Pulmonary Arteriovenous MalformationCPAMNCT04449614
A Review of Surgical Management of Congenital Pulmonary Airway Malformations (CPAM): A Decade of Experience
An observational study of Pulmonary Arteriovenous Malformation and Congenital Disorders, run by King's College Hospital NHS Trust at 1 site in United Kingdom.
Trial summary
Congenital pulmonary malformation in children is a rare abnormality mostly diagnosed before birth during antenatal ultrasound examinations. These lesions may expand to form lung cysts in children, cause recurrent lung infections and has a potential for malignant change. Read more about this study
- Who is it for?
- Ages Up to 16 years
- Conditions the study is for
- Pulmonary Arteriovenous Malformation, Congenital Disorders
- What is being tested?
- Thoracoscopic CPAM resection
- Type of treatment
- Surgery or procedure
- How many people
- 72 participants
- Where
- 1 siteUnited Kingdom
- Study dates
- 10 Jan 2018to 28 Feb 2020
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages Up to 16 years
- All sexes
- No healthy volunteers
- Child
Study population: All infants and children who had undergone thoracoscopic surgery for a CPAM from 2008 to 2017 at a regional centre
Who may be able to join
- All children undergoing surgery for a CPAM removal between 2008 - 2017 at a regional hospital.
Who cannot join
- Lack of informed consent. Inability to contact parents/guardian for the required post-operative interview
Trial locations and contacts
1 site in 1 country.
About this study
Congenital pulmonary malformation in children is a rare abnormality mostly diagnosed before birth during antenatal ultrasound examinations. These lesions may expand to form lung cysts in children, cause recurrent lung infections and has a potential for malignant change. Therefore, surgical removal in childhood is favoured as the treatment of choice.
The surgical correction may involve 'open' surgery or 'key hole' surgery. There is, however, a variation in surgical and anaesthetic techniques and timing of this surgery and subsequent complications reported post-surgery.
The purpose of this investigation is to review anaesthetic and surgical case notes and the subsequent well-being of all children who underwent lung surgery to remove above lung lesions over the last 10 years (2008-2017) at a regional centre. The aim is to look at the current status of these children in relation to their health, growth and development evaluated via a 20-minute structured telephone interview with prior consent.
Read the detailed description
Congenital pulmonary airway malformation (CPAM) (previously named congenital cystic adenomatoid malformation) is a rare abnormality, first described in 1949. CPAM results from adenomatoid proliferation of the terminal bronchioles causing cyst formation, which may impair normal alveolar growth. It occurs more commonly in males and has an estimated incidence of 1:11000 to 1:35000. Most are diagnosed on routine antenatal ultrasonography. Most involve lower lobes, occasionally lesions are bilateral. CPAM lesions are usually managed by surgical removal in childhood due to the risk of serious infection, and potential for malignant change. A recent meta-analysis has favoured surgical management.
Historically, correction of CPAM was by open thoracotomy and surgical resection. This has evolved, however, and since 2008 more than 50 surgical corrections have been carried out thoracoscopically at King's College Hospital. The perioperative anaesthetic management of these children has also evolved, with the increasing use of selective lung ventilation.
The age range at surgery is variable, usually 1 - 14 years, and just over half being asymptomatic. Anaesthetic techniques are variable: for example, the deployment of selective lung ventilation techniques, the choice of anaesthetic agents and the types of invasive monitoring. Surgical duration is typically 3-4 hours. A degree of hypercarbia occurs in most cases during the procedure because of reduced minute ventilation to facilitate surgical access, and the absorption of carbon dioxide insufflated into the pleural space. Around 1/3rd receive blood transfusions. Perioperative complications include: respiratory problems causing hypoxia, requirement for re-intubation, conversion to open thoracotomy, bradycardia, surgical emphysema and sometimes the requirement for prolonged post-operative respiratory support including ventilation, CPAP and chest drain management, bronchopulmonary fistulae, and chylothorax and rarely seizures and cerebral infarction.
In general, post-operative care includes at least 24 hours critical care and another 6-7 days in hospital. The mainstay of early post-operative pain control is either with morphine using a nurse controlled intravenous analgesia system, or with epidural analgesia. These are supplemented with oral analgesic regimens. Children who were preoperatively symptomatic seem more likely to develop perioperative complications.
