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Timothy M Crombleholme

Publications and source records attributed to Timothy M Crombleholme.

44 records · Page 3Linked to original sources

History of fetal diagnosis and therapy: Children's Hospital of Philadelphia experience.

Since its inception in 1995, the mission of the Center for Fetal Diagnosis and Treatment at the Children's Hospital of Philadelphia (CHOP) has been to provide comprehensive, multidisciplinary expertise in all facets of prenatal diagnosis, reproductive genetics, and prenatal, perinatal, and postnatal treatment for abnormal fetuses. Most of the authors were trained and/or served on faculty at centers in San Francisco (N.S.A., A.W.F., T.M.C., L.J.H.) or Detroit (A.W.F, M.P.J.). Accurate prenatal detection by serial sonographic study and the development of ultrafast magnetic resonance imaging (MRI) scanners has permitted delineation of the natural history of anatomic malformations. Definition of the pathophysiological features that affect clinical outcome and formulation of management based on prognosis has allowed families to make informed decisions. Outcomes have been improved through carefully planned and coordinated perinatal management. In some cases of life-threatening or severely debilitating defects, prenatal surgery has been offered.

Female↗

Chorioamniotic membrane separation following open fetal surgery: pregnancy outcome.

OBJECTIVE: To review the incidence of posthysterotomy chorioamniotic membrane separation and delivery outcome following open fetal surgery [myelomeningocele (MMC); cystic adenomatoid malformation (CCAM); congenital diaphragmatic hernia (CDH); sacrococcygeal teratoma (SCT)]. STUDY DESIGN: Retrospective review of a maternal population undergoing open fetal surgery at a single tertiary level program (1998-2001) following the initiation of close postoperative ultrasound follow-up for membrane separation. Onset of membrane separation was coded as not present (NP), immediate (<2 weeks) or delayed (>2 weeks) from day of surgery. RESULTS: Fifty-three charts were reviewed: MMC 43, CCAM 7, CDH 1, and SCT 2. In the MMC group there were 26 NP, 8 immediate, and 9 delayed. Preterm labor occurred in 4 patients with only 2 having had membrane separation. Risk of membrane separation is increased for surgery done at less than 23 weeks gestation (p < 0.005). Delay from MMC surgery to delivery was 11.0, 9.8, 12.0 weeks for NP, immediate, and delay, respectively. In the MMC group, there were 3 neonatal deaths (NND) at 9, 9, and 21 days post surgery (PROM/PTL; chorioamnionitis, PROM/PTL, respectively). No membrane separation was present in the CCAM, CDH, and SCT cases. CONCLUSIONS: (1) Membrane separation was significantly more likely to occur if surgery was performed prior to 23 weeks. (2) Membrane separation post hysterotomy (17/50 = 34%) may be associated with an increased risk of PROM but not delivery before 30 weeks gestation. (3) Delivery prior to 33 weeks gestation for MMC groups was 12/43 (28%) with 3 NND (7%). (4) Elective delivery at 36-37 weeks gestation was possible for 43% of the fetal surgery population.

Amnion↗

Abnormal umbilical cord Dopplers may predict impending demise in fetuses with sacrococcygeal teratoma. A report of 2 cases.

OBJECTIVE: To identify factors predictive of fetal demise in fetuses with sacrococcygeal teratoma (SCT). METHOD: The recent management of monochorionic twins discordant for a large SCT and a singleton with a large SCT were reviewed. RESULTS: Serial fetal echocardiography and ultrasonography with Doppler flow measurements documented rapid growth of the SCT in both cases with a relatively modest increase in combined cardiac output. No placentomegaly or hydrops was observed at any time. In both fetuses with SCT, evolution of abnormal umbilical artery waveforms was observed with the ultimate development of reversed end-diastolic umbilical arterial flow that was followed by sudden fetal demise. CONCLUSION: Death in these 2 fetuses with large SCTs in the absence of placentomegaly/hydrops or hemodynamic changes suggestive of evolving high-output failure suggests a previously unrecognized mechanism of death in fetuses with large rapidly growing SCTs. In these cases, fetal demise may only be heralded by abnormal umbilical artery waveforms that progress to the premorbid observation of reversed diastolic umbilical artery blood flow. Umbilical artery waveform analysis should be closely monitored with other hemodynamic parameters in fetuses with large SCTs. In such fetuses, depending on the gestational age, abnormalities in umbilical artery waveform should be considered indications for early delivery or in utero intervention to prevent fetal demise.

Adult↗

Abnormal umbilical cord Doppler sonograms may predict impending demise in fetuses with sacrococcygeal teratoma. A report of two cases.

OBJECTIVE: To identify factors predictive of fetal demise in fetuses with sacrococcygeal teratoma (SCT). METHOD: The recent management of monochorionic twins discordant for a large SCT and a singleton with a large SCT was reviewed. RESULTS: Serial fetal echocardiography and ultrasonography with Doppler flow measurements documented rapid growth of the SCT in both cases with a relatively modest increase in combined cardiac output. No placentomegaly or hydrops was observed at any time. In both fetuses with SCT, evolution of abnormal umbilical artery waveforms was observed with the ultimate development of reversed end-diastolic umbilical arterial flow that was followed by sudden fetal demise. CONCLUSION: Death in these 2 fetuses with large SCTs in the absence of placentomegaly/hydrops or hemodynamic changes suggestive of evolving high-output failure suggests a previously unrecognized mechanism of death in fetuses with large rapidly growing SCTs. In these cases, fetal demise may only be heralded by abnormal umbilical artery waveforms that progress to the premorbid observation of reversed diastolic umbilical artery blood flow. Umbilical artery waveform analysis should be closely monitored with other hemodynamic parameters in fetuses with large SCTs. In such fetuses, depending on the gestational age, abnormalities in umbilical artery waveform should be considered indications for early delivery or in utero intervention to prevent fetal demise.

