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Biomedical subjects

D L Farmer

Publications and source records attributed to D L Farmer.

At least 19 recordsLinked to original sources
PubMed2002

Gastroschisis: small hole, big cost.

BACKGROUND/PURPOSE: This study was designed to assess the outcome and financial costs incurred for the treatment of gastroschisis. METHODS: A retrospective analysis was conducted of all patients with gastroschisis at a single institution over the past decade (n = 69). Hospital costs were determined and standardized to December 2001 dollars. RESULTS: Of the 69 patients, average gestational age at delivery was 35.9 weeks. Thirty-six patients had a primary fascial closure; 33 had a silo placed. The mean time to first feeding was 22 days and full feeding, 33 days. Average length of stay was 47 days. There were 3 deaths (2 shortly after birth, and one 131 days later owing to sepsis). The average cost of hospitalization and physician fees for patients with gastroschisis was $123,200. Using multivariate regression analysis, significant variables (P <.05) associated with cost of hospitalization were number of operative procedures, ventilatory days, male gender, and length of stay. Room expenses (43%), physician fees (15%), respiratory and pulmonary care (10%), and supply and devices (10%) made up the majority of costs. CONCLUSIONS: Cost of care associated with treatment for gastroschisis is high. Strategies designed to reduce cost must limit gastrointestinal, respiratory, and operative complications and reduce length of stay.

California↗
PubMed2002

Congenital diaphragmatic hernia and hydrops: a lethal association?

BACKGROUND/PURPOSE: Nonimmune hydrops in the fetus is a finding that often portends death. The association and prognosis of fetuses with congenital diaphragmatic hernia (CDH) and hydrops is not known. METHODS: A retrospective review of all prenatally diagnosed cases and referrals of CDH was performed. Variables analyzed included gestational age at diagnosis and delivery, side of hernia, presence of associated anomalies and hydrops, and neonatal outcome. RESULTS: Since 1993, 474 prenatal referrals for CDH have been made. One hundred seventy-five were evaluated; 15 fetuses had hydrops (9%). Five patients had CDH, hydrops, and associated lethal anomalies. In the remaining 10 patients, 6 of the diaphragmatic defects were right-sided and 4 were left-sided. All except one had a major portion of the liver herniated into the chest. Six fetuses had prenatal intervention. Five neonates died shortly after birth. There were 5 long-term survivors; all received prenatal intervention. CONCLUSIONS: The association of CDH and hydrops is rare but often results in fatality. Hydrops appears to be associated with liver in the hernia, right-sided lesions, and lethal anomalies. Fetal intervention can be performed successfully in patients with CDH and hydrops, and may improve long-term survival rate in this group.

Abnormalities, Multiple↗
PubMed2002

Fetoscopic treatment for discordant twins.

BACKGROUND/PURPOSE: In rare instances in monochorionic twin pregnancies, one twin can have a discordant anomaly (eg, cystic hygroma). If this twin dies in utero, neurologic injury and death can occur in the surviving cotwin. To protect the normal twin, the authors developed an approach to separate the circulations and ablate the umbilical cord of the abnormal twin. METHODS: From September 1998 to February 2001, 6 cases of discordant anomalous twins were diagnosed by prenatal ultrasound scan in which the anomaly was lethal or parents desired prenatal termination for this abnormal twin. All underwent surgical intervention with gestational ages varying from 19 to 24 weeks. RESULTS: Depending on cord insertion site and placental anatomy, blood flow was interrupted to the anomalous fetus by either radiofrequency ablation (RFA; 2 cases), cord transection (1 case), or cord transection after laser ablation of communicating vessels (3 cases). Fetal death occurred in one normal twin 4 days postoperatively. Average age at delivery for the 5 surviving fetuses was 34.5 weeks' gestation. On follow-up, all surviving infants are neurologically intact. CONCLUSION: An otherwise normal monochorionic twin threatened by an anomalous cotwin can be salvaged successfully with a strategy tailored to interrupt the vascular connections between the 2 twins.

