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Background Pulmonary hypoplasia and persistent pulmonary hypertension account for significant mortality and morbidity in neonates with congenital diaphragmatic hernia (CDH). Global lung immaturity and studies in animal models suggest the [...]

Byline: Manabu Okawada (1), Tadaharu Okazaki (1), Atsuyuki Yamataka (1), Toshihiro Yanai (1), Yoshifumi Kato (1), Hiroyuki Kobayashi (1), Geoffrey J. Lane (1), Takeshi Miyano (1) Keywords: Congenital diaphragmatic hernia; Protocolized management; Echocardiography; Pulmonary hypertension; High frequency oxygenation Abstract: A review of 100 consecutive cases of congenital diaphragmatic hernia (CDH) treated at our institute focusing on the efficacy of protocolized management (PM) was conducted. Of the 100 cases, 14 who became symptomatic more than 24 h after birth, and seven with fatal anomalies (four cardiac and three chromosomal) were excluded, leaving 79 subjects for this study. Of these, 41 were diagnosed prenatally (PD). Subjects were divided into four groups. Group I: No PD, no PM (n = 34), Group II: No PD, PM (n = 4), Group III: PD, no PM (n = 21), and Group IV: PD, PM (n = 20). PM includes criteria for planned delivery, use of high frequency oxygenation, nitric oxide, echocardiography (EC), and a medication schedule. Overall survival rates for Groups I, II, III, and IV were 73.5% (25/34), 75% (3/4), 38.1% (8/21), and 70.0% (14/20), respectively. Survival rates were higher when PM was used: 70.8% (Groups II, IV) versus 60.0% (Groups I, III). Survival rates were significantly lower if diagnosed prenatally (PD+): 53.7% (Groups III, IV) versus 73.7% (Groups I, II) (P < 0.01). However, in PD+ groups, survival was significantly higher if PM was used (P < 0.05). PM significantly reduced length of hospital stay (35.5 vs. 52.0 days: P < 0.05). EC was found to be a predictor for survival while post-ductal AaDO.sub.2 was not. In 17 cases with cardiac anomalies, PM did not affect survival. Our study suggests that use of PM for prenatally diagnosed CDH cases is associated with improved outcome, although the components of PM need to be tested in prospective trials to determine their true value. Author Affiliation: (1) Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan Article History: Registration Date: 01/08/2006 Online Date: 13/09/2006

Byline: M. J. Smith (1), T. S. Paran (1), F. Quinn (1), M. T. Corbally (1) Keywords: Neonate; Congenital diaphragmatic hernia; Tissue replacement; Tissue engineering; SIS Abstract: There is no ideal bio-tensile material for tissue replacement in paediatric surgical conditions. A variety of materials have been described to replace or reinforce tissue deficits in congenital diaphragmatic hernia (CDH). The problems with these materials range from extensive surgery to body wall deformity and hernia recurrence. The ideal graft would be safe, strong, have the potential to grow and not require replacement, not affect chest wall development or produce deformity, and have minimal risk of recurrence. Surgisis is a biomaterial composed of porcine intestinal submucosa that provides inherent tensile strength as a collagen lattice and is ultimately replaced by native collagen tissue. Our objective was to prospectively assess the utility of Surgisis as a replacement graft in a cohort of 10 children undergoing primary or recurrent CDH repair. The graft was well tolerated, and there was no recurrence of herniation. We conclude that Surgisis collagen mesh may be a useful alternative for tissue replacement or reinforcement in difficult cases of CDH and diaphragmatic agenesis. The biological framework of Surgisis may also prove useful in other situations of complex tissue loss in children. Author Affiliation: (1) Department of Paediatric Surgery, Our Lady's Hospital for Sick Children, 12, Crumlin, Dublin , Ireland Article History: Registration Date: 22/09/2004 Accepted Date: 02/04/2004 Online Date: 24/11/2004