Objective The purpose of this investigation is to review all children who underwent CPAM surgery over the last 10 years at a single regional centre and carry out a descriptive evaluation of pre-operative factors, anaesthetic and surgical factors, peri- and post-operative morbidity, mortality and subsequent long term outcome.
Methods A retrospective review of all CPAM resections performed since 2008 at a single centre. The data will be retrieved from electronic and paper based medical records, anaesthetics records and operation notes. A prospective analysis of the post-operative course and events will be undertaken. A detailed evaluation of these children in relation to their health, growth and development will be carried out via a structured 20-minute telephone interview with prior consent.
Analysis Outcomes will be compared across preoperatively symptomatic and asymptomatic groups using Mann-Whitney/Wilcoxon test or Student t test for continuous data or the chi square for categorical data. All tests were 2-tailed, and P< 0.05 was considered statistically significant.
- Being tested
- Thoracoscopic CPAM resection
- Medical subject headings
- Pulmonary Arteriovenous Fistulas, Congenital, Hereditary, and Neonatal Diseases and Abnormalities
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Thoracoscopic CPAM resectionProcedure or surgery
Surgical removal of lung lesion
| Study group | Role | What they receive |
|---|---|---|
| 1 | Inclusion criteria: All consenting Infants and children who have had A Congenital Pulmonary Airway Malformation (CPAM) surgically removed by thoracoscopy over a 10 year period (2008-2017) in a regional centre.
Exclusion criteria: Non consenting participants Receives: Procedure: Thoracoscopic CPAM resection |
What is being measured
The primary outcome is the main question the study is designed to answer.
Perioperative morbidity associated with the CPAM removal surgical episode
This will be a descriptive measure of perioperative complications, namely, number including converted to 'open' surgery from thoracoscopy and its reasons, postoperative respiratory complications needing active therapy such as return to theatre, reintubation, insertion of chest drains to manage pneumothorax, haemothorax or chylothorax, duration of intensive care needed and duration of hospital stay needed.
Measured: 28 days postoperative
Perioperative mortality associated the CPAM removal surgical episode
This will include 28 day mortality associated with the surgical episode
Measured: 28 days postoperative
Secondary outcomes (2)
Post surgery long-term well being as reported by parents/guardian
This will be a qualitative assessment of the child's postoperative well-being as reported by the parents/guardian during hospital follow up clinics or an assessment by telephone using a standard questionnaire to assess well-being, growth and development of the child as reported by the parents or guardian. This will in addition include the frequency of any respiratory symptoms, therapeutic needs and hospitalisations encountered by the child following the removal of CPAM.
Measured: A prospective 1 year to complete a telephone follow-up of all participant children who had their surgery performed between 2007-2017
Post surgery long-term development and growth of children as reported by parent/guardian
This will be parental opinion of child's well-being when compared to that of preoperative cognitive function, development, and general health. In summary this is a qualitative assessment of child's health as reported by the parents.
Measured: A prospective 1 year to complete a telephone follow-up of all participant children who had their surgery performed between 2007-2017
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted29 Jun 2020
- Study start10 Jan 2018
- Primary completion30 Sep 2019
- Study completion28 Feb 2020
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (10)Papers the sponsor has linked to this study.
- Other trials for Pulmonary Arteriovenous MalformationStudies of the same condition, open and under way.
- More trials run by King's College Hospital NHS TrustThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Retrospective
- Enrolment
- 72 participants (actual)
Sponsor and investigators
- Lead sponsor
- King's College Hospital NHS Trust
- Sponsor type
- University, hospital or other
- Collaborators
- King's College London
- Responsible party
- Sponsor
- Investigators
- Chulananda Goonasekera, Principal investigator, Consultant Anaesthetist
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04449614
- Sponsor's protocol ID
- IRAS 238420
- Acronym
- CPAM
- Status last verified
- Apr 2018
- First submitted
- 10 Oct 2018
- Last updated
- 29 Jun 2020
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- Undecided
Publications
Subramanyam R, Ledbetter K, Fleck R, Mahmoud M. Images in Anesthesiology: Congenital Pulmonary Airway Malformation. Anesthesiology. 2017 Aug;127(2):382. doi: 10.1097/ALN.0000000000001602. No abstract available.
