Transient hypogammaglobulinemia of infancy and unclassified syndromic immunodeficiencies are highly common in oesophageal atresia patients.

Due to the high rate of post-operative sepsis and other infectious complications, a routine immunological screening protocol has been initiated since 2015 in our paediatric surgery clinic for all patients admitted with oesophageal atresia (EA) and warrant a delayed definitive treatment. In our study, we aimed to evaluate the immunodeficiencies in EA patients, by comparing them to healthy age-matched controls. As a prospective cohort study, EA patients admitted between 2015 and 2022, who had their definitive operation after the newborn period (>28 days of age) were included. On admission, serum concentrations of IgG, IgA, IgM, lymphocyte subset levels, C3 and C4 levels, specific IgG antibody responses against hepatitis B, hepatitis A, measles, varicella zoster were evaluated. The patients were age-matched with healthy controls to compare the results and followed up until three years of age. If a humoral immunodeficiency was detected, intravenous immunoglobulin treatment was administered before major oesophageal surgery and during follow-up. 31 EA patients (18 M/13F) with a mean age of 13.3 ± 9.0 months were compared with 40 age-matched healthy controls. Mean serum IgG levels were found to be statistically lower than controls in all age groups (P < .05). Transient hypogammaglobulinemia of infancy (THI) and unclassified syndromic immunodeficiencies (USI) were found to be strikingly high, accounting for 29.0% and 22.5%, respectively, adding up to 51.5% of EA patients. This is the first study evaluating immunodeficiencies in EA patients found in the reviewed literature. More than half of EA patients that required delayed surgery had humoral immunodeficiency, so preoperative screening and immunology referral may improve patient outcomes.

A rare complication of pediatric liver transplantation: Post-transplant diaphragmatic hernia.

AIM: Acquired post-transplant diaphragmatic hernia (PTDH) is a rare complication of liver transplantation (LT) in children. We aimed to present our experience in PTDH, and a possible causative background is discussed. METHODS: Medical records of patients who had undergone diaphragmatic repair following LT between 2015 and 2023 were reviewed. Demographic information, details of primary diseases necessitating LT, transplantation techniques, and clinical findings associated with PTDH were evaluated. RESULTS: There were seven patients with PTDH. Median age at transplantation was 69 (range: 9-200) months. Five patients received a left lateral sector, one patient had a right lobe, and one had a left lobe graft. Time between LT and PTDH was 9 (2-123) months. One patient who was diagnosed in the postoperative 10th year was asymptomatic. Respiratory distress and abdominal pain were the main symptoms among all. All patients underwent laparotomy, and primary repair was performed in six patients, and one patient required mesh repair because of a large defect. Small intestine herniated in most cases. There were two complicated cases with perforation of the stomach and colonic volvulus. There is no recurrence or long-term complications for the median 60 (20-119) month follow-up period. CONCLUSION: PTDH is a rare but serious complication. Majority of symptomatic cases present within the first postoperative year, whereas some late-presenting cases may not be symptomatic. Inadvertent injury to the inferior phrenic vasculatures due to excessive use of cauterization for control of hemostasis may be a plausible explanation in those cases.

Fiberoptic endoscopic evaluation of swallowing (FEES) study: the first report in children to evaluate the oropharyngeal dysphagia after esophageal atresia repair.

BACKGROUND: The aim of this study was to evaluate the swallowing problems by fiberoptic endoscopic evaluation of swallowing (FEES) study in both short- and long-gap patients after esophageal atresia (EA) repair. METHODS: Hospital records of patients who had undergone surgery for EA were reviewed retrospectively. Patients were divided into two groups as short-gap (SG) group (n:16) and long-gap (LG) group (n:10) to compare the swallowing problems. FEES study was performed, and the results were discussed in detail. RESULTS: There were twenty-six (16 M/10 F) patients with a mean age at evaluation was 7.52 ∓ 3.68 years. Mean follow-up period was 75.35 ∓ 44.48 months. In FEES study, pharyngeal phase abnormalities were detected in 10 patients (38.4%). Pharyngeal phase abnormalities were detected significantly higher in LG group (p:0.015). Laryngeal penetration/aspiration was seen in four patients on FEES study (15.3%). All of them was in LG group (40%). Laryngeal penetration/aspiration was seen significantly higher in LG group (p:0.014). CONCLUSION: This is the first study to conduct FEES study in children after esophageal atresia repair to evaluate their swallowing conditions. Even though our sample is small, swallowing problems are more common than expected in the cases of LG when compared to SG.

