lung. Forty-four (65%) infants had pneumothoraces, and 4 infants had interstitial fibrosis. Pulmonary hemorrhage was seen in 35 cases (50 maximum peak inspiratory pressure [mean +/- SD] was 40.4+/-7.9 cm H2O and lowest modified ventilatory index [respiratory rate x peak airway pressure] was 2323+/-836). The degree of pulmonary hypoplasia was evaluated by lung weight with the ratio of the observed combined lung weight to the expected lung weight based on birth weight and gestational age. The ratio based on birth weight was 57%+/-25%, and the ratio based on gestational age was 60%+/-26%. Twenty-one infants (35%) had nonpulmonary anomalies. The most significant was a 10% incidence of congenital heart disease. Apart from this, lethal nonpulmonary anomalies were rare. CONCLUSION These results suggest that lung injury secondary to mechanical ventilation plays an important role in the mortality rate of patients with CDH, which may become increasingly significant when there is underlying pulmonary hypoplasia.

Severe tracheomalacia associated with esophageal atresia: Results of surgical treatment☆

This study reviews our experience from 1977 to 1991 with 32 children born with esophageal atresia and tracheoesophageal fistula (EA-TEF) who later underwent surgery for severe tracheomalacia. Indications for surgery included dying spells (21), inability to extubate the airway (nasotracheal tube 5, tracheotomy 3), and recurrent pneumonia (3). Diagnosis was confirmed by chest x-ray, esophagogram, and bronchoscopy. Aortopexy was performed in 31 of 32 children; a splint without aortopexy was used in one older girl. Splinting was also necessary in two of the 31 at the initial operation, when aortopexy failed to prevent tracheal collapse. There were four initial failures. Currently, 29 children are well (median follow-up, 6.6 years). Two have a tracheostomy in place, and one, who had a vascular ring divided after aortopexy, died at home of unknown cause 1 month later. These findings indicate that aortopexy provides long-term relief of severe symptoms of tracheomalacia associated with EA-TEF in almost all affected children. When aortopexy fails, the insertion of an airway splint may succeed; otherwise, tracheostomy is necessary.

Adnexal torsion in children and adolescents: new trends to conservative surgical approach – Our experience and review of literature

The purpose of this study is to discuss the surgical treatment for ovarian torsion in children and adolescents with a focus on the procedures of adnexal conservation surgery and its frequency in the literature of the last 10 years. We retrospectively reviewed the medical charts of 127 operative ovarian lesions including 30 ovarian torsions (23.6%) treated in two pediatric centers over a 10-year period. Age at presentation, presenting symptoms, diagnostic studies, surgical procedure and pathological findings were analyzed. Mean age was 13.7 years. Conservative surgery has been performed in 46.7% of the cases and laparoscopic approach in 40%. Ovarian torsion occurred in 56.7% on ovaries with functional lesion, in 23.3% on normal adnexa and in 20% on ovaries with benign neoplasm. The article includes a literature review (2000–2010) and a statistical analysis which shows a slow increase in conservative surgery from 28 to 45%. Laparoscopic surgery accounts for 23.5%. Literature review shows 40.5% normal adnexa, 33.2% non-neoplastic lesions, 25.3% benign neoplasms and 1% malignant neoplasms. The surgical treatment of children and adolescents presenting adnexal torsion should be practiced as an emergency and it should be more conservative as possible in order to maximize the future reproductive potential.

The Role of Testicular Volume in Adolescents With Varicocele: The Better Way and Time of Surgical Treatment

PURPOSE We report varicocele prevalence in adolescents. Surgical treatment has been proposed in adolescents with relevant testicular disproportion to avoid fertility problems in adulthood. We prospectively analyzed the testicular volume variation in adolescents with varicocele and hypoplastic testis. MATERIALS AND METHODS In a 2-year period we selected 54 consecutive pediatric patients with a median age of 14.5 years (range 13 to 16) who had left varicocele using certain criteria, including testicular volume discrepancy greater than 20%, no previous inguinal-testicular surgery and no symptoms. Adolescents were divided into 2 groups, including 27 who underwent surgical correction with lymphatic sparing microsurgical varicocelectomy (intervention) and 27 who were only observed (control). After surgery or at first observation patients were evaluated clinically and by ultrasound at 3, 6 and 12 months. Testicular volume was estimated by the prolate ellipsoid formula. RESULTS We noted significant improvement in testicular volume with less than 20% disparity between the 2 gonads in 23 patients (85.2%) in the intervention group and in 8 controls (29.6%). Two recurrences (7.4%) were reported in the intervention group, each in an adolescent with increased testicular volume. CONCLUSIONS Our study confirms significantly increased testicular volume in many surgically treated boys and shows that physiological catch-up growth occurs in adolescents with varicocele without treatment. Considering critically results in each group, in select cases clinical and ultrasound followup is indicated before intervention due to a possible spontaneous decrease in testicular asymmetry. Further histopathological studies are needed to identify the relationship between testicular hypoplasia, irreversible damage and future fertility problems to determine which adolescents should be treated.

