Long-term follow up of infants with congenital diaphragmatic hernia

https://doi.org/10.1053/j.sempedsurg.2007.01.009Get rights and content

With improving treatment strategies for congenital diaphragmatic hernia (CDH) infants, an increase in survival of more severely affected patients can be expected. Consequently, more attention is now focused on long-term follow up of these patients. Many reports have emphasized associated morbidity, including pulmonary sequelae, neurodevelopmental deficits, gastrointestinal disorders, and other abnormalities. Therefore, survivors of CDH remain a complex patient population to care for throughout infancy and childhood, thus requiring long-term follow up. Much information has been provided from many centers regarding individual institutional improvements in overall survival. Few of these, however, have reported long-term follow up. The aim of this review is to describe the long-term outcome of survivors with CDH and to suggest a possible follow-up protocol for these patients.

Section snippets

Pulmonary outcome

Most adolescent CDH survivors are healthy and enjoy normal lives. However, in a significant proportion of patients, long-term pulmonary sequelae are detectable and dependent on the severity of lung hypoplasia and the degree of lung injury.8, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23

Before the introduction of “advanced” treatment modalities (ECMO, gentle ventilation, HFV, iNO), long-term CDH survivors treated with conventional mechanical ventilation were reported healthy and without

Neurological outcome

A variable, but significant proportion of CDH survivors have neurological sequelae (Table 1), including developmental delay, motor, cognitive, and behavioral disorders.11, 15, 23, 25, 28, 30, 46, 47, 48, 49 Several factors are associated with neurodevelopmental outcome in CDH survivors. The need for ECMO seems related to a poorer neurological outcome. Some authors suggest that CDH patients treated with ECMO do less well than infants treated with ECMO for other reasons. Bernbaum and coworkers46

Gastrointestinal outcome

Gastroesophageal reflux (GER), with all its associated nutritional (and pulmonary) consequences, is the most common sequela.15, 65, 66, 67, 68, 69, 70 This condition was rarely referred to in association with CDH before 1990,66 but recent studies have shown that GER is commonly found in short- and long-term survivors of CDH.69, 70 A review of the incidence of GER among CDH patients in different studies is reported in Table 1. These data suggest that nearly 40% of babies operated on for CDH will

Musculoskeletal outcome

The close relationship between development of the lung, diaphragm, and thoracic cage is highlighted by the frequency of musculoskeletal abnormalities87, 88, 89 in children who have undergone CDH repair. Chest asymmetry and pectus deformity are the most commonly described, followed by vertebral deformity (scoliosis and kyphosis).10, 11, 16, 19, 22, 33, 58, 83, 90 Because chest wall and spinal deformities develop throughout the entire growth period, their true incidence may be defined only when

Quality of life

As previously stated, CDH patients face life-threatening morbidity during the neonatal period and some CDH-related symptoms during their first years of life.34, 58, 95 Disease-specific symptoms, not unique to CDH patients, are more frequently reported in CDH patients than in the general population. Poley at al96 found a significantly higher symptom score for CDH patients under the age of 19 (mean score 6.8) than for their contemporaries in the control group (mean score 4.9). Moreover, analyzing

References (98)

  • J.A. D’Agostino et al.

    Outcome for Infants with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: the first year

    J Pediatr Surg

    (1995)
  • K. Vanamo et al.

    Long-term pulmonary sequelae in survivors of congenital diaphragmatic defects

    J Pediatr Surg

    (1996)
  • C.S. Muratore et al.

    Pulmonary morbidity in 100 survivors of congenital diaphragmatic hernia monitored in a multidisciplinary clinic

    J Pediatr Surg

    (2001)
  • S. Jaillard et al.

    Outcome at 2 years of infants with congenital diaphragmatic hernia: a population-based study

    Ann Thorac Surg

    (2003)
  • G. Stefanutti et al.

    Cardiopulmonary anatomy and function in long-term survivors of mild to moderate congenital diaphragmatic hernia

    J Pediatr Surg

    (2004)
  • S. Kamata et al.

    Long-term follow-up of patients with high-risk congenital diaphragmatic hernia

    J Pediatr Surg

    (2005)
  • R.A. Cortes et al.

    Survival of severe congenital diaphragmatic hernia has morbid consequences

    J Pediatr Surg

    (2005)
  • P.J. Davis et al.

    Long-term outcome following extracorporeal membrane oxygenation for congenital diaphragmatic hernia: the UK experience

    J Pediatr

    (2004)
  • J.B. Atkinson et al.

    ECMO and the management of congenital diaphragmatic hernia with large diaphragmatic defects requiring a prosthetic patch

    J Pediatr Surg

    (1992)
  • H.L. Hedrick et al.

    Right congenital diaphragmatic hernia: prenatal assessment and outcome

    J Pediatr Surg

    (2004)
  • N. Ssemakula et al.