Byline: Maciej BagAaj (1) Keywords: Congenital diaphragmatic hernia; Late presentation; Children Abstract: Articles dealing with late-presenting congenital diaphragmatic hernia (CDH) in children published until 2003 were collectively reviewed. The main purpose of the presented study was to gain insight into the clinical spectrum of this variant of CDH and to discuss its clinical and surgical aspects. In 125 articles, 362 patients were reported. Left posterolateral hernia was the dominant anatomical type, found in 79.4% of the children. Male-to-female distribution was nearly 2:1 in both right- and left-sided CDH. Associated anomalies were noted in 8.6% of children. Sixty-five percent of children with right CDH presented within the 1st year of life, whereas only 41.7% of patients with left CDH belonged to this age group. The children with right CDH presented with chronic symptoms in 57.4% of cases, while among those with left CDH, acute presentation prevailed, constituting 60.5% of this group. No strict correlation between age and clinical symptoms was noted, although the youngest patients tended to present with respiratory symptoms. Late-presenting diaphragmatic hernia is associated with a wide range of clinical symptoms occurring in various constellations. Dyspnea and vomiting were the most frequent symptoms found in patients with either chronic or acute manifestation. Preoperative mortality was 3.9% (14 children). In 45 children (12.4%), complications resulting from diaphragmatic hernia were noted, and in a further 11 children iatrogenic preoperative complications were noted. Among 152 children with reported CDH, true hernia with sac was found in 32.7%. Eight children (2.2%) died in the early postoperative period, while a further 13 patients (3.6%) had postoperative complications requiring surgical management. Recurrence of hernia was noted in seven children (1.9%). This collective review of basic epidemiological and clinical data from a large group of patients seems to support a hypothesis for a congenital nature of the diaphragmatic defect and an acquired nature of 'herniation' of the viscera. The wide clinical spectrum of late-presenting CDH seems to be conditioned by two factors: timing of herniation and type of intraabdominal viscera displaced into the chest. Author Affiliation: (1) Department of Pediatric Surgery, Wroclaw Medical University, M. Sklodowska 52, 50-367 , Wroclaw, Poland Article History: Registration Date: 12/08/2004 Accepted Date: 14/06/2004 Online Date: 03/09/2004

Byline: Hans Skari (1), Kristin Bjornland (1), Bjorn Frenckner (3), Lars Goran Friberg (4), Marja Heikkinen (5), Timo Hurme (6), Borger Loe (7), Gunnhild Mollerlokken (8), Ole Henrik Nielsen (9), Niels Qvist (10), Risto Rintala (11), Katarina Sandgren (12), Willy Serlo (13), Kari Wagner (2), Tomas Wester (14), Ragnhild Emblem (1) Keywords: Congenital diaphragmatic hernia; Extracorporeal membrane oxygenation; High-frequency oscillation ventilation; Mode of delivery; Prenatal diagnosis Abstract: There is no consensus on the treatment of congenital diaphragmatic hernia (CDH), and practice seems to vary between centres. The main purpose of the present study was to survey current practice in Scandinavia. Thirteen paediatric surgical centres serving a population of about 22 million were invited, and all participated. One questionnaire was completed at each centre. The questionnaire evaluated management following prenatal diagnosis, intensive care strategies, operative treatment, and long-term follow-up. Survival data (1995--1998) were available from 12 of 13 centres. Following prenatal diagnosis of CDH, vaginal delivery and maternal steroids were used at eight and six centres, respectively. All centres used high-frequency oscillation ventilation (HFOV), nitric oxide (NO), and surfactant comparatively often. Five centres had extracorporeal membrane oxygenation (ECMO) facilities, and four centres transferred ECMO candidates. The majority of centres (7/9) always tried HFOV before ECMO was instituted. Surgery was performed when the neonate was clinically stable (11/13) and when no signs of pulmonary hypertension were detected by echo-Doppler (6/13). The repair was performed by laparotomy at all centres and most commonly with nonabsorbable sutures (8/13). Thoracic drain was used routinely at seven centres. Long-term follow-up at a paediatric surgical centre was uncommon (3/13). Only three centres treated more than five CDH patients per year. Comparing survival in centres treating more than five with those treating five or fewer CDH patients per year, there was a tendency towards better survival in the higher-volume centres (72.4%) than in the centres with lower volume (58.7%), p =0.065. Author Affiliation: (1) Section of Paediatric Surgery, Rikshospitalet University Hospital, N-0027 , Oslo, Norway (2) Section of Paediatric Anaesthesiology, Rikshospitalet University Hospital, N-0027 , Oslo, Norway (3) Department of Paediatric Surgery, Astrid Lindgren's Children's Hospital, Karolinska Hospital, 171 76 , Stockholm, Sweden (4) Department of Paediatric Surgery, Children's Hospital, Astra Sjukhuset, 416 85 , Gothenborg, Sweden (5) Department of Paediatric Surgery, Kuoppio University Hospital, 70211 , Kuoppio, Finland (6) Department of Paediatric Surgery, Turku University Hospital, 20520 , Turku, Finland (7) Department of Paediatric Surgery, Ulleval University Hospital, 0027 , Oslo, Norway (8) Department of Paediatrics, St. Olavs Hospital, 7000 , Trondheim, Norway (9) Department of Paediatric Surgery, Rigshospitalet, 2100 , Copenhagen, Denmark (10) Surgical Department A, Odense University Hospital, 5000 , Odense, Denmark (11) Department of Paediatric Surgery, Helsinki University Hospital for Children and Adolescents, 00029 , Helsinki, Finland (12) Department of Paediatric Surgery, Lund University Hospital, 226 52 , Lund, Sweden (13) Department of Paediatric Surgery, Oulu University Hospital, 90221 , Oulu, Finland (14) Department of Paediatric Surgery, Children's Hospital, 751 85 , Uppsala, Sweden Article History: Registration Date: 30/03/2004 Accepted Date: 07/01/2004 Online Date: 20/05/2004 Article note: Contributors' list