Clinical factors affecting mortality in children with malrotation of the intestine

In children with symptoms secondary to malrotation of the intestine, a retrospective statistical study was undertaken to identify factors associated with an increased risk of mortality. Between 1964 and 1989, laparatomy was performed on 182 children. For study purposes, the children were divided into three groups. Group I included 71 patients with an obstruction only in the duodenum. The remaining 111 children, all of whom had midgut volvulus, were further divided; those without gut necrosis (79) into group II, and those with necrosis (32) into group III. One child each died in groups I and II, and 15 in group III. In all children we evaluated the relationship between mortality and age at presentation, presence of associated serious abnormalities, time from onset of symptoms to surgery, and the presence of necrotic bowel. For group III, we considered the influence of percentage of bowel resected on mortality. In these 182 children the factors associated with an increased risk of mortality were presence of necrosis (P < .0001), presence of other abnormalities (P = .0008), and younger age (P = .0084). Time from onset of symptoms to surgery was not associated with statistically increased risk of mortality. The 1% mortality noted in children without intestinal necrosis (group I and II) was related to associated abnormality. For group III the estimated probability of survival ranged from .999 for patients with 10% of intestinal necrosis to .351 for whose with 75% of intestinal necrosis, assuming the best prognostic conditions (patient older than 3 months with no associated serious abnormalities).

Repair of long tracheal defects with cryopreserved cartilaginous allografts

Tracheoplasties with various autografts (cartilage, periosteum, pericardium) have been used in the treatment of long-segment tracheal stenosis. Previous studies have shown that cartilage allografts survive transplantation on a long-term basis in various sites of the body. In this study we set out to determine if cryopreserved cartilage and cryopreserved tracheal allografts would survive when used to cover tracheal defects in animals. A rectangular defect (2.8 +/- 0.3 cm long and incorporating 50% of tracheal circumference) was created in the thoracic trachea of 18 piglets. The defect was covered with the excised tracheal segment in 6 (group A, control group), with a cryopreserved tracheal allograft in 6 (group B), and with a cryopreserved cartilage allograft harvested from the scapula in 6 (group C). The allografts were cryopreserved, by a standard slow-freezing technique, at -80 degrees C for more than 21 days. All animals survived the grafting procedure and were killed after 2 months. None had signs of airway obstruction. Using the trachea above the defect as the standard, the mean sagittal narrowing of the airway in the repaired trachea was 0.4 mm in group A, 0.7 mm in group B, and 0.6 mm in group C; the coronal diameter in normal and grafted trachea was similar. The lumen of all grafts was lined by regenerating respiratory epithelium, and cilia were seen in many. Some cartilage was reabsorbed in group A and B but cartilage islands were present in all. In group A, reabsorption of cartilage was minimal. These findings suggest that segments of trachea or cartilage allografts can be cryopreserved, stored, and, subsequently, used when necessary for tracheoplasty.

The treatment of internal anal sphincter achalasia with botulinum toxin.

Abstract. Internal anal sphincter (IAS) achalasia is a disorder of defecation in which the IAS fails to relax. Botulinum toxin (BT), which has been successfully used to relax the anal and lower esophageal sphincters, was injected twice into the IAS of one adolescent and three infants with manometric, radiologic, and in 2 cases histochemical diagnosis of anal achalasia; in the adolescent a third injection was necessary. Spontaneous defecation was achieved in all patients following the second injection. In one case a diagnosis of short-segment Hirschsprungs disease was obtained after the second injection. Local infiltration of BT into the IAS proved effective in the treatment of IAS achalasia. Double-blind studies and longer follow-up periods are needed to better evaluate these preliminary results and define the limits of this promising therapy.

Successful tracheal autotransplantation with a vascularized omental flap

A major problem in tracheal transplantation is the restoration of an adequate vascular supply to the transplanted trachea. In 12 piglets, a segment (6 rings) of thoracic trachea was removed and the excised segment was then sutured back in place. In 9 animals (group A), a vascularized omental flap was wrapped around the autotransplanted trachea. In the other 3 pigs (group B), the omentum was not used. Eight of 9 group A pigs were killed, 1 or 2 months later, having had no signs of airway obstruction; the 9th pig was killed after 14 days because of airway obstruction. The 3 pigs in group B were killed after 11 to 13 days because of progressive respiratory obstruction. In the 8 asymptomatic pigs in group A, the omental flap was viable and tracheal growth was normal with no differences in diameter between normal and autotransplanted trachea. Histologically intact cartilage was lined with respiratory epithelium. In the one group A pig who was killed early, the omental flap was necrotic. In this pig and in the 3 group B animals, extensive tracheal necrosis and nonviable cartilage were observed. These findings indicate that in the pig, a 6-ring segment of trachea can be transplanted with vascularization provided by an omental flap.

Chest fast MRI: an Imaging alternative on pre-operative evaluation of Pectus Excavatum

BACKGROUND Standard imaging methods in evaluating chest wall deformities, such as Pectus Excavatum (PE) in paediatric and adolescent patients, include baseline 2-view chest radiography and chest CT scan. Only few studies to date investigated the value of fast MRIin the pre operative assessment of patient affected by PE. OBJECTIVE To evaluate the efficacy of chest fast MRI in pre-operative management of patient affected by PE. To obtain the Haller Index (HI) and Asymmetry Index (AI) from chest fast MRI protecting patients from radiation exposure. MATERIALS AND METHODS We analyzed the data of 42 consecutive patients with severe PE who underwent minimally invasive repair between March 2007 and March 2010. All 42 patients received chest fast MRI, but only the first 5 in view of the results, were studied also with chest ultrafast CT scan. In both examinations, data at the deepest point of the depression were collected. RESULTS Severity indices of the deformity using HI and AI, collected from CT scan and fast MRI in the first 5 patients, were comparable. In the remaining 37 fast chest MRI offered good images of the chest wall deformities with no radiation exposure, detailing anatomical information such as displacement and rotation of the heart or great vessels anomalies. CONCLUSION This study suggests the use of chest MRI in pre operative workup for patients with PE to obtain severity indices (Haller Index and Asymmetry Index avoiding radiation exposure to paediatric patients.