PubMed 28719530Cloutier MM, Schaeffer DA, Hight D. Congenital cystic adenomatoid malformation. Chest. 1993 Mar;103(3):761-4. doi: 10.1378/chest.103.3.761.
PubMed 8449065Sfakianaki AK, Copel JA. Congenital cystic lesions of the lung: congenital cystic adenomatoid malformation and bronchopulmonary sequestration. Rev Obstet Gynecol. 2012;5(2):85-93.
PubMed 22866187Mann S, Wilson RD, Bebbington MW, Adzick NS, Johnson MP. Antenatal diagnosis and management of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med. 2007 Dec;12(6):477-81. doi: 10.1016/j.siny.2007.06.009. Epub 2007 Oct 22.
PubMed 17950681David M, Lamas-Pinheiro R, Henriques-Coelho T. Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation. Neonatology. 2016;110(2):101-15. doi: 10.1159/000440894. Epub 2016 Apr 13.
PubMed 27070354Sueyoshi R, Koga H, Suzuki K, Miyano G, Okawada M, Doi T, Lane GJ, Yamataka A. Surgical intervention for congenital pulmonary airway malformation (CPAM) patients with preoperative pneumonia and abscess formation: "open versus thoracoscopic lobectomy". Pediatr Surg Int. 2016 Apr;32(4):347-51. doi: 10.1007/s00383-015-3848-z. Epub 2015 Dec 12.
PubMed 26661941CH'IN KY, TANG MY. Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol (Chic). 1949 Sep;48(3):221-9. No abstract available.
PubMed 18137795Laberge JM, Flageole H, Pugash D, Khalife S, Blair G, Filiatrault D, Russo P, Lees G, Wilson RD. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther. 2001 May-Jun;16(3):178-86. doi: 10.1159/000053905.
PubMed 11316935Kapralik J, Wayne C, Chan E, Nasr A. Surgical versus conservative management of congenital pulmonary airway malformation in children: A systematic review and meta-analysis. J Pediatr Surg. 2016 Mar;51(3):508-12. doi: 10.1016/j.jpedsurg.2015.11.022. Epub 2015 Dec 9.
PubMed 26775193Khan H, Kurup M, Saikia S, Desai A, Mathew M, Sheikh A, Goonasekera CDA. Morbidity after thoracoscopic resection of congenital pulmonary airway malformations (CPAM): single center experience over a decade. Pediatr Surg Int. 2021 May;37(5):549-554. doi: 10.1007/s00383-020-04801-1. Epub 2021 Jan 3.
PubMed 33388955
Study at a glance
From the registry record, last updated by the sponsor on 29 Jun 2020. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- A Review of Surgical Management of Congenital Pulmonary Airway Malformations (CPAM): A Decade of Experience
- ClinicalTrials.gov ID
- NCT04449614
- Acronym / sponsor ID
- CPAM / IRAS 238420
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 72 participants (actual)
- Lead sponsor
- King's College Hospital NHS TrustUniversity, hospital or other
- Collaborators
- King's College London
- Study start
- 10 Jan 2018 (actual)
- Primary completion
- 30 Sep 2019 (actual)
- Study completion
- 28 Feb 2020 (actual)
- First posted
- 29 Jun 2020 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Congenital pulmonary malformation in children is a rare abnormality mostly diagnosed before birth during antenatal ultrasound examinations. These lesions may expand to form lung cysts in children, cause recurrent lung infections and has a potential for malignant change. Therefore, surgical removal in childhood is favoured as the treatment of choice.
The surgical correction may involve 'open' surgery or 'key hole' surgery. There is, however, a variation in surgical and anaesthetic techniques and timing of this surgery and subsequent complications reported post-surgery.
The purpose of this investigation is to review anaesthetic and surgical case notes and the subsequent well-being of all children who underwent lung surgery to remove above lung lesions over the last 10 years (2008-2017) at a regional centre. The aim is to look at the current status of these children in relation to their health, growth and development evaluated via a 20-minute structured telephone interview with prior consent.