Long-term outcome and efficiency of symptom-selective approach to assess gastroesophageal reflux prior to gastrostomy in neurologically impaired children.

PURPOSE: To evaluate the necessity of preoperative screening for gastroesophageal reflux (GER) prior to gastrostomy in neurologically impaired children. METHODS: Medical records of neurologically impaired children, who have undergone laparoscopic gastrostomy between January, 2004 and June, 2018, were retrospectively reviewed. Before the year of 2014, all patients who required gastrostomy had been routinely screened for GER pre-operatively, but after the year of 2014, only the ones with GER-related symptoms were tested. The characteristics and outcomes of Routine Screening (RS) and Selective Screening (SS) periods were compared. RESULTS: There were 55 and 54 patients in the RS and SS periods, respectively. Demographics, primary pathologies, and mean follow-up durations (> 2 years) were similar. The rate of GER screening was significantly lower in the SS period (29.6% vs. 63.6%). The rate of Laparoscopic Nissen Fundoplication (LNF) combined with gastrostomy was significantly lower in the SS period (14.8% vs. 38.2%). During follow-up, the rates of new-onset GER symptoms (13% vs. 11.7%) and LNF requirement later on (6.5% vs. 8.8%) were statistically similar between the two periods. CONCLUSION: Routine screening for GER is not necessary prior to gastrostomy in neurologically impaired children. Symptom-selective screening algorithm is safe and efficient in the long term.

Two-stage approach in the management of thoracic neuroenteric cyst with spinal extension: thoracoscopic excision following dorsal laminectomy.

BACKGROUND: Neuroenteric cysts (NC) are rare pathologies and localized generally in posterior mediastinum or abdomen where they may extend to spinal canal through a vertebral defect. Isolated spinal lesions require dorsal/ventral laminectomy and thoracic ones thoracotomy or thoracoscopy. Posterolateral approach via thoracotomy is generally performed for lesions with both thoracic and spinal components. Minimal invasive excision of a thoracic NC with spinal extension in an infant is presented herein. CASE REPORT: A term female newborn with an antenatal (26th week) diagnosis of congenital diaphragmatic hernia (CDH) was admitted. On physical examination, she was normal except mild dyspnea and CDH were excluded on radiogram. Left parenchymal opacity necessitated thorax tomography that revealed lobulated cystic lesion (6 × 3.5 × 4.5 cm) in posterior mediastinum. MRI showed intraspinal extension of the lesion through a hemivertebrae (6th). Two-stage procedure was planned for suspected neuroenteric cyst. First, intraspinal component was excised with dorsal laminectomy and the connection was closed. Then, the thoracic component was excised thoracoscopically. Histopathological evaluation confirmed the diagnosis. Total parenteral nutrition and high dose somatostatin analog was needed due to transient left chylothorax on postoperative course. She was well and symptom-free in postoperative period. CONCLUSION: Neuroenteric cysts may lead to misdiagnoses in antenatal period. MRI is critical to show spinal and vertebral pathologies in suspected cases. Thoracoscopy may safely be performed for thoracic lesions with spinal extension in two-stage approach following closure of the connection and excision of the spinal component.

Pulmonary arteriovenous malformation in children.

Although pulmonary arteriovenous malformations (PAVM) are relatively rare in children, they are important in the differential diagnosis of common pulmonary problems, such as hypoxemia, hemoptysis and dyspnea on exertion. We report the cases of two PAVM patients with different presentations and describe the treatment strategies.

Esophageal Atresia Associated with Congenital Duodenal Obstruction: Turkish Esophageal Atresia Registry (TEAR) Evaluation.

