Congenital Diaphragmatic Hernia: Update on Regional Experience

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Khalid Al-Umran, CABP; Saleem Khawaja, FRCS; Adekunle H. Dawodu, FRCP(Ed); Abdulatif Al-Arfj, Fachartz From the Departments of Pediatrics (Drs. Al-Umran and Dawodu), and Surgery (Drs.

1 Khalid Al-Umran, CABP; Saleem Khawaja, FRCS; Adekunle H. Dawodu, FRCP(Ed); Abdulatif Al-Arfj, Fachartz From the Departments of Pediatrics (Drs. Al-Umran and Dawodu), and Surgery (Drs. Khawaja and Al-Arfaj), College of Medicine and Medical Sciences, King Faisal University, Dammam. Address reprint requests and correspondence to Dr. Al-Umran: King Fahd Hospital of the University, P.O. Box 40140, Al-Khobar 31952, Saudi Arabia. Accepted for publication 8 October A consecutive series of 34 patients with congenital diaphragmatic hernia are reported from a tertiary neonatal care unit in the Eastern Province. All the patients presented within the first 24 hours of life with left-sided defect. The overall mortality (56%) and postoperative mortality (44%) are comparable with reports from other centers. The outcome of treatment correlated well with the age on presentation, the size of the defect, and preoperative blood gas results. Our approach to management has been modified lately, in the light of recent experience from other centers. The surgery is delayed until the neonate is stabilized with biochemical values of PaCO 2 = 45 torr or less; ph = 7.2 or higher; and PaO 2 = 50 torr or more. This management policy did not adversely affect overall survival. The problems encountered with patient transfer and the need for continued improvement in the organization of regionalized care are highlighted. K Al-Umran, S Khawaja, AH Dawodu, A Al-Arfj, Congenital Diaphragmatic Hernia: Update on Regional Experience. 1991; 11(5): Congenital diaphgragmatic hernia (CDH), diagnosed antenatally or presenting within the first 24 hours of life, is associated with a high mortality despite improved anesthesia and early surgical correction of anatomical defects [1,2]. The cause of death is usually hypoxia and respiratory failure due to high pulmonary vascular resistance and lung hypoplasia [3]. We identified lack of team approach and inadequate intensive care as additional contributory factors for unacceptably highmortality in a previous communication involving a small number of patients [4]. In view of the poor outcome, despite early surgery among infants with CDH, attention has shifted to extending the period of stabilization to correct hypoxia, acidosis and hypotension before surgery. This policy has not adversely affected survival [5] and in some studies has significantly reduced mortality [6]. Thus, a preliminary report by management has included improved team efforts and intensive care facilities and, lately, adoption of an approach to delay surgery in an attempt to improve oxygenation, acid-base and hemodynamic status [5,6]. This report represents an update of the epidemiological profile of a larger number of patients with CDH presenting within the first 24 hours of life at a tertiary center in the Eastern Province of Saudi Arabia. It also documents the outcome of changes in the management policy. Material and Methods The neonatal intensive care unit (NICU) at King Fahd Hospital of the University (KFHU), Al-Khobar, serves as the main Level III facility in the Eastern Province of Saudi Arabia. Since it opened in 1983, we have managed and studied 34 consecutive infants with CDH who were admitted with respiratory distress within the first 24 hours of life. Most patients were transferred from other hospitals. Referral arrangements were usually made by telephone, and infant transportation was provided by referring hospitals.

