Archives of International Surgery

ORIGINAL ARTICLE
Year
: 2019  |  Volume : 9  |  Issue : 2  |  Page : 34--38

Late presentation of congenital diaphragmatic hernia in children: A single-institution experience


Yogender S Kadian, Chandroday Kumar, Pradeep Kajal, Mahavir Jangra 
 Department of Paediatric Surgery and Surgery, Pt. B.D. Sharma PGIMS, Rohtak, Haryana, India

Correspondence Address:
Dr. Yogender S Kadian
8-UH, Medical Campus, PGIMS, Rohtak, Haryana - 124 001
India

Abstract

Background: Congenital diaphragmatic hernia (CDH) with late presentation beyond the neonatal period is rare and often misdiagnosed, with delayed treatment. We report the management of a series of patients that presented after the neonatal period. Patients and method: we reviewed our experience over 4 years (2015-2019) in a single institution to determine the factors for late presentation of congenital diaphragmatic hernia, associated anomalies, complications and its management. Results: We had six patients diagnosed as late presenting CDH and operated in our institution over 4 years . The patients ranged in age from one year to 10 years with males to females ratio 2:1. All the hernia defects were left sided. Except one patient all have abnormal gut rotation. The mode of presentations were ; one patient presented with gastric perforation due to misdiagnosis of pneumothorax and ICD placement. Another one had irreducible gut gangrene, 2 cases have left lobe of liver along with small bowel, colon and spleen and stomach, The other 2 patients had small bowel, colon and spleen and stomach as hernia content. All cases were managed by primary repair of defect with interrupted non absorbable suture through transabdominal approach. Two cases had post op pleural effusion and pneumothorax and required intercostal tube placement, and all the patient were kept in intensive care for 48 hours but without any ventilator support. Conclusions: There should be a high index of suspicion in every case presenting with persistent vomiting along with abdominal pain, recurrent pneumonia, chronic respiratory conditions or acute abdominal condition with doubtful x-ray chest.



How to cite this article:
Kadian YS, Kumar C, Kajal P, Jangra M. Late presentation of congenital diaphragmatic hernia in children: A single-institution experience.Arch Int Surg 2019;9:34-38


How to cite this URL:
Kadian YS, Kumar C, Kajal P, Jangra M. Late presentation of congenital diaphragmatic hernia in children: A single-institution experience. Arch Int Surg [serial online] 2019 [cited 2020 Sep 21 ];9:34-38
Available from: http://www.archintsurg.org/text.asp?2019/9/2/34/291748


Full Text



 Introduction



Congenital diaphragmatic hernia (CDH) is a common cause of respiratory distress in neonates. It was first described 150 years ago and occurs as early as 6–8 weeks of gestation.[1] Its estimated incidence has been reported as 1/2000–5000 live births.[2] It occurs due to incomplete closure of the pleuroperitoneal channel during fetal development. It is a life-threatening condition with a high mortality in the early neonatal period. However, with increasing awareness, most of the cases are diagnosed antenatally and managed accordingly. A few cases remain undiagnosed and present beyond the neonatal period wherein the risk of serious morbidity and mortality is reported to be less than early presentation.[3] About 5–30% of cases of CDH can present beyond the neonatal period.[4] However, it has been seen that these patients have a better outcome in comparison to the neonatal group. This study has been done to reinforce the argument of the favorable outcome of this subset of patients of CDH. In this manuscript, a retrospective analysis of patients with CDH regarding their late presentation, associated features, diagnosis, management, complications, and prognosis has been done.

 Patients and Method



This is a retrospective review of six children with a provisional diagnosis of CDH in the pediatric surgery department, in a north Indian public hospital from 2015–2019. All the patients were between the age group of 1 and 10 years. Patients with post-traumatic hiatus hernia, eventration of the diaphragm were excluded. Intensive care unit stay and postoperative ventilator care were applied depending on the patient's conditions. Each case was evaluated concerning age, sex, mode of presentation, site of the hernia, associated anomalies, diagnostic method, operative findings, postoperative complications, and prognosis.

 Results



During the study period of 4 years, 29 neonates and children were admitted with a diagnosis of congenital diaphragmatic hernia (CDH) out of which 6 (20.6%) had a late presentation which forms the subject of this presentation. The patient's age ranged from 1 to 10 years. The male to female ratio was 2:1. All the hernia defects were left-sided. Five patients had malrotation of the gut and one had Meckel's diverticulum as associated anomalies. The patients presented with nonspecific symptoms and signs, including vomiting, abdominal pain, dyspnoea, and cough in four patients. One patient presented with gastric perforation due to a misdiagnosis of pneumothorax and intercostal tube placed on the left side of the chest and another patient presented with acute abdomen and was found to have an irreducible gut gangrene as hernia content and was managed accordingly [Table 1].{Table 1}