INTRODUCTION: Coexistent congenital duodenal obstruction and esophageal atresia (EA) is known to have significant morbidity and mortality. Management strategies are not well-defined for this association. The data from the Turkish EA registry is evaluated. MATERIALS AND METHODS: A database search was done for the years 2015 to 2022. RESULTS: Among 857 EA patients, 31 (3.6%) had congenital duodenal obstruction. The mean birth weight was 2,104 (± 457) g with 6 babies weighing less than 1,500 g. Twenty-six (84%) had type C EA. The duodenal obstruction was complete in 15 patients and partial in 16. Other anomalies were detected in 27 (87%) patients. VACTERL-H was present in 15 (48%), anorectal malformation in 10 (32%), a major cardiac malformation in 6 (19%), and trisomy-21 in 3 (10%). Duodenal obstruction diagnosis was delayed in 10 (32%) babies for a median of 7.5 (1-109) days. Diagnosis for esophageal pathologies was delayed in 2. Among 19 babies with a simultaneous diagnosis, 1 died without surgery, 6 underwent triple repair for tracheoesophageal fistula (TEF), EA, and duodenal obstruction, and 3 for TEF and duodenal obstruction in the same session. A staged repair was planned in the remaining 9 patients. In total, 15 (48%) patients received a gastrostomy, the indication was long-gap EA in 8. Twenty-five (77%) patients survived. The cause of mortality was sepsis (n = 3) and major cardiac malformations (n = 3). CONCLUSION: Congenital duodenal obstruction associated with EA is a complex problem. Delayed diagnosis is common. Management strategies regarding single-stage repairs or gastrostomy insertions vary notably depending on the patient characteristics and institutional preferences.

Determining the Risk Factors for Anastomotic Stricture Development after Esophageal Atresia Repair: Results from the Turkish Esophageal Atresia Registry.

INTRODUCTION: Anastomotic stricture (AS) is the second most common complication after esophageal atresia (EA) repair. We aimed to evaluate the data in the Turkish Esophageal Atresia Registry to determine the risk factors for AS development after EA repair in a large national cohort of patients. METHODS: The data between 2015 and 2021 were evaluated. Patients were enrolled into two groups according to the occurrence of AS. Patients with AS (AS group) and without AS (non-AS group) were compared according to demographic and operative features, postoperative intubation status, and postoperative complications, such as anastomotic leaks, fistula recanalization, and the presence of gastroesophageal reflux (GER). A multivariable logistic regression analysis was performed to define the risk factors for the development of AS after EA repair. RESULTS: Among the 713 cases, 144 patients (20.19%) were enrolled into the AS group and 569 (79.81%) in the non-AS group. The multivariable logistic regression showed that, being a term baby (odds ratio [OR]: 1.706; p = 0.006), having a birth weight over 2,500 g (OR: 1.72; p = 0.006), presence of GER (OR: 5.267; p < 0.001), or having a recurrent tracheoesophageal fistula (TEF, OR: 4.363; p = 0.006) were the risk factors for the development of AS. CONCLUSIONS: The results of our national registry demonstrate that 20% of EA patients developed AS within their first year of life. In patients with early primary anastomosis, birth weight greater than 2,500 g and presence of GER were risk factors for developing AS. When patients with delayed anastomosis were included, in addition to the previous risk factors, being a term baby, and having recurrent TEF also became risk factors. LEVEL OF EVIDENCE: III.

Prenatal diagnosis of infantile myofibromatosis of the lung: a case report and review of the literature.

We present a case of infantile myofibromatosis of the lung detected at 32 weeks' gestation. The fetus was monitored with weekly ultrasound examinations measuring the mass size and amniotic fluid index. On day 2 after delivery, due to respiratory distress, an exploratory thoracotomy was undertaken and the mass was resected.

Nutritional Experiences of Parents of Children Who Had Gastric Transposition Surgery.

This study aimed to understand the postoperative nutritional experiences of parents of children who had undergone gastric transposition surgery after corrosive esophageal injury. The study had a descriptive qualitative research design, and used a structured interview form as a result of the COVID-19 pandemic. The population of the study consisted of the parents of 12 children who had undergone gastric transposition surgery at the pediatric surgery clinic of a university hospital in an urban area in Turkey. Thematic analysis was conducted on the transcripts using the MAXQDA program. Four main themes emerged and five supporting sub-themes were identified. The main themes were what we experienced in the postoperative nutrition process, coping with how we live, what we want to know, and our recommendations to healthcare professionals. Family-centered care should be a main focus when caring children who have had gastric transposition surgery, and children and their parents should be supported by a multidisciplinary team.

Single-incision laparoscopy-assisted gastric transposition combined with thoracoscopic esophagectomy for esophageal replacement.