Byline: Tadaharu Okazaki (1), Sumio Kohno (1), Shiro Hasegawa (1), Naoto Urushihara (1), Atsushi Yoshida (1), Shinya Kawano (1), Akihiro Saito (2), Yasuhiko Tanaka (2) Keywords: Congenital diaphragmatic hernia Nitric oxide Pulmonary hypertension Echocardiography High frequency oscillating ventilation Abstract: To date, uniform standards for congenital diaphragmatic hernia (CDH) management have not existed. The purpose of this study was to evaluate the evolving clinical outcome of the patients with CDH and to present our recent management protocol using echocardiography. Sixty patients treated for CDH at our hospital from 1978 through 2001 were reviewed. Periods of treatments were divided arbitrarily into three periods 1978--1991 (period I, n=26), 1992--1994 (period II, n=6), 1995--2001 (period III, n=28). Immediate repair was performed during period I. We performed preoperative stabilization and delayed repair since the start of period II, and nitric oxide (NO) was introduced in period III. In period III, our management strategy was the use of fentanyl for sedation and analgesia vasoactive agents such as dopamine, dobutamine, and prostagrandin E1 in selected cases the use of high-frequency oscillating ventilation (HFOV), inhaled NO and venovenous extracorporeal membrane oxygenation (ECMO) if indicated. The details of stabilization management and the timing of surgery were determined using echocardiography to evaluate pulmonary hypertension (PH) by measuring dimension and shunt patterns through the ductus arteriosus (DA), right pulmonary artery (rPA) and left pulmonary artery (lPA). Overall, 42 of 60 patients survived (70%). The number of patients surviving in each period was 14 of 26 (54%) in period I, 4 of 6 (67%) in period II, and 24 of 28 (86%) in period III. Seventeen of 28 patients in period III required inhaled NO (group A). Of these 17 patients, 5 required ECMO of these 5, 3 were long-term survivors. The remaining 11 patients from period III who were managed without NO (group B) survived. In left-sided CDH cases, the dimension of DA at admission in group A (5.07+-1.79 mm) was significantly larger than in group B (2.99+-1.68 mm) (P&lt 0.01). The dimension of rPA in group A (3.37+-0.80 mm) was significantly smaller as compared with group B (4.28+-0.72 mm) (P&lt 0.01). Although the dimension of lPA was not significantly different between group A (3.03+-0.74 mm) and group B (3.46+-0.48 mm), lPA blood flow was noticeably stronger in group B. DA shunt patterns were bi-directional (53%), right-to-left (40%) and left-to-right (7%) in group A, whereas no patients in group B showed a right-to-left shunt pattern. After confirmation of closure of DA or dominant left-to-right shunt, and marked increase of pulmonary arterial blood flow, patients in both group A and B underwent surgery successfully. In four non-survivors, findings of improving PH were not observed. We conclude that echocardiographic examination is useful to manage persistent pulmonary hypertension with recent treatment modalities including NO and HFOV and to determine the proper timing of surgery, which contributes to an improved outcome of CDH. Author Affiliation: (1) Department of Pediatric Surgery, Shizuoka Children's Hospital, 860 Urushiyama, 420-8660, Shizuoka, Japan (2) Department of Cardiology, Shizuoka Children's Hospital, Shizuoka, Japan Article History: Accepted Date: 17/08/2002 Article note: Electronic Publication