The diagnosis was mainly made by chest X-ray and in one patient contrast-enhanced computed tomography (CECT) scan was needed for making a diagnosis [Figure 1]. All the cases were managed by primary repair of defect with interrupted nonabsorbable (prolene) suture through a transabdominal approach [Figure 2] and [Figure 3]. Initially, no intercostal tube was placed on the ipsilateral chest cavity; however, 2 cases had postoperative complications as pneumothorax and pleural effusion and required intercostal tube drainage [Figure 4] and [Figure 5]. No case required post-op ventilation but all the patients were kept in intensive care for 48 h. All patients were discharged in good condition and are on regular follow-up.{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}

 Discussion



Congenital diaphragmatic hernia occurs as a result of the failure of closure of the pleuroperitoneal canal during fetal development. With increasing awareness and ultrasonography (USG) most of these cases are diagnosed during the antenatal period, while some of them were diagnosed in the prenatal and early neonatal periods. However, 5–45.5% of cases may appear healthy during the neonatal period and manifest in later life.[5],[6] In the present study, 20.6% of patients were diagnosed as CDH beyond the neonatal period. The basic defect is identical in neonates and late presenting cases but the symptoms, management, and the complications are different in older children compared to the newborns. It is difficult to explain this pattern of behavior in CDH cases but the probable explanation may be herniation of gut loops which may occur beyond the neonatal period and also another reported feature in these cases is the rarity of associated lung hypoplasia.[7] In the literature, the male-to-female ratio was approximately 2: 1,[5] and in our study, the male-to-female ratio is also 2: 1. Diagnosis of CDH is usually made by chest X-ray but it is not always confirmatory as it may mimic other chest conditions such as lower lobe pneumonia, pneumothorax, pleural effusion, or a lung lesion thereby potentially leading to misdiagnosis.[8],[9] In these situations, contrast study or CECT scan may be needed for making a diagnosis. In the present study, CECT was done in one patient.

Infants with CDH are classified by the timing of herniation the severity of pulmonary hypoplasia, survival, and the presence of late pulmonary sequelae.[10] In the classification, type 1 refers to cases in which the herniation has occurred in the early period of rapid bronchial division. It results in bilateral pulmonary hypoplasia and perinatal death. Type 2 refers to cases in which the herniation has occurred in the late period of rapid bronchial division. It results in unilateral pulmonary hypoplasia. The neonatal outcome of this disease is characterized by pulmonary hypoplasia, potential pulmonary hypertension, and alterations of blood gas. Moreover, preoperative stabilization should be a priority in these cases of CDH with neonatal presentation and stabilization should be guided by precise cardiorespiratory criteria.[6],[10] Some of these cases die in the neonatal period, whereas, others survive with considerable pulmonary problems. Type 3 refers to cases in which the herniation has occurred during either late gestation or the early neonatal period. However, herniation might present at birth with mild respiratory distress. As a result, pulmonary hypoplasia is minimal or absent in these patients and most of them survive with no detectable respiratory problems.[9] In type 4, the herniation has presented after the neonatal period. They have no pulmonary hypoplasia and survive with no respiratory problems. In our study, most cases belong to type 3 and 4.

The secondary abnormalities representing the effects of herniated abdominal viscera in the thorax are nonfixation of the mesentery with malrotation of the intestinal tract and lung hypoplasia.[11] Therefore, the late-presenting type 3 group may have intestinal malrotation and pulmonary hypoplasia, whereas the type 4 group does not have any intestinal malrotation and pulmonary hypoplasia. The basic defect is identical in neonates and late presenting cases but the symptoms, management, and complications are different in older children than in newborns. In our study, malrotation of the gut was the most common secondary malformation. Two patients had postoperative complications as pneumothorax and pleural effusion as postoperative complications which were managed by intercostal tube drainage. Placement of the intercostal tube, post-repair is not required[12] in early presentation but some authors recommend that it should be put in all patients with the delayed presentation because of risk pleural effusion and ipsilateral lung collapse.[13],[14] In the present study, the intercostal tube was not placed at the time of repair of the hernial defect but it was needed in two patients because of pleural effusion in the postoperative period. From the experience of six patients although small in number, authors recommend that insertion of the intercostal tube should be part of the operative procedure in all the cases of late presenting CDH anomaly. However, patients with late-presenting CDH have a more favorable prognosis due to less severe or absent lung hypoplasia[9] as seen in the present study. Acute gastric volvulus associated with CDH is extremely rare but can have life-threatening complications, fortunately, none of our patients had this complication.

 Conclusion



A late-presentation of CDH is an uncommon condition in children beyond the neonatal period. Due to nonspecific symptoms, it is difficult to diagnose with simple chest X-ray, hence a high index of suspicion is needed to screen such patients and contrast study or CECT scan may be needed to make an early diagnosis and then surgical treatment. Moreover, these patients have a favorable outcome as compared to the neonatal age group CDH and placement of intercostal tube following the repair of this condition should be a routine in all such cases.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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