INTRODUCTION: We present a novel approach to single-incision laparoscopy-assisted gastric transposition combined with thoracoscopic esophagectomy in a child with long-segment corrosive esophageal stricture (CES). MATERIALS AND SURGICAL TECHNIQUE: A 2.5-year-old boy with a history of caustic ingestion underwent periodic esophageal dilatations at 3-week intervals, four sessions of topical mitomycin C application, and a strict antacid/H2 antagonist therapy for 8 months. The esophageal replacement was indicated because of persistent corrosive esophageal stricture. First, thoracoscopic native esophagus dissection was performed. After gastric mobilization with single-incision laparoscopy, esophagectomy and esophagogastric anastomosis were performed through the cervical incision. There was no complication, and the patient had gained weight by the end of the 12-month follow-up. DISCUSSION: Single-incision laparoscopy-assisted gastric transposition combined with thoracoscopic esophagectomy is feasible, safe, and effective in patients with corrosive esophageal stricture.

Pediatric bedside tracheostomy in the pediatric intensive care unit: six-year experience.

In this study, we evaluated the experience of a single center pediatric intensive care unit in pediatric bedside tracheostomies performed during a six-year period. Thirty-one bedside tracheostomies were performed on 31 patients aged 2 months to 18 years. The major indication for tracheostomy was prolonged ventilator dependence. Twenty-two complications, 6 major and 16 minor, were observed in 18 patients. Early complications were observed in 5 patients and all were managed immediately without serious outcomes. Ten patients died during the study period and only one death was directly related to the tracheostomy; the remaining 9 patients died due to their underlying disease. Eleven patients were successfully decannulated, 12 patients were discharged home with their tracheostomies and 5 of these 12 patients required home ventilation. Although children who required tracheostomy had a high overall mortality (32.3%), the prognosis of these patients depends primarily on the underlying medical condition.

Radiological nodule behavior: A critical parameter in the surgical management of pediatric pulmonary metastases.

Divarci E, Arslan S, Dökümcü Z, Kantar M, Demirag B, Öniz H, Ertan Y, Alper H, Erdener A, Özcan C. Radiological nodule behavior: A critical parameter in the surgical management of pediatric pulmonary metastases. Turk J Pediatr 2018; 60: 372-379. Radiological nodule behavior (RNB) means the course of nodule appearance on consecutive CT scans. In this study, we aimed to discuss the effects of RNB on prognosis of patients with pulmonary metastasis. Retrospective analysis of patients who underwent pulmonary metastasectomy (PM) between 2005-2015 was performed. RNBs were grouped as stable, enlarging or new rising nodules. The effects of RNBs were analyzed on recurrence, 1- and 3-year event free survival (EFS) and mortality. Twenty-seven patients (18 male) underwent PM with a median age of 15 years (3-18 years). The diagnoses were listed as osteosarcoma (13), Wilms` tumor (7), Ewing`s sarcoma (3), synovial sarcoma (2), rabdomyosarcoma (1) and mixed germ cell tumor of testis (1). RNBs were new rising in 15 patients (55%), enlarging in seven patients (26%) and stable in five patients (19%). Vital tumor metastasis was detected in all of the patients with enlarging nodules (100%); in 10 of the 15 patients with new rising nodules (66%) and none of the patients with stable nodules. None of the patients with stable nodules developed recurrence or died after PM (p˂0.05). In patients with enlarging nodules, metastases recurred and they died in the postoperative period. These findings were similar in different types of tumors. RNB could be used as a critical parameter in deciding surgical management strategies of pulmonary metastases. Stabile nodules should be observed by close follow-up with serial CT scans without surgery. All of the suspected new rising nodules should undergo surgical sampling to avoid unnecessary chemotherapy. Nodule progression under chemotherapy is a poor prognostic criteria for overall survival.

Topical Mitomycin C Application Is Effective Even in Esophageal Strictures Resistant to Dilatation Therapy in Children.

