Interventional procedure consultation document

Thoracoscopic repair of congenital diaphragmatic hernia in neonates

Treating hernia of the diaphragm in newborn babies using keyhole surgery through the chest

Congenital diaphragmatic hernia is a life-threatening condition caused by failure of the diaphragm to form properly during a baby’s development in the womb. This results in the baby’s abdominal organs moving up into the chest cavity, compressing its lungs and heart. In thoracoscopic repair, small incisions are made in the chest to insert surgical instruments, with the aim of moving the abdominal organs out of the chest and repairing the diaphragm.

The National Institute for Health and Clinical Excellence (NICE) is examining thoracoscopic repair of congenital diaphragmatic hernia in neonates and will publish guidance on its safety and efficacy to the NHS in England, Wales, Scotland and Northern Ireland. NICE’s Interventional Procedures Advisory Committee has considered the available evidence and the views of Specialist Advisers, who are consultants with knowledge of the procedure. The Advisory Committee has made provisional recommendations about thoracoscopic repair of congenital diaphragmatic hernia in neonates.

This document summarises the procedure and sets out the provisional recommendations made by the Advisory Committee. It has been prepared for public consultation. The Advisory Committee particularly welcomes:

• comments on the provisional recommendations
• the identification of factual inaccuracies
• additional relevant evidence, with bibliographic references where possible.

Note that this document is not NICE’s formal guidance on this procedure. The recommendations are provisional and may change after consultation.

The process that NICE will follow after the consultation period ends is as follows.

• The Advisory Committee will meet again to consider the original evidence and its provisional recommendations in the light of the comments received during consultation.
• The Advisory Committee will then prepare draft guidance which will be the basis for NICE’s guidance on the use of the procedure in the NHS in England, Wales, Scotland and Northern Ireland.

For further details, see the Interventional Procedures Programme manual, which is available from the NICE website (www.nice.org.uk/ipprogrammemanual).

NICE is committed to promoting through its guidance race and disability equality and equality between men and women, and to eliminating all forms of discrimination. One of the ways we do this is by trying to involve as wide a range of people and interest groups as possible in the development of our guidance on interventional procedures. In particular, we aim to encourage people and organisations from groups in the population who might not normally comment on our guidance to do so. We also ask consultees to highlight any ways in which draft guidance fails to promote equality or tackle discrimination and give suggestions for how it might be improved. NICE reserves the right to summarise and edit comments received during consultations, or not to publish them at all, where in the reasonable opinion of NICE, the comments are voluminous, publication would be unlawful or publication would otherwise be inappropriate.

Closing date for comments: 28 September

Target date for publication of guidance: January 2010

1 Provisional recommendations

1.1 Current evidence on the safety and efficacy of thoracoscopic repair of congenital diaphragmatic hernia (CDH) in neonates is adequate to support the use of this procedure provided that normal arrangements are in place for clinical governance, consent and audit.

1.2 During the consent process, parents should be informed in particular about the possibility of conversion to abdominal repair and about the risk of recurrence.

1.3 This procedure should only be carried out by surgeons with specific training and experience in laparoscopic and thoracoscopic surgery in neonates and children.

1.4 NICE encourages collaboration between the units performing this procedure in the collection of data and publication of results.

2 The procedure

2.1 Indications and current treatments

2.1.1 Congenital diaphragmatic hernia results from failure of complete fusion of the developing fetal diaphragm – a process that normally occurs between gestational weeks 6 and 8. The defect may be anterior (Morgagni’s hernia) or more commonly, posterolaterally (Bochdalek hernia). Migration of abdominal organs into the thoracic cavity can lead to pulmonary hypoplasia, hypertension and respiratory failure at birth.

2.1.2 Current management of CDH in neonates usually involves initial ventilatory support and supportive care, to allow labile cardiopulmonary physiology to improve, followed by surgical reduction of the hernia, usually through an abdominal approach, and repair of the diaphragmatic defect.

2.2 Outline of the procedure

2.2.1 The aim of this procedure is to reduce the herniated abdominal organs and repair the diaphragmatic defect.

2.2.2 Thoracoscopic repair of CDH in neonates is carried out with the patient under general anaesthesia and in the lateral decubitus position. Between 2 and 4 trocars can be used, with CO₂ insufflation of the pleural space to partially collapse the lung sufficiently to achieve good exposure of the defect and to reduce the herniated viscera within the abdomen. Following the reduction of the herniated content, the diaphragm is repaired using non-absorbable interrupted sutures (for small defects) or patches (if defects are relatively large). Where technically possible, posterolateral diaphragm stitches are passed around the posterolateral ribs and tied extracorporeally. Patients usually require temporary chest drain insertion and ventilatory support following the procedure.

