Congenital high airway obstruction syndrome successfully managed with <i>ex‐utero</i> intrapartum treatment

Oepkes, Dick; Teunissen, A. K. K.; Velde, Marc Van de; Devlieger, Hugo; Delaere, Pierre; Deprest, Jan

doi:10.1002/uog.899

Cited by 33 publications

(21 citation statements)

References 6 publications

(8 reference statements)

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“…In utero anatomical repair improves lung development, but it cannot be offered to fetuses with liver herniation: liver reduction would kink the umbilical vein and cause fetal death [12]. Another strategy is that of tracheal occlusion (TO), which is based on nature's interesting experiment with congenital laryngeal atresia, leading to excessive lung growth [13]. Experimental TO leads to increased levels of lung tissue stretch, triggers lung growth, and reverses pulmonary hypoplasia before birth as shown in morphological studies [14,15].…”

Section: Concept and Clinical Implementation Of Fetal Therapymentioning

confidence: 99%

Current consequences of prenatal diagnosis of congenital diaphragmatic hernia

Deprest

Jani

Schoubroeck

et al. 2006

Journal of Pediatric Surgery

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Section: Concept and Clinical Implementation Of Fetal Therapymentioning

confidence: 99%

Current consequences of prenatal diagnosis of congenital diaphragmatic hernia

Deprest

Jani

Schoubroeck

et al. 2006

Journal of Pediatric Surgery

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“…Conversely, experience with ‘open' fetal surgery was initially limited to procedures on placental circulation [31,32]. In view of the potential need for an expanded fetal surgery portfolio, we started by sending one of our staff members (J.D.)…”

Section: Methodsmentioning

confidence: 99%

Prenatal Diagnosis and Patient Preferences in Patients with Neural Tube Defects around the Advent of Fetal Surgery in Belgium and Holland

Ovaere

Eggink²,

Richter³

et al. 2014

Fetal Diagn Ther

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Introduction: We review the characteristics and prenatal choices of patients recently evaluated for neural tube defects (NTD) at two tertiary units. The prenatal diagnosis of NTD allows parents to consider all prenatal options. In selected cases of spina bifida aperta this also includes fetal surgery, which we started offering after combined ‘in-house' and ‘exported' training. Material and Methods: This is a retrospective review of prospectively collected data on NTD diagnosed over the last 8 years and recent fetal surgery referrals. Results: A total of 167 patients were referred for assessment at a median of 19 weeks. Cranial lesions were diagnosed significantly earlier than spinal lesions. Of the open spinal lesions, 77% were isolated. Of these, 22% were managed expectantly and 1 (1%) had fetal surgery. There was no correlation between parental decisions on prenatal management with disease-specific severity markers. We had 14 fetal surgery referrals, all but 1 from beyond our typical referral area; 6 of the assessed patients were operated on, 4 were expectantly managed and 4 requested termination of pregnancy (TOP). These pregnancy outcomes were in the expected range. Discussion: Open spina bifida is mainly diagnosed in the second trimester and 76% of subjects request TOP, irrespective of the severity indicators. The number of local patients considering fetal surgery is low.

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“…Until presently, tracheal agenesis has not been prenatally diagnosed, which has prevented the option for the EXIT strategy [4][5][6] . However, this case demonstrates that if the correct diagnosis can be made and the pregnant mother can be transferred to an institution with appropriate experience, EXIT offers the potential for salvage of these babies [7][8][9] . It is important to note that delivery via EXIT-to-airway strategy is the only initial hurdle in the management of infants with CHAOS, as the postnatal course can be extremely challenging and best undertaken by centers experienced in the care of these unique patients.…”

Section: Discussionmentioning

confidence: 99%

Congenital High Airway Obstruction Syndrome due to Complete Tracheal Agenesis: An Accident of Nature with Clues for Tracheal Development and Lessons in Management

Vaikunth¹,

Morris²,

Polzin

et al. 2009

Fetal Diagn Ther

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Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition characterized by complete blockage of the fetal airways associated with hydrops. We present a case of CHAOS due to the rare cause of complete tracheal agenesis. The ex utero intrapartum therapy (EXIT) strategy was employed to allow for neck and mediastinal exploration. Thymectomy allowed dissection to the level of the carina demonstrating the confluence of dilated mainstem bronchi but no trachea and no connection to the esophagus. A 2.5 endotracheal tube was inserted into the right mainstem bronchus and secured to the left clavicle. At 3 months of age, the infant succumbed to sepsis from Enterobacter mediastinitis due to friction between the tracheostomy tube and the nasogastric tube resulting in erosion of the esophagus. Complete tracheal agenesis, as seen in this case, is consistent with the failure of normal tracheal elongation as suggested by newer theories of foregut development. This case illustrates the most severe form of tracheal atresia causing CHAOS ever salvaged by the EXIT procedure at birth. The subsequent postnatal course highlights the need for early tracheal replacement in this particularly challenging form of CHAOS.

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Congenital high airway obstruction syndrome successfully managed with ex‐utero intrapartum treatment

Cited by 33 publications

References 6 publications

Current consequences of prenatal diagnosis of congenital diaphragmatic hernia

Current consequences of prenatal diagnosis of congenital diaphragmatic hernia

Prenatal Diagnosis and Patient Preferences in Patients with Neural Tube Defects around the Advent of Fetal Surgery in Belgium and Holland

Congenital High Airway Obstruction Syndrome due to Complete Tracheal Agenesis: An Accident of Nature with Clues for Tracheal Development and Lessons in Management

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