PURPOSE: Several treatment techniques may be used in the treatment of esophageal strictures. The purpose of this study was to present the effects of topical mitomycin C (TMC) as an useful adjunct to dilatation therapy in esophageal strictures. MATERIALS AND METHODS: A retrospective analysis of patients who underwent TMC between February 2015 and July 2016 was performed. Dysphagia score, periodic dilatation index, and number of dilatations were compared before and after intervention to investigate the efficacy of TMC. RESULTS: TMC was performed on 20 patients with a median age of 3.5 years (2 to 17 y). The diagnosis was corrosive esophageal strictures in 14 patients, anastomotic strictures in 5 patients, and congenital esophageal stricture in 1 patient. The length of the stricture was long in 10 patients (50%). The median dysphagia score decreased from 2 (1 to 3) to 0 (0 to 2) after application (P<0.001). The median number of dilatation sessions decreased from 5 (1 to 41) to 1 (0 to 11) after intervention (P<0.001). The median periodic dilatation index decreased from 1 (0.66 to 1.34) to 0 (0 to 1.33) after TMC (P<0.001). Regular esophageal dilatation was not necessary in 16 patients after application (80%). The length of the stricture did not affect the efficacy of TMC. The success of treatment was lower in patients with a long treatment period before TMC (>3 y) (50%). No complications were seen in a median follow-up period of 16 months (7 to 22 mo). CONCLUSIONS: TMC application has a significant positive effect as an adjunct to dilatation therapy in most of the patients with different types of esophageal strictures. It should be performed as a safe and efficient treatment option even in patients who were resistant to dilatation therapy.

Thoracoscopic Treatment of Pulmonary Hydatid Cysts May Have a High Morbidity Risk in Children: Retrospective Analysis.

OBJECTIVE: Thoracoscopic treatment of pulmonary hydatid cyst (PHC) has been considered to be a good treatment option in both children and adults for nearly 25 years. However, there have been very few pediatric studies published during this period. Our goal is to review our results and evaluate the efficiency of thorascopy in pediatric patients. MATERIALS AND METHODS: The medical records of patients with PHC who were surgically treated between 2005 and 2015 were reviewed. As all cysts larger than 5 cm in diameter were surgically removed, cysts less than 5 cm in diameter were medically treated and were not included in the study. Demographics, cyst characteristics, and operative/postoperative data were compared between patients who underwent thorascopy (thoracoscopy group) and patients who underwent thoracotomy (thoracotomy group). Chi-square and t-test were used for statistical analysis where appropriate. RESULTS: There were 26 consecutive children (14 girls, 12 boys; mean age 9.4±2.7) included in the study. Except for 2 incidentally diagnosed patients, all were symptomatic, 4 had multifocal lesions, and multiorgan involvement was detected in 11 patients. Thoracoscopy was performed in 10 patients, and conversion was necessary in 2 patients due to unsuccessful fistula ligation attempts. The thoracoscopy group included 8 thoracoscopically treated patients, and remaining patients constituted the thoracotomy group (n=18). Comparison of preoperative characteristics of the groups was insignificant, whereas the overall complication rate (residual bronchial fistula, prolonged air leak, pneumothorax, and localized air cyst) and median hospital stay were significantly higher in the thoracoscopy group. There was no mortality and no recurrence at the postoperative follow-up after 37.4 months. CONCLUSION: The thoracoscopic approach to PHC may have a high risk of conversion and postoperative complication rate. Thoracotomy in children still seems to be the approach of choice for PHC larger than 5 cm. Routine thoracoscopic hydatid cyst treatment is yet far from being the gold standard, whereas thoracoscopy may be preferred in selected patients.

Surgical approaches to the aberrant right subclavian artery.

Aberrant subclavian artery (arteria lusoria) is a rare congenital anomaly that usually does not produce symptoms. Symptomatic patients require surgical intervention. The operative approach to correct this condition has been controversial. Herein, we describe surgical approaches to the aberrant right subclavian artery. From 2000 through 2004, 3 children and 1 adult with aberrant right subclavian artery underwent operation. Our surgical approach varied according to the age of the patient. A muscle-sparing right thoracotomy was used in the pediatric patients, and a supraclavicular approach was used in the adult. Patients were treated successfully by division of the aberrant right subclavian artery and translocation to the right common carotid artery, without graft interposition. There was no operative or late morbidity or death. Symptoms were completely relieved in all patients. Although an extrathoracic approach is applicable and reliable for adult patients, we believe that adequate exposure for the described procedure is best accomplished through a right thoracotomy in pediatric patients. This approach enables optimal mobilization of the distal right subclavian artery without leaving a long stump and enables direct anastomosis to the ipsilateral carotid artery.

The Efficacy of Intralesional Steroid Injection in the Treatment of Corrosive Esophageal Strictures in Children.