Byline: Essam A. Elhalaby (1), Magda H. Abo Sikeena (2) Keywords: Congenital diaphragmatic hernia Pneumothorax Pleural effusion Intestinal obstruction Abstract: The late-presenting congenital diaphragmatic hernia (CDH) represents a considerable diagnostic challenge. This study was undertaken to define various patterns of delayed presentation and to analyze pitfalls in the diagnosis and treatment of these patients. Thirty-three children with CDH were treated between 1993 and 2000 15 of these (45.5%) who were diagnosed after the age of 2 months--14 years, median of 2.5 years are reported. Thirteen had a Bochdalek hernia and 2 had a Morgagni hernia. The diaphragmatic defect was right-sided in 6 cases and left-sided in 9. Five patients presented acutely, 3 with respiratory distress and 2 with gastrointestinal (GI) obstruction. The remaining 10 presented with chronic respiratory or GI complaints. Inappropriate insertion of a chest drain occurred in 3 patients misdiagnosed as having pleural effusion (2) and a pneumothorax (1). Two patients had previous chest radiographs reported normal. Plain radiographs were sufficient to make a definitive diagnosis in only 6 patients GI contrast studies were necessary in the other 9. All patients were treated through an abdominal approach with primary closure of the diaphragmatic defect without a patch. A distinct hernia sac was present in 6 cases, and associated malrotation in 6. All except 1 patient survived the operation with rapid improvement of their GI and respiratory symptoms. We conclude that: (1) late-presenting CDH should be included in the differential diagnosis of any child with persistent GI or respiratory problems associated with an abnormal chest X-ray film (2) nasogastric tube placement must be considered as an early diagnostic or therapeutic intervention when the diagnosis is suspected and (3) GI contrast studies should be a part of the diagnostic work-up of these patients. Author Affiliation: (1) Department of Pediatric Surgery, Faculty of Medicine, Tanta University, Tanta, Egypt (2) Department of Pediatrics, Faculty of Medicine, Tanta University, Tanta, Egypt (3) Borg Elmhata, Midan Elmhata, Tanta, 31111, Egypt Article History: Accepted Date: 21/06/2001 Article note: Electronic Publication