2 On arrival to the NICU, a thorough clinical assessment of each infant was made. Endotracheal intubation was performed when indicated, a nasogastric tube was passed to decompress the stomach, and a chest x-ray was taken to verify endotracheal tube placement and to rule out pneumothorax. Umbilical arterial catheter placement and, when necessary, radial artery sampling were performed for monitoring of blood gases. Respiratory support was provided using as low a post-inspiratory pressure (PIP) and positive end expiratory pressure (PEEP) as possible and at breathing rates of 60-90/min. Muscle relaxants were used when indicated. Volume expanders and inotropics (dopamine) were used when the systolic blood pressure was below 50 torr. Metabolic acidosis was corrected with bicarbonate therapy. In the first three years ( ) of the study, patients were operated on as soon as arrangements for surgery could be completed (Group 1). From 1986, however, surgery was delayed until the patient s respiratory status was stabilized in an attempt to achieve PaCO 2 of less than 45 torr, ph of greater than 7.2 and PaO 2 of greater than 50 torr. Surgery consisted of abdominal approach to reduce the hernia and close the diaphragmatic defect. The size of the defect was empirically classified as: large when 9 cm or more in diameter, and small if less. Prosthesis (Marlex mesh) was used in the closure of the defect when necessary. On the completion of reduction and repair, an ipsilateral chest tube was inserted. The patients were then returned to the NICU where respiratory and other intensive care supports were continued. The data were processed in a Data General computer using SAS software, and the results were analyzed using Student s t test and Fisher s exact test where appropriate. Results Of the 34 infants studied, 6 were born in this hospital and 28 born elsewhere. The incidence of CDH, based on births in this hospital was 1/3,000 live births. There were 27 males and seven females. All but three were delivered at full term. Average birth weight was 2,950 grams. Nineteen of the referred patients had no nasogastric tube on arrival, and nine who required endotracheal intubation had not been intubated. Only 20 of the referred patients were adequately escorted. Chest x-ray confirmed diaphragmatic hernia in all patients. All hernias were left-sided. Associated anomalies were present in 14 patients (41%); the most common anomaly was intestinal malrotation. Cardiac malformations (including one true dextrocardia) were seen in six, and multiple anomalies seen in four. In two infants there was concomitant congenital hypertrophic pyloric stenosis. Factors which influenced the outcome were age at diagnosis (Table 1), the presence of hypothermia on admission, the size of the defect, preoperative blood gases (Table 2), and the predetermined change in management policy (Table 3). Infants with an age of six-hours or less were associated with poor outcomes and postoperative mortality exceeding 50%. Survival among those who presented after the age of six hours was 67%. Overall mortality was 56% (Table 1). Seven children died without surgery, and postoperative mortality was 44%. Hypothermia (35.5 C or less), which occurred in 50% of the referred cases, was associated with higher mortality when compared with the presence of normothermia on arrival (80% versus 30%, P = 0.05). Of the 27 infants who were operated on, 16 were considered to have large defects and mortality among these was 56%, compared with those with small defects (27%). The difference was, however, not significant. Preoperative blood gas values correlated with outcome. All the three parameters, ph 7.0 or less, PaCO 2 of 80 torr or more, and PO 2 of 35 torr or less were highly predictive of nonsurvival. A multivariate analysis revealed factors which did not influence the outcome, including Apgar scores or whether the infant was born in this hospital or outside the hospital. The effect of extended preoperative stabilization period showed a trend towards improved postoperative survival, but the difference was not statistically significant. Discussion The incidence of CDH, as determined by the hospital population, is similar to that reported in the literature [3]. The study also confirms the preponderance of the left-sided hernia among those seen in the newborn period. Although no sex preference has been consistently reported, most of our patients (80%) were males.

3 Age on presentation Table 1. Age at presentation and outcome. No. pts. Mortality Preoperative Postoperative Total < 6hrs (53) 14 (67) 6-24 hrs (33) 5 (38) Total (44) 19 (56) P value < Table 2. Preoperative blood gas results and outcome. Blood gas results Survivors Non-survivors P value ph ± ± PaCO 2 torr 60 ± ± PO2 112 ± ± Mean, ± SEM. Group* Group I Group II Table 3. Outcome during the two periods of study. No. pts. Mortality Overall Preoperative Postoperative (54) 9 (60) (36) 10 (53) P value < *The two groups were comparable in terms of gestational age, birth weight, sex, laterality, age at diagnosis, intubation and condition on transfer. The prognosis in neonatal CDH remains a disturbing question. Of the many factors which are known to influence the outcome of management, the age at diagnosis (< 6 hours) and the preoperative blood gas values (ph 7.0, PCO2 60 torr, and P02 80 torr) are the most frequently reported predictors of poor outcome [3,4,7]. These observations were also confirmed in this series. The overall mortality in the present study is similar to that reported in the literature, surpassing the figure of 80% as reported in our earlier communication [4]. The decrease in mortality was attributed to improvement in neonatal management. A striking feature in our series which significantly increased the mortality was the prevalence of hypothermia among referred cases from other hospitals in the region. An apparent reason for this was considered to be due to inadequate transport. The guidelines for transporting critically ill neonates are known [8], and an optimal system of transport is expected to improve the hemodynamic status of as many as 40% of the neonates [9]. Further improvement in this aspect of care in the region requires organization of perinatal care education at the local and regional levels. Optimally, various health organizations in the region should pool resources to develop two to three tertiary units whose responsibility would include the transportation of high-risk neonates in the framework of national regionalization of perinatal care [10]. Furthermore, improved antenatal care surveillance should allow antenatal diagnosis and in utero transfer, thus minimizing the added risks of neonatal transportation. Until recently, there has been a mythical acceptance of CDH as a precipitate surgical emergency in which hernia contents must be promptly reduced to allow expansion of the compressed lung. This view, however, is rapidly changing with increasing knowledge of disease behavior. In Ruff et al s [11] analysis, children with CDH, over eleven years, demonstrated no direct relationship between survival and the timing of surgery. There is