PURPOSE OF THE STUDY: Esophageal dilatation can be insufficient in the treatment of severe corrosive esophageal strictures. In this study, we aimed to present the efficacy of intralesional steroid injection as an adjunct to dilatation therapy. MATERIALS AND METHODS: Retrospective analysis of children who underwent intralesional steroid injection between 2004 and 2014 was performed. Patients' age, type of corrosive substance, length of stricture, number of injection and dilatation sessions and complications were reviewed. The success was evaluated by the comparison of number, frequency, and requirement of dilatation therapy before and after injection. RESULTS: Intralesional steroid injection was performed to 32 children with a mean age of 3.6±2.5 years. The types of corrosive agents were alkali (24) or acid (8). Mean number of injection sessions was 2.5±1.1(1 to 6). Mean number of dilatation sessions was decreased from 10±8.8 to 5.4±4.6 after injection (P=0.003). Mean frequency of dilatations was extended from 3.6±0.9 weeks to 8.7±3.9 weeks (P=0.000). Dilatation treatment was successfully terminated in 25 of 27 children with short-segment strictures after injection (92%). Whereas all of the children with long-segment strictures could not resolved and finally required esophageal replacement (5 patients). One patient had transient cushingoid phenotype as a complication. There was not seen any major complication-like perforation. The mean follow-up period was 6±3 years. CONCLUSIONS: Intralesional steroid injection is an effective adjunct to dilatation in most of the children with short-segment strictures. It should be performed as a safe and efficient treatment option in patients with short-segment corrosive esophageal strictures resistant to dilatation therapy.

Enzymatic debridement of burn wound with collagenase in children with partial-thickness burns.

Seventy-eight pediatric burn patients treated by enzymatic debridement with collagenase clostridiopeptidase A (CCA), were compared to 41 patients those burn wounds were excised surgically. Patients whose burn wounds were initially assessed as partial-thickness at admission were enrolled in the study. Total removal of eschar was achieved in 49 of 78 (62.8%) patients by CCA only (group D). In 29 patients (37.2%), therapy with CCA was ceased because of the development of burn wound infection or a manifest need for grafting of the wound, therefore, these patients underwent tangential wound excision (group DS). The records of 41 patients, treated by early tangential excision, having similar burn wounds by extent and depth with groups D and DS were used as controls (group S). There was no significant difference between the time to achieve a clean wound bed in groups D, DS and S (mean 7.8, 8, and 7 days, respectively, P>0.05). In group D, none of the patients required blood transfusion, except one. Patients in group DS were found to have fewer excisions (mean 1.1) when compared to those in group S (mean 1.5, P<0.05). The shortest hospital stay was found in group D (12.5 days, P<0.01). In conclusion, the use of CCA, provided a short hospital stay, reduced the overall need for surgery and blood transfusions in patients with partial-thickness burns. Thus, CCA should be considered as an initial treatment of choice for removal of eschar in children, having a partial-thickness burn wound without infection.

Acute communicating haematocele: unusual presentation after blunt abdominal trauma without solid organ injury.

Haematocele is an unusual scrotal disorder in children, and is generally associated with direct trauma to the inguinoscrotal region. A 6-year-old boy with acute communicating haematocele who had a history of blunt abdominal trauma one week earlier is presented. Interestingly, no solid organ injury was detected on his previous admission for abdominal trauma, nor there was a history of hydrocele.

Initial oxygenation response to inhaled nitric oxide predicts improved outcome in congenital diaphragmatic hernia.

BACKGROUND: Pulmonary hypertension (PH) is the most important complication of congenital diaphragmatic hernia (CDH) and still has a high mortality rate. The aim of this study was to evaluate the effectiveness of inhaled nitric oxide therapy in PH due to CDH. METHODS: Hospital records of children who had undergone inhaled nitric oxide therapy for PH due to CDH between June 2009 and December 2011 were reviewed. RESULTS: Twenty-nine patients had a diagnosis of CDH at the time of study, and eight of these patients underwent nitric oxide therapy because of failure of conventional ventilation techniques, which was successful in five of these patients. Patients who had a good overall outcome of nitric oxide therapy experienced rapid improvement (pretreatment, mean PaO2 = 44.8 mmHg; after the first hour of therapy, mean PaO2 = 96.8 mmHg), whereas patients with no response did not have a similar course (pretreatment, PaO2 = 37 mmHg; after the first hour, PaO2 = 54.6 mmHg). CONCLUSION: Inhaled nitric oxide therapy seems to increase survival in PH due to CDH. No predictive parameters to orient patient selection could be identified; however, the early response seemed to predict the overall outcome. Good results in our series were attributed to routine use of sildenafil and dopamine, along with the nitric oxide inhalation.