Byline: Ahmed H. Al-Salem (1), Akhter Nawaz (2), Hilal Matta (2), Alic Jacobsz (2) Keywords: KeywordsaCongenital diaphragmatic hernia; Morgagni's hernia Abstract: aThe majority of congenital diaphragmatic hernias (CDH) occur through the foramen of Bochdalek herniation through the foramen of Morgagni (MH) is rare. Fifteen children (12 males and 3 females) with congenital MH (7 right, 3 left, 5 bilateral) were treated over a period of 15 years, comprising 11% of a total of 135 children with different types of CDH. The majority (12, 80%) had repeated chest infections. In 1 the hernia was discovered accidentally during evaluation of trauma, and another presented in the neonatal period with acute respiratory distress (ARD). The diagnosis was made on plain lateral chest radiograph when there was anterior herniation of bowel loops, and in these cases the diagnosis was confirmed by barium enema. Computed tomography was useful in preoperative diagnosis when the hernia contents were solid. All patients were operated upon transabdominally except 1, and in all cases there was a hernia sac. Associated anomalies were present in 10 (66.7%) patients: 4 (26.7%) had malrotation, 4 (26.7%) congenital heart disease, and 3 (20%) Down's syndrome. Our study shows a relatively high frequency of MH in our patients. MH rarely presents in the neonatal period, but when it does, it causes ARD. The majority of patients with MH present beyond the neonatal period with repeated attacks of chest infection, and although late-presenting MH is relatively benign, it nevertheless causes significant morbidity. Thus, clinical awareness and early diagnosis and surgical treatment are important factors. Author Affiliation: (1) Division of Pediatric Surgery, Department of Surgery, Qatif Central Hospital, Saudi Arabia, SA (2) Division of Pediatric Surgery, Department of Surgery, Tawam Hospital, Al-Ain, United Arab Emirates, AE (3) P.O. Box 18432, Qatif 31911, Saudi Arabia, SA Article note: Accepted: 21 March 2001

Byline: E. C. Jesudason (1), M. G. Connell (1), D. G. Fernig (1), D. A. Lloyd (1), P. D. Losty (1) Keywords: KeywordsaCongenital diaphragmatic hernia; Pulmonary hypoplasia; Organ culture; Cell culture; Embryology Abstract: aIt has been hypothesised that the liver induces lung hypoplasia in congenital diaphragmatic hernia (CDH) by non-compressive intrathoracic growth rather than traditional mass herniation. Utilising a co-culture system, we tested the capacity of liver cells to inhibit lung growth by contact rather than compression. Heart, liver, and lungs were microdissected from normal rat embryos (n&gt 20 from at least three litters) on day 13.5 of gestation. Monolayer cultures of enzymatically dispersed livers and hearts were established at the same cell density. Lung primordia were cultured in direct contact with hepatic cells or partitioned from them by a permeable polytetrafluoroethylene membrane. This permits the contributions of diffusable factors and cell contact to be distinguished. Lungs were similarly cultured in direct contact with or partitioned from cardiac cells. Lungs cultured in isolation served as further controls. Daily inspection permitted assessment of in-vitro lung growth. Growth of lungs in direct contact with hepatic cells was equivalent to that of lungs partitioned from liver cells. Lungs in direct contact with cardiac cells and lungs partitioned from cardiac cells were also not inhibited compared to lungs cultured in isolation. Early lung development is thus not inhibited by humoral or contact-mediated interactions with embryonic liver cells. Lung hypoplasia in CDH is therefore unlikely to originate from contact inhibition with the developing liver. An intrinsic pulmonary defect may better explain hypoplastic lung development in CDH. Author Affiliation: (1) Institute of Child Health, Alder Hey Children's Hospital, Eaton Road, Liverpool L12 2AP, UK, GB

Byline: C. Sreenan (1), P. Etches (1), H. Osiovich (3) Keywords: KeywordsaCongenital diaphragmatic hernia; Extracorporeal membrane oxygenation Abstract: aThis study documents how congenital diaphragmatic hernia (CDH) is managed in level III neonatal intensive care units (NICUs) in western Canada and examines perinatal factors predictive of the need for extracorporeal membrane oxygenation (ECMO). Information was obtained retrospectively from all level III NICUs in western Canada about the management of infants with CDH between 1992 and 1996 91 infants with isolated CDH were identified. A prenatal diagnosis was made in 42 cases (46%). Surfactant was used in 53%, high-frequency oscillation (HFO) in 29%, and nitric oxide (NO) in 27%. Of the 69 infants born in referral centers, 29 (42%) were referred for possible ECMO 17 (59%) of those required ECMO, with 65% survival. The overall requirement for ECMO was 30%. Death or ECMO occurred in 40% of cases overall. Overall survival was 82%. Survival in those needing ECMO was 74%, and in those not needing ECMO 86%. Significant predictors of death or ECMO were: prenatal diagnosis (P&lt 0.05), maximum postductal arterial partial pressure of oxygen ([P.sub.a]O.sub.2) &lt 100mmHg (P&lt 0.001), and an oxygenation index (OI) at 6h&gt 15 (P&lt 0.001). In cases where there is a prenatal diagnosis of CDH the mother should deliver at an ECMO center. Alternatively, an OI of &gt 15 at 6h and [P.sub.a]O.sub.2&lt 100mmHg should prompt referral to an ECMO center. Author Affiliation: (1) Neonatal Intensive Care Unit, Royal Alexandra Hospital, 10240 Kingsway Avenue, Edmonton, Alberta, Canada T5H 3V9, CA (2) Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada, CA (3) Division of Neonatology, Children's &amp Women's Health Centre of British Columbia, Rm. 1N1, 4480 Oak Street, Vancouver, BC V6H 3V4, Canada, CA Article note: Accepted: 19 November 1999