Adult↗

Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations.

OBJECTIVE: To determine whether fetuses that underwent thoracoamniotic shunt placement for treatment of pleural effusion (PE) or macrocystic congenital cystic adenomatoid malformation (CCAM) have an improved outcome as compared with an untreated population. METHODS: A retrospective review from a single tertiary center was performed using thoracoamniotic shunt placement to treat PE or macrocystic CCAM between 1998 and 2001. Thoracoamniotic shunts were used on 26 occasions in 19 pregnancies. RESULTS: The average gestational age at the diagnosis of PE and CCAM was 22 + 4 and 20 + 0 weeks, respectively. Shunts were offered in pregnancies complicated by hydrops or at significant risk for pulmonary hypoplasia. Shunts were placed at 26 + 2 weeks (average) and 23 + 1 weeks (average) in the PE and CCAM groups, respectively. In CCAM patients, the mean pre- and postshunting mass volumes were 50.5 and 25.7 cm(3), representing a 51% reduction in mass volume following shunt placement. In the PE group, the average delivery age was 33 + 5 weeks, with an average shunt placement to delivery time of 7 + 3 weeks. In the CCAM group, the average delivery was 33 + 3 weeks, with an average shunt placement to delivery time of 10 + 2 weeks. The postnatal survival rates were 67% (6/9) and 70% (7/10) in the PE and CCAM groups, respectively. CONCLUSIONS: (1) Thoracoamniotic shunts should be considered as a treatment option for selected PE or macrocystic CCAM fetuses with hydrops or a significant risk for pulmonary hypoplasia; (2) the neonatal survival with shunting was similar for PE and CCAM groups and was improved as compared with literature reports, and (3) fetuses with CCAM presented earlier with hydrops than those with PE. Successful shunting resulted in a prolongation of pregnancy into the 3rd trimester in both groups.

Adult↗

Prenatal diagnosis and management of mainstem bronchial atresia.

The prenatal diagnosis, natural history and management of mainstem bronchial atresia have not been described previously. We report two cases of prenatally diagnosed proximal bronchial atresia. The first patient presented at 18 weeks with sonographic and MRI findings consistent with bronchial atresia with fetal hydrops. The mother developed the mirror syndrome and labor was induced. A non-viable fetus was delivered at 25 weeks. The second patient presented at 16 weeks gestation with evidence of an intrathoracic mass that was subsequently prenatally diagnosed as a right mainstem bronchial atresia. The right lung increased rapidly in size and was associated with the onset of fetal hydrops. At 24 weeks, fetal pneumonectomy was performed but the fetus expired intraoperatively due to cardiovascular collapse. Post-mortem findings in both cases confirmed the presence of an atretic mainstem bronchus with massive enlargement of the lung. Bronchial atresia involving the mainstem bronchus is associated with a poor prognosis.

Bronchi↗

Impact of continuous intraoperative monitoring on outcomes in open fetal surgery.

OBJECTIVES: There are shifts in fetal hemodynamics during open fetal surgery that were not appreciated until the use of intraoperative fetal echocardiography. We have developed an intraoperative monitoring strategy to continuously assess fetal hemodynamics. We hypothesized that this approach would enhance intraoperative management and survival. METHODS: Medical records of open fetal surgery patients were reviewed since the implementation of this approach. Intraoperative fetal monitoring was accomplished by continuous echocardiography, pulse oximetry, establishment of intravenous access, and arterial blood gas and hemoglobin measurement. Overall survival was compared to fetal surgeries performed prior implementation of this monitoring strategy. RESULTS: Resections of a congenital cystic adenomatoid malformation or a sacrococcygeal teratoma in nine hydropic fetuses were performed while using this monitoring strategy. Intraoperative echocardiography resulted in a change of management in 7 of 9 fetuses. The main observations on fetal echocardiography resulting in intraoperative intervention were decreased ventricular filling, bradycardia, and decreased ventricular contractility. Therapy included administration of volume expanders and/or inotropic agents. Overall fetal survival was 78% compared to a survival of 42% prior to the implementation of this approach. CONCLUSION: Continuous intraoperative fetal monitoring provides real time assessment of fetal hemodynamics which results in changes in intraoperative management. The overall outcomes in these critically ill fetuses have been improved.

Echocardiography↗

Prenatal MR findings of the middle interhemispheric variant of holoprosencephaly.

We report a case of the middle interhemispheric variant of holoprosencephaly (MIH) with noncleavage of the posterior portion of the frontal lobes and the parietal regions in a fetus at 22 weeks' gestation. To our knowledge, this is the first case of the rare MIH variant to be diagnosed in utero by use of ultrafast MR imaging and one of the few such reports to document gross and microscopic pathologic findings. Neuroimaging results correlated with those of gross and microscopic pathologic specimens obtained from the stillborn child. We conclude that ultrafast MR imaging can accurately distinguish holoprosencephaly subtypes in utero, which may affect counseling of parents.

Adult↗