Detailed description
Congenital pulmonary airway malformation (CPAM) (previously named congenital cystic adenomatoid malformation) is a rare abnormality, first described in 1949. CPAM results from adenomatoid proliferation of the terminal bronchioles causing cyst formation, which may impair normal alveolar growth. It occurs more commonly in males and has an estimated incidence of 1:11000 to 1:35000. Most are diagnosed on routine antenatal ultrasonography. Most involve lower lobes, occasionally lesions are bilateral. CPAM lesions are usually managed by surgical removal in childhood due to the risk of serious infection, and potential for malignant change. A recent meta-analysis has favoured surgical management.
Historically, correction of CPAM was by open thoracotomy and surgical resection. This has evolved, however, and since 2008 more than 50 surgical corrections have been carried out thoracoscopically at King's College Hospital. The perioperative anaesthetic management of these children has also evolved, with the increasing use of selective lung ventilation.
The age range at surgery is variable, usually 1 - 14 years, and just over half being asymptomatic. Anaesthetic techniques are variable: for example, the deployment of selective lung ventilation techniques, the choice of anaesthetic agents and the types of invasive monitoring. Surgical duration is typically 3-4 hours. A degree of hypercarbia occurs in most cases during the procedure because of reduced minute ventilation to facilitate surgical access, and the absorption of carbon dioxide insufflated into the pleural space. Around 1/3rd receive blood transfusions. Perioperative complications include: respiratory problems causing hypoxia, requirement for re-intubation, conversion to open thoracotomy, bradycardia, surgical emphysema and sometimes the requirement for prolonged post-operative respiratory support including ventilation, CPAP and chest drain management, bronchopulmonary fistulae, and chylothorax and rarely seizures and cerebral infarction.
In general, post-operative care includes at least 24 hours critical care and another 6-7 days in hospital. The mainstay of early post-operative pain control is either with morphine using a nurse controlled intravenous analgesia system, or with epidural analgesia. These are supplemented with oral analgesic regimens. Children who were preoperatively symptomatic seem more likely to develop perioperative complications.
Objective The purpose of this investigation is to review all children who underwent CPAM surgery over the last 10 years at a single regional centre and carry out a descriptive evaluation of pre-operative factors, anaesthetic and surgical factors, peri- and post-operative morbidity, mortality and subsequent long term outcome.
Methods A retrospective review of all CPAM resections performed since 2008 at a single centre. The data will be retrieved from electronic and paper based medical records, anaesthetics records and operation notes. A prospective analysis of the post-operative course and events will be undertaken. A detailed evaluation of these children in relation to their health, growth and development will be carried out via a structured 20-minute telephone interview with prior consent.
Analysis Outcomes will be compared across preoperatively symptomatic and asymptomatic groups using Mann-Whitney/Wilcoxon test or Student t test for continuous data or the chi square for categorical data. All tests were 2-tailed, and P< 0.05 was considered statistically significant.
Study design
- Observational model
- Cohort
- Time perspective
- Retrospective
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| 1 | – | Procedure: Thoracoscopic CPAM resectionInclusion criteria: All consenting Infants and children who have had A Congenital Pulmonary Airway Malformation (CPAM) surgically removed by thoracoscopy over a 10 year period (2008-2017) in a regional centre. Exclusion criteria: Non consenting participants |
Who may qualify
- Ages
- Up to 16 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child
- Study population
- All infants and children who had undergone thoracoscopic surgery for a CPAM from 2008 to 2017 at a regional centre
Key inclusion criteria (1 listed)
- All children undergoing surgery for a CPAM removal between 2008 - 2017 at a regional hospital.
Key exclusion criteria (1 listed)
- Lack of informed consent. Inability to contact parents/guardian for the required post-operative interview
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Thoracoscopic CPAM resectionSurgical removal of lung lesion | Procedure or surgery | 1 |
Primary outcome
Perioperative morbidity associated with the CPAM removal surgical episode
Time frame: 28 days postoperative
This will be a descriptive measure of perioperative complications, namely, number including converted to 'open' surgery from thoracoscopy and its reasons, postoperative respiratory complications needing active therapy such as return to theatre, reintubation, insertion of chest drains to manage pneumothorax, haemothorax or chylothorax, duration of intensive care needed and duration of hospital stay needed.