Byline: L. Desfrere (1), P. H. Jarreau (1), M. Dommergues (2), A. Brunhes (1), P. Hubert (3), C. Nihoul-Fekete (4), P. Mussat (1), G. Moriette (1) Keywords: Key words Congenital diaphragmatic hernia; Antenatal diagnosis; Postnatal management; High-frequency oscillatory ventilation; Intensive care unit; Neonate Abstract: Objective: a) To analyze the influence of a new management strategy on the outcome of neonates with antenatally diagnosed congenital diaphragmatic hernia (CDH) b) to determine early prognosis respiratory factors with the new strategy.APDesign: Retrospective study. Setting: Level III perinatal center. Patients and method: Between 1985 and 1997, 51 consecutive neonates with antenatally diagnosed CDH were admitted to our level III neonatal intensive care unit. Before 1992 (period 1 n = 19), we used conventional mechanical ventilation and early surgery requiring transfer. Since 1992 (period 2 n = 32), we prospectively tested a new approach including (a) systematically use of high-frequency oscillatory ventilation (HFOV) regardless of the initial clinical severity, (b) delayed surgery following stabilization requiring transfer to a different surgical unit, but (c) no transfer of unstable patients with surgery under HFOV in our neonatal intensive care unit (n = 10). The two cohorts were comparable in terms of potential ante and postnatal prognostic indicators. Results: Survival was improved with the new strategy: 21/32 (66 %) vs. 5/19 (26 %) P < 0.02. This improvement between periods 1 and 2 was due to a decrease in both preoperative and postoperative deaths in the later period. The better survival during period 2 was associated with the appearance of very late deaths, frequent pleural effusions, and the survival of more severe forms having evolved to a chronic respiratory insufficiency. Survivors were ventilated for longer time with longer duration of oxygen supplementation. The best oxygenation index (OI), alveolar arterial difference and oscillation amplitude (P/P) during the first 24 h, but not the best PaCO.sub.2, were the most reliable prognostic indicators during period 2. An OI a$? 10 with a P/P a$? 55 cm[H.sub.2]O was associated with a very good prognosis (94 % survival). Conclusions: The prognosis of antenatally diagnosed CDH was improved by systematic HFOV on admission, no systematic transfer, and delayed surgery. This improvement is associated with modification of postnatal outcome. Author Affiliation: (1) Services de Medecine Neonatale, UFR Cochin-Port Royal, 123 Bd. de Port Royal, 74679 Paris Cedex 14, France e-mail: luc.desfrere@cch.ap-hop-paris.fr Tel.: + 33-1-42 34 12 60 Fax: + 33-1-43 29 03 38, FR (2) Medecine Foetale, UFR Cochin-Port Royal, 123 Bd. de Port Royal, 74679 Paris Cedex 14, France, FR (3) Services de Reanimation Pediatrique Polyvalente, Hopital Necker-Enfants Malades, Paris, France, FR (4) Chirurgie Pediatrique, Hopital Necker-Enfants Malades, Paris, France, FR Article note: Received: 7 October 1999/Final revision received: 14 February 2000/Accepted: 25 February 2000