Sections 2.3 and 2.4 describe efficacy and safety outcomes from the published literature that the Committee considered as part of the evidence about this procedure. For more detailed information on the evidence, see the overview, available at www.nice.org.uk/IP847overview

2.3 Efficacy

2.3.1 A meta-analysis of 3 non-randomised comparative studies including a total of 143 patients treated by thoracoscopic (n = 62) or open (n = 81) repair reported recurrence rates of 16% (10/62) and 5% (4/81) respectively (follow-up not stated), with a recurrence risk ratio of 3.21 (95% confidence interval [CI] 1.11 to 9.29). Non-randomised comparative studies of 57 and 48 patients treated by thoracoscopic (n = 29, n = 13) or open (n = 28, n = 35) repair reported recurrence rates of 21% (6/29) and 25% (2/8) in the thoracoscopic groups compared with 7% (2/28) and 8% (3/40) in the open repair groups respectively (p = 0.25, p = 0.19 respectively): follow-up intervals were not stated.

2.3.2 A non-randomised comparative study of 30 patients treated by thoracoscopic (n = 18) or laparoscopic repair (n = 12) reported ‘easy reduction’ of the hernia in 83% (15/18) and 42% (5/12) of patients respectively; ‘difficult reduction’ in 11% (2/18) and 33% (4/12); and that it was impossible to reduce the hernia contents in 6% (1/18) and 25% (3/12) of patients respectively. A case series of 45 patients reported that reduction of hernia contents was ‘easily accomplished’ in 67% (30/45) of patients.

2.3.3 The non-randomised comparative study of 57 patients reported conversion from a thoracoscopic procedure to an open procedure in 1 patient because the liver could not be reduced into the abdomen. The case series of 45 patients reported conversion to an open procedure in 9% (4/45) of patients; 3 conversions were due to difficulty in reducing the hernia content into the abdominal cavity and 1 was due to a decrease in oxygen saturation.

2.3.4 Median duration of postoperative ventilation was 2 days after thoracoscopic repair and 4 days after open repair in the non-randomised comparative study of 73 patients (p = 0.04), but it was similar in the 2 groups in the study of 57 patients (5 days in each group; p = 0.56).

2.3.5 The Specialist Advisers listed key efficacy outcomes as reduction in postoperative abdominal adhesions, improved postoperative pain, duration of hospital stay, resumption of enteral nutrition and cosmetic appearance.

2.4 Safety

2.4.1 A mortality rate of 3% (2/62) in the thoracoscopic group compared with 12% (10/81) in the open group with a mortality risk ratio of 0.33 (95% CI 0.01 to 1.13) was reported in the meta-analysis of 143 patients (follow-up not stated). The non-randomised comparative study of 57 patients reported no significant difference in postoperative mortality rates between the thoracoscopic group and the open group (7% [2/29] vs 21% [6/28]; p = 0.14).

2.4.2 Death due to haemorrhage in 1 patient was reported in the open group of the non-randomised comparative study of 73 patients at a median of 37 months follow-up. Death after severe bronchopneumonia and pneumothorax was reported in 1 patient each in the case series of 45 patients (timing of events not stated).

2.4.3 The non-randomised comparative study of 57 patients reported no significant difference in major infection rates (defined as abscess, systemic sepsis or abdominal wall patch infection) in the thoracoscopic group compared with the open group (17% [5/29] vs 4% [1/28], p = 0.19) (not otherwise described).

2.4.4 Gastrointestinal perforation rates of 7% (2/29) in the thoracoscopic group and 7% (2/28) in the open group were reported in the non-randomised comparative study of 57 patients.

2.4.5 The Specialist Advisers considered theoretical adverse events to include solid/hollow visceral injury, physiological instability, inability to detect malrotation and complications of volvulus, and hypercarbia if not carefully insufflated.

2.5 Other comments

2.5.1 The Committee considered evidence that included some infants over 30 days but less than 12 months.

3 Further information

3.1 For related NICE guidance see www.nice.org.uk

Bruce Campbell

Chairman, Interventional Procedures Advisory Committee

September 2010

_{Personal data will not be posted on the NICE website. In accordance with the Data Protection Act names will be anonymised, other than in circumstances where explicit permission has been given.}

_{It is the responsibility of consultees to accurately cite academic work in order that they can be validated.}