Perioperative mortality associated the CPAM removal surgical episode
Time frame: 28 days postoperative
This will include 28 day mortality associated with the surgical episode
2 secondary outcomes
Post surgery long-term well being as reported by parents/guardian
Time frame: A prospective 1 year to complete a telephone follow-up of all participant children who had their surgery performed between 2007-2017
Post surgery long-term development and growth of children as reported by parent/guardian
Time frame: A prospective 1 year to complete a telephone follow-up of all participant children who had their surgery performed between 2007-2017
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 29 Jun 2020.
- Background: PubMed 28719530Subramanyam R, Ledbetter K, Fleck R, Mahmoud M. Images in Anesthesiology: Congenital Pulmonary Airway Malformation. Anesthesiology. 2017 Aug;127(2):382. doi: 10.1097/ALN.0000000000001602. No abstract available.
- Background: PubMed 8449065Cloutier MM, Schaeffer DA, Hight D. Congenital cystic adenomatoid malformation. Chest. 1993 Mar;103(3):761-4. doi: 10.1378/chest.103.3.761.
- Background: PubMed 22866187Sfakianaki AK, Copel JA. Congenital cystic lesions of the lung: congenital cystic adenomatoid malformation and bronchopulmonary sequestration. Rev Obstet Gynecol. 2012;5(2):85-93.
- Background: PubMed 17950681Mann S, Wilson RD, Bebbington MW, Adzick NS, Johnson MP. Antenatal diagnosis and management of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med. 2007 Dec;12(6):477-81. doi: 10.1016/j.siny.2007.06.009. Epub 2007 Oct 22.
- Background: PubMed 27070354David M, Lamas-Pinheiro R, Henriques-Coelho T. Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation. Neonatology. 2016;110(2):101-15. doi: 10.1159/000440894. Epub 2016 Apr 13.
- Results publication: PubMed 26661941Sueyoshi R, Koga H, Suzuki K, Miyano G, Okawada M, Doi T, Lane GJ, Yamataka A. Surgical intervention for congenital pulmonary airway malformation (CPAM) patients with preoperative pneumonia and abscess formation: "open versus thoracoscopic lobectomy". Pediatr Surg Int. 2016 Apr;32(4):347-51. doi: 10.1007/s00383-015-3848-z. Epub 2015 Dec 12.
- Results publication: PubMed 18137795CH'IN KY, TANG MY. Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol (Chic). 1949 Sep;48(3):221-9. No abstract available.
- Results publication: PubMed 11316935Laberge JM, Flageole H, Pugash D, Khalife S, Blair G, Filiatrault D, Russo P, Lees G, Wilson RD. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther. 2001 May-Jun;16(3):178-86. doi: 10.1159/000053905.
- Results publication: PubMed 26775193Kapralik J, Wayne C, Chan E, Nasr A. Surgical versus conservative management of congenital pulmonary airway malformation in children: A systematic review and meta-analysis. J Pediatr Surg. 2016 Mar;51(3):508-12. doi: 10.1016/j.jpedsurg.2015.11.022. Epub 2015 Dec 9.
- Derived from this study: PubMed 33388955Khan H, Kurup M, Saikia S, Desai A, Mathew M, Sheikh A, Goonasekera CDA. Morbidity after thoracoscopic resection of congenital pulmonary airway malformations (CPAM): single center experience over a decade. Pediatr Surg Int. 2021 May;37(5):549-554. doi: 10.1007/s00383-020-04801-1. Epub 2021 Jan 3.
- Individual participant dataSharing planned: Undecided
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Chulananda Goonasekera principal investigator
Consultant Anaesthetist
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Pulmonary Arteriovenous Malformation and Congenital Disorders, run by King's College Hospital NHS Trust at 1 site in United Kingdom.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 1 inclusion and 1 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 29 Jun 2020. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United Kingdom. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Perioperative morbidity associated with the CPAM removal surgical episode (time frame: 28 days postoperative). The study lists 2 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT04449614, which also lists 10 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 29 Jun 2020.