Byline: B. Thebaud (1), C. Saizou (1), C. Farnoux (1), J. F. Hartman (1), J. C. Mercier (1) Abstract: Introduction: Congenital diaphragmatic hernia (CDH) remains a frustating cause of respiratory failure associated with persistent pulmonary hypertension of the newborn (PPHN). Although inhaled nitric oxide (iNO) is effective in many infants with PPHN, it often fails to improve oxygenation in infants with CDH. As the increase in vascular smooth muscle cyclic guanosine monophosphate (cGMP) in response to iNO may be impeded by increased phosphodiesterase type-V (PDE-V) activity, it has been suggested that PDE-V blockade potentiates the efficiency of iNO. Case reports: We used dypiridamole (Persantine), a specific PDE-V inhibitor, in two patients with CDH. Prenatal diagnosis showed a left-sided CDH at 23 weeks of gestation (GA) with intrathoracic stomach and left heart underdevelopment in the one infant and a right-sided CDH at 26 weeks GA with intrathoracic liver in the other. After antenatal corticoids, planned delivery was performed by the vaginal route at 38 weeks GA. Preoperative stabilization was achieved by high frequency oscillation, iNO and inotropic support over 24 h. Both had early pneumothorax drained by a chest tube. Despite optimization of ventilatory and hemodynamic support with surfactant replacement, iNO and adrenaline, oxygenation worsened progressively. Dypiridamole was introduced intravenously at 27 and 40 h, respectively, and improved oxygenation over the next 12 h. However, oxygenation again deteriorated and both patients died. Conclusion: Dypiridamole enhanced the response to iNO in PPHN associated with CDH, although this effect was transient. Combined therapy of iNO with PDE-V inhibitors may improve pulmonary vasodilation in some forms of PPHN which do not respond to iNO, thereby reducing the need for extracorporeal membrane oxygenation (ECMO) and improving outcome. Author Affiliation: (1) Service de Pediatrie-Reanimation, Hopital Robert Debre, Universite Paris VII -- Faculte de Medecine Xavier-Bichat, 48, boulevard Serurier, F-75 019 Paris, France, FR Article note: Received: 6 July 1998 Accepted: 13 November 1998

Byline: P. Daher (1,3), S. Zeidan (1), E. Azar (1), M. Khoury (2), I. Melki (2), R. Mikhael (2) Keywords: Diaphragmatic hernia; Right diaphragmatic hernia; Child Abstract: In right congenital diaphragmatic hernia (RCDH), several clinical diagnostic pitfalls are possible and should be known to those caring for infants and children with this disorder. The records of the 18 patients at Hotel Dieu de France Hospital with a history of CDH between 1990 and 1999 were collected those of the ten who had a RCDH were reviewed retrospectively. The mean age at diagnosis was 6 months the male-to-female ratio was 2:3. The delay between the first symptom and the diagnosis ranged between 0 and 10.5 months (mean 4.5 months). An acute presentation was observed in four cases, consisting of respiratory distress in three the 4th presented with gastric volvulus and intestinal obstruction. The presenting symptoms were mild in four cases recurrent respiratory infections in three and failure to thrive in one. The diagnosis was incidental in two cases during the evaluation of respiratory symptoms attributed to an atrial septal defect. The radiologic findings provided by a chest radiograph (CxR) were sufficient to make an accurate diagnosis in eight cases and peritoneography was useful in one. In six cases, the presenting CxR had been misinterpreted as normal or acute lobar pneumonia. Pathologic findings at surgery consisted of lateral and posterior right diaphragmatic defects in nine cases the defect was lateral and anterior in one. A hernia sac was found in seven cases malrotation was present in three. Surgical correction was done by an abdominal approach in nine cases and a thoracic approach in one. The diaphragmatic defect was repaired by transverse closure in six cases, diaphragm plication in three and prosthetic closure in one. The postoperative outcome was uneventful in eight cases. Two patients died. Thus, RCDH seems to cause less severe symptoms than left-sided LCDH. It usually manifests beyond the neonatal period as respiratory or gastrointestinal symptoms. The diagnosis should be made easily by a CxR. The presence of a hernia sac correlated with a mild presentation. An abdominal surgical approach is preferred. Author Affiliation: (1) Pediatric Surgery Division, Hotel Dieu De France Hospital, Achrafieh, Lebanon (2) Pediatric Intensive Care Unit, Hotel Dieu De France Hospital, Achrafieh, Lebanon (3) C/O Hotel Dieu De France, P.O Box 166830, Achrafieh, Lebanon Article History: Accepted Date: 19/02/2002 Online Date: 15/04/2003

