Congenital cystic adenomatoid malformation of the lung (CCAM) and Bronchopulmonary sequestration (BPS) [Elektronische Ressource] : prenatal diagnosis, pre- and postnatal interventions, and early- and long-term outcome (14 years clinical experience with 60 patients) / vorgelegt von Eva Maria Pott Bärtsch

Eva Maria Pott Baertsch

Sujets

• Gesundheit

Informations

• Publié par: ludwig-maximilians-universitat_munchen
• Publié le: 01 janvier 2009
• Nombre de lectures: 24
• Langue: English
• Poids de l'ouvrage: 1 Mo

Extrait

From the Fetal Treatment Center
of the University of California, San Francisco Medical Center, USA
Director: Prof. Dr. med. Michael R. Harrison
&
Aus der Klinik und Poliklinik für Frauenheilkunde und Geburtshilfe - Großhadern
der Ludwig-Maximilians-Universität München, Deutschland
Direktor: Prof. Dr. med. Klaus Friese

Congenital cystic adenomatoid malformation of the lung (CCAM) and
Bronchopulmonary sequestration (BPS):
Prenatal diagnosis, pre- and postnatal interventions, and early- and long-term outcome
(14 years clinical experience with 60 patients)

Dissertation
zum Erwerb des Doktorgrades in der Medizin
an der Medizinischen Fakultät der
Ludwig-Maximilians-Universität zu München

vorgelegt von
Eva Maria Pott Bärtsch
aus
Köln
2009













Mit Genehmigung der Medizinischen Fakultät
der Universität München


Berichterstatter: Prof. Dr. Alexander Strauss
Mitberichterstatter: Priv. Doz. Dr. Joseph Rosenecker
Prof. Dr. Orsolya Genzel-Boroviczény
Dekan: Prof. Dr. med. Dr. h.c. M. Reiser, FACR, FRCR

Tag der mündlichen Prüfung: 30.07.2009
























„Über sieben Brücken musst du geh'n …“, Song


























Kongenitale zystisch-adenomatoide Malformation der Lunge (CCAM) und
Bronchopulmonale Sequestration (BPS):
Pränatale Diagnose, prä- und postnatale Interventionen, sowie Früh- und
Langzeitverlauf
(14 Jahre klinische Erfahrung mit 60 Patienten)




Congenital cystic adenomatoid malformation of the lung (CCAM) and
Bronchopulmonary sequestration (BPS):
Prenatal diagnosis, pre- and postnatal interventions, and early- and long-term outcome
(14 years clinical experience with 60 patients)


Klinik und Poliklinik für Frauenheilkunde und Geburtshilfe - Großhadern
Klinikum der Ludwig-Maximilians-Universität München, Deutschland
Prof. Klaus Friese, Prof. Alexander Strauss


&


Fetal Treatment Center,
Division of Pediatric Surgery, Department of Surgery, and
Division of Ultrasound, Department of Radiology,
University of California, San Francisco Medical Center, USA
Prof. Michael R. Harrison, Prof. Diana L. Farmer, Prof. Ruth B. Goldstein


























Table of contents 1
Table of contents
PART I .............................................................................................................. 5
1. Zusammenfassung Teil I ............................................................................. 7
2. Summary Part I.......................................................................................... 18
3. Introduction................................................................................................ 27
4. Patients and Methods ................................................................................ 29
4.1 The patients .............................................................................................................. 29
4.1.1 Fetal patient population at the UCSF Medical Center, USA, and at the University
Hospital Großhadern of the LMU Munich, Germany ..............................................................29
4.1.2 Recruitment of the fetal study patients ............................................................................30
4.2 The materials and methods..................................................................................... 30
4.2.1 Long-term follow-up questionnaire .................................................................................30
4.2.2 Medical records review....................................................................................................31
4.2.3 Prenatal ultrasonographic data.........................................................................................31
4.2.4 Perinatal and pediatric outcome data ...............................................................................35
4.2.5 Statistical analysis............................................................................................................37
5. Results ......................................................................................................... 38
5.1 The patients’ response and data source................................................................. 38
5.2 The children’s outcomes.......................................................................................... 40
5.2.1 The survival rates of the children in the fetal non-intervention groups and in the fetal
treatment group were excellent.................................................................................................40
5.2.2 The early respiratory outcome correlated with the children’s age at surgical
intervention...............................................................................................................................43
5.2.3 Prematurity was a significant factor of the early outcome, and it predominated the
(potential) fetal intervention group...........................................................................................44
5.2.4 The final respiratory outcome was excellent, and it was not determined by the early
respiratory outcome ..................................................................................................................46
5.2.5 Interim respiratory outcomes correlated with early respiratory outcomes during the
period before significant improvement.....................................................................................47
5.2.6 Re-hospitalizations in childhood were required for 55% of the children after invasive
fetal treatment and for 54% of the non-operated children for delayed surgery ........................48
5.2.7 Other non-respiratory complications were postoperative complications, prematurity-
associated feeding problems, transient cardiac symptoms, and rare congenital anomalies......49
5.3 The prognostic value of the prenatal ultrasound parameters ............................. 54
5.3.1 Not the distinct prenatal diagnosis, but the type of the cystic lung lesion was a
potential predictive factor of the early outcome .......................................................................54
5.3.2 The final size of the mass was the most important predictor of the early outcome.........57
5.3.3 A hydrops fetalis and some of the hydrops-associated symptoms were associated with
the size of the mass and they were very strong predictors of the early outcome......................63
5.3.4 The degree of the mediastinal shift correlated with the size of the mass and it was also
a strong predictor of the early outcome ....................................................................................67
Table of contents 2
6. Discussion.................................................................................................... 69
6.1 Prediction of the outcomes ...................................................................................... 69
6.1.1 The set of prenatal early outcome predictors that this 60-patients-study disclosed,
strikingly coincides with the predictor set generated by an extended statistical analysis of
published data ...........................................................................................................................69
6.1.2 The size of the mass is the crucial pivotal point of the early outcome and it requires
serial observation due to its non-static character ......................................................................77
6.1.3 First signs of a hydrops in fetuses with large lesions mark the beginning of serious
complications starting before birth, but they can be averted with fetal treatment ....................85
6.1.4 Marked ascites and the type of the lesion are, in contrast to polyhydramnios,
independent outcome predictors ...............................................................................................93
6.1.5 A major reason for the overestimation of the mortality rate is that the “hidden survival
rate” is unaccounted for ............................................................................................................95
6.1.6 The long term outcome is excellent.................................................................................97
6.2 Management of the patients.................................................................................. 101
6.2.1 Prenatal management recommendations........................................................................101
6.2.2 Postnatal management recommendations......................................................................104
PART II..................................................................