4 supporting evidence proving not all children having identical and severe hypoplasia of the lungs succumb to a delay in surgery [12]. Moreover, recent experiences documented in Toronto, Canada [5,13], Rotterdam, The Netherlands [14], and Nottingham, UK [6] have shown that: (a) emergency surgical repair is often followed by deterioration rather than improvement, which has been our experience as well; (b) there is a distinct possibility that unsatisfactory respiratory parameters may improve with appropriate resuscitative measures; (c) by delaying surgery, time is available in which to detect and treat such situations as persistent fetal circulation (PFC); and (d) poor hemodynamic and ventilatory status on admission which does not respond to appropriate resuscitation and usually fails to improve with surgery. These experiences and observations have lent support to the strategy of preoperative stabilization and delayed surgery, and the results are encouraging. In 1986 we opted for this line of management in our unit. Although the overall survival rate is not significantly different from the earlier period of study, there is a clear trend towards declining postoperative mortality (Table 3). Our goal is to achieve preoperative blood gas values of PaCO 2 of 45 torr before surgery. If necessary, we use a conventional ventilator with high breathing rate of /min in resistant hypercarbia. Congenital diaphragmatic hernia is one of the frequently encountered respiratory emergencies in our environment. The management of a hypoplastic lung in a liveborn poses a great challenge. Appropriate strategies must be adopted in diagnosing and treating various complications that may arise from the use of excessive ventilatory pressures, increased pulmonary vascular resistance, and pharmacotherapeutic agents. We have used conventional means (umbilical artery catheterization and radial artery sampling) in diagnosing persistent fetal circulation (PFC), but its exact frequency in our unit is not known. A wider use of pulse oximeter and M mode echocardiography may help to detect PFC more frequently and reasonably early. Although we continue to use tolazoline in treating PFC, our experience has not been encouraging, probably because PFC was usually a terminal event in many of our patients. The exact incidence of CDH and other surgically correctable lesions in Saudi Arabia is not known. The hospital-based figures are crude. Moreover, in the case of CDH there is a large element of hidden mortality [15] and some cases may not be diagnosed before death. The local experience suggests that, of the major congenital malformations encountered among the liveborn infants, one third will require surgical intervention [16]. This knowledge brings into focus the need for a national registry on congenital malformations. The data collection may commence at regional levels. This can then form a basis for cumulative information on surgically correctable malformations which can be used to chart future strategies in national health planning. References 1. Simson JNL, Eckstein HB. Congenital diaphragmatic hernia: a 20-year experience. Br J Surg 1985;72: Adzick NS, Harrison MR, Glick PL, et al. Diaphragmatic hernia in the fetus: prenatal diagnosis and outcome in 94 cases. J Pediatr Surg 1985;20: Cullen ML, Klein MD, Philippart Al. Congenital diaphragmatic hernia. Surg Clin North Am 1985;65: Khwaja S, Al-Breiki H, Grant C, et al. Congenital diaphragmatic hernia. Saudi Med J 1985;6: LangerJC,FillerRM,Bohn DJ, Shandling B, et al. Timing of surgery for congenital diaphragmatic hernia: is emergency operation necessary? J Pediatr Surg 1988;23: Cartlidge PHT, Mann NP, Kapila L. Preoperative stabilization in congenital diaphragmatic hernia. Arch Dis Child 1986;61: Adelman S, Benson CP. Bochdalek hernias in infants: factors determining mortality. J Pediatr Surg 1973;11: Bush GH, Dangel P, Rickham PP. Neonatal physiology and its effect on pre- and postoperative management. In: Neonatal Surgery, ed 2. Rickham PP, Lister J, Irving IM, eds. Boston, London: Butterworth, 1980; Marshall A, Sumnar E. Improved prognosis in congenital diaphragmatic hernia: experience of 62 cases over a two-year period. J Royal Soc Med 1982;75: Al-Faraidy A, Dawodu AH, Uduman SA, et al. Survey of newborn care facilities in the Kingdom of Saudi Arabia. King Abdul Aziz City for Science and Technology, Report 12. Riyadh, Ruff SJ, Campbell JR, Harrison MR, Campbell T. Pediatric diaphragmatic hernias: an 11-year experience. Am J Surg 1980;139: Wiener ES. Congenital posterolateral diaphragmatic hernia: new dimensions in management. Surgery

5 1982;92: Sakai K, Tamura M, Hosokawa Y, et al. The effect of surgical repair on respiratory mechanics in congenital diaphragmatic hernia. J Pediatr 1987;3: Hazebroek FWJ, Tibboel D, Box AP, et al. Congenital diaphragmatic hernia: impact of preoperative stabilization: a postoperative pilot study in 13 patients. J Pediatr Surg 1988;23: Harrison MR, Bjordal RI, Landmark F, Knutrud O. Congenital diaphragmatic hernia: the hidden mortality. J Pediatr Surg 1987;13: Magbool G, Al-Mulhim I, Uduman SA, et al. Congenital anomalies in liveborn Saudi infants. Emirates Med J 1989;7:7-10.

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