Byline: Anurag Krishna (1), Noorullah Zargar (1) Keywords: Congenital diaphragmatic hernia Bochdalek hernia Laparoscopy Repair Abstract: The standard treatment of congenital diaphragmatic hernia is by open operation. We have successfully performed a laparoscopic repair in an asymptomatic 2-year-old child. We recommend this as a safe alternative treatment modality in diaphragmatic hernias presenting past infancy. The role of laparoscopic correction in the newborn period, however, remains to be determined. Author Affiliation: (1) Department of Pediatric Surgery, Sir Ganga Ram Hospital, New Delhi, India (2) 6/15, Shanti Niketan, New Delhi 110 021, India Article History: Accepted Date: 24/10/2001 Article note: Electronic Publication

Byline: W. D. A. Ford (1), C. P. Kirby (1), C. S. Wilkinson (2), M. E. Furness (3), A. J. Slater (4) Keywords: KeywordsaFetal diaphragmatic hernia; Steroids Abstract: aCongenital diaphragmatic hernia (CDH) is a common abnormality affecting 1 in 2,000 gestations. The mortality exceeds 50% despite recent advances in postnatal treatment. The widespread antenatal use of glucocorticoids to induce lung maturation in fetuses at risk of premature delivery suggests a potential for a therapeutic effect in other fetuses with impaired lung development. The parents of three fetuses referred with CDH and features suggesting a poor postnatal prognosis (early diagnosis, liver herniation, and lung area-to-head circumference ratio &lt 1.0, or associated abnormalities) elected to receive maternal betamethasone starting at 24 to 26 weeks' gestation rather than undergo a fetal tracheal plug. All three infants survived and were extubated within 10 days. The long-term use of antenatal steroids in the treatment of CDH may thus be of benefit and warrants further study. Author Affiliation: (1) Department of Paediatric Surgery, Women's and Children's Hospital, Adelaide, Australia, AU (2) Department of Perinatal Medicine, Women's and Children's Hospital, Adelaide, Australia, AU (3) Department of Women's Ultrasound, Women's and Children's Hospital, Adelaide, Australia, AU (4) Department of Paediatric Intensive Care, Women's and Children's Hospital, Adelaide, Australia, AU (5) Department of Paediatric Surgery, Women's and Children's Hospital, 72 King William Road, North Adelaide SA 5006, Australia, AU Article note: Accepted: 26 February 2001

Infectious complications after surgical treatment for congenital diaphragmatic hernia (CDH) are rare. (1) We present the case of a patient with a nonhealing skin fistula following surgical treatment for CDH. [...]

Case report An 85-year-old woman presented with weight loss and a change in bowel habit over the preceding 3 months. Colonoscopy showed several benign-looking polyps in the transverse colon, which [...]

ABBREVIATIONS. CDH, congenital diaphragmatic hernia; RCT, randomized controlled trial; ECMO, exfracorporeal membrane oxygenation. The article of Harrison et al (1,2) is a major contribution to the literature despite its disappointing [...]

Byline: A. K. Sharma (1), Sunil Kumar Kothari (1), Chavi Gupta (1), Prema Menon (1), Akshay Sharma (1) Keywords: KeywordsaRight diaphragmatic rupture; Blunt abdominal trauma Abstract: aThe diagnosis of right-sided diaphragmatic rupture (RDR) due to blunt abdominal trauma is often missed in the acute setting, especially in the absence of other thoracoabdominal injuries. We describe two such children. The problems associated with the diagnosis and management of RDR are discussed to emphasize the need to have a high index of suspicion for this entity. Author Affiliation: (1) Department of Pediatric Surgery, Sir Padampat Mother and Child Health Institute, S.M.S. Medical College, Jaipur-302004, Rajasthan, India, IN