    Survival of patients with congenital diaphragmatic hernia during the ECMO era: an 11-year experience

    J Pediatr Surg

    (1997)
  • K.P. Van Meurs et al.

    Congenital diaphragmatic hernia: long-term outcome in neonates treated with extracorporeal membrane oxygenation

    J Pediatr

    (1993)
  • D. Bohn et al.

    Ventilatory predictors of pulmonary hypoplasia in congenital diaphragmatic hernia, confirmed by morphologic assessment

    J Pediatr

    (1987)
  • M. Nagaya et al.

    Development in lung function of the affected side after repair of congenital diaphragmatic hernia

    J Pediatr Surg

    (1996)
  • T.M. Tsang et al.

    Diaphragmatic agenesis as a distinct clinical entity

    J Pediatr Surg

    (1995)
  • R.L. Moss et al.

    Prosthetic patch durability in congenital diaphragmatic hernia: a long-term follow-up study

    J Pediatr Surg

    (2001)
  • E.D. McGahren et al.

    Neurological outcome is diminished in survivors of congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation

    J Pediatr Surg

    (1997)
  • T.A. Nield et al.

    Neurodevelopmental outcome at 3.5 years of age in children treated with extracorporeal life support: relationship to primary diagnosis

    J Pediatr

    (2000)
  • A. Dotta et al.

    Effects of surgical repair of congenital diaphragmatic hernia on cerebral hemodynamics evaluated by near-infrared spectroscopy

    J Pediatr Surg

    (2005)
  • K.K. Nobuhara et al.

    Long-term outlook for survivors of congenital diaphragmatic hernia

    Clin Perinat

    (1996)
  • A. Rasheed et al.

    Neurodevelopmental outcome after congenital diaphragmatic hernia: extracorporeal membrane oxygenation before and after surgery

    J Pediatr Surg

    (2001)
  • R.W. Hunt et al.

    Patterns of cerebral injury in a series of infants with congenital diaphragmatic hernia utilizing magnetic resonance imaging

    J Pediatr Surg

    (2004)
  • C.S. Muratore et al.

    Nutritional morbidity in survivors of congenital diaphragmatic hernia

    J Pediatr Surg

    (2001)
  • S.D. West et al.

    Follow up of infants with congenital diaphragmatic hernia

    Semin Perinatol

    (2005)
  • C.J.H. Stolar et al.

    Anatomic and functional abnormalities of the esophagus in infants surviving congenital diaphragmatic hernia

    Am J Surg

    (1990)
  • C.S. Muratore et al.

    Congenital diaphragmatic hernia: where are we and where do we go from here?

    Semin Perinatol

    (2000)
  • D.L. Sigalet et al.

    Gastroesophageal reflux associated with large diaphragmatic hernias

    J Pediatr Surg

    (1994)
  • L. Schoeman et al.

    Late death after extracorporeal membrane oxygenation for congenital diaphragmatic hernia

    J Pediat Surg

    (1999)
  • M. Nagaya et al.

    Gastroesophageal reflux occurring after repair of congenital diaphragmatic hernia

    J Pediatr Surg

    (1994)
  • B. Qi et al.

    An experimental study on the pathogenesis of gastroesophageal reflux after repair of diaphragmatic hernia

    J Pediatr Surg

    (1997)
  • S. Naik et al.

    Prediction of morbidity during infancy after repair of congenital diaphragmatic hernia

    J Pediatr Surg

    (1996)
  • L.M. Kohr et al.

    The incidence of dysphagia in pediatric patients after open heart procedures with transesophageal echocardiography

    Ann Thorac Surg

    (2003)
  • K. Vanamo et al.

    Long-term gastrointestinal morbidity in patients with congenital diaphragmatic defects

    J Pediatr Surg

    (1996)
  • J.S. Janik et al.

    An assessment of the surgical treatment of adhesive small bowel obstruction in infants and children

    J Pediatr Surg

    (1981)
  • M.R. Price et al.

    Altered diaphragm function modifies neonatal lung growth: biologic morphometric assessment

    J Pediatr Surg

    (1993)
  • K. Vanamo et al.

    Chest wall and spinal deformities in adults with congenital diaphragmatic defects

    J Pediatr Surg

    (1996)
  • I.A.F. Stokes et al.

    Three-dimensional osseo-ligamentous model of the thorax representing initiation of scoliosis by asymmetric growth

    J Biomechanics

    (1990)
  • M.J. Poley et al.

    The cost-effectiveness of treatment for congenital diaphragmatic hernia

    J Pediatr Surg

    (2002)
  • A.P. Metkus et al.

    Cost per anomaly: what does a diaphragmatic hernia cost?

    J Pediatr Surg

    (1995)
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