Abnormalities, Multiple↗
PubMed2001

Fetal intervention for myelomeningocele: effect on postnatal bladder function.

PURPOSE: Myelomeningocele is the most common congenital malformation of the central nervous system noted on prenatal ultrasound. Due to its significant postnatal sequelae, treatment in utero could potentially have a profound impact on the newborn. Others have reported fetal surgical techniques for in utero repair of myelomeningocele and its potential benefits on motor and neurological function. We report our urodynamic findings in the newborn after in utero repair of spina bifida in an effort to characterize postnatal bladder function. MATERIALS AND METHODS: A retrospective review of the fetal surgery database at University of California San Francisco was performed identifying patients with a diagnosis of myelomeningocele. Prenatal surgical repair of myelomeningocele was considered if a normal karyotype was present, no other significant congenital anomalies were evident and gestational age was less than 24 weeks. The spinal defects were in the lumbar or lumbosacral region. All surgery was performed before 24 weeks of gestations. RESULTS: Fetal surgery to correct myelomeningocele was performed in 6 patients. All patients were born premature at 32 weeks of gestation or less. Videourodynamics performed at age 1 month in 4 patients indicated decreased bladder capacity for weight, increased detrusor storage pressures and significant post-void residual. Hydronephrosis was demonstrated in 4 patients on renal/bladder ultrasound, and moderate vesicoureteral reflux was seen in 3. CONCLUSIONS: Patients with spinal bifida treated in utero appear to have the same changes in urodynamic parameters and anatomical abnormalities in the urinary tract as other children with spinal defects who have undergone standard postnatal care. In utero treatment of spinal bifida may expose the newborn to the effects of prematurity. The long-term effects on bladder function in the fetus after in utero repair of myelomeningocele remain unknown. A randomized controlled trial is necessary to evaluate long-term bladder function as well as other outcome variables in this experimental approach to patients with myelomeningocele.

Female↗
PubMed2001

The surgical airway.

Traumatic airway injuries are rare in children, partly due to their unique anatomy. The larynx is well protected from direct blows behind the mandibular arch, and only a small portion of the trachea is unprotected above the manubrium due to the relatively short neck. Furthermore, the tracheobronchial tree is less prone to injuries as compared with adults due to its elasticity. A high index of suspicion is thus needed to adequately diagnose and manage pediatric airway injuries. Laryngotracheal injuries in particular may present with discreet initial symptoms that if undiagnosed may rapidly progress to loss of airway. The most important signs of laryngeal injury include hoarseness and subcutaneous emphysema. Tracheobronchial injuries often present with dramatic symptoms, the most common being pneumothorax, which does not resolve after placement of chest tube, or large persistent air leaks. Endoscopy is mandatory on suspicion of injury to the larynx, trachea, or bronchi. CT scan may be helpful in determining the extent of injury to the larynx. Correct management of the airway in laryngotracheal injuries has a direct impact on morbidity and mortality. Endotracheal intubation over a flexible bronchoscope during spontaneous ventilation and in halothane anesthesia is the method of choice in children, but it should be performed in the operating room with the possibility of emergency tracheotomy. Cricothyroidotomy should be avoided in all laryngotracheal injuries because this method may aggravate the injury. Most laryngotracheal injuries in children can be conservatively managed. Extensive injuries, including displaced fractures of the cartilage, injuries to the recurrent nerves, and laryngotracheal separation, require surgical intervention. Injuries to bronchi and the thoracic trachea that do not cause a persistent air leak, and where the lungs expand completely after insertion of chest tubes, may be managed conservatively. All other injuries to the tracheobronchial tree should be repaired surgically as soon as feasible. Induction of anesthesia and opening of the chest may make ventilation difficult and are best managed by selective intubation of the contralateral lung. Long-term outcome after laryngeal, tracheal, and bronchial injuries in children, if managed swiftly and accurately, is usually excellent unless other injuries are present. The final result is improved by early recognition and early surgical intervention. These children need to be followed endoscopically for months and sometimes years in order to diagnose and treat stenoses as soon as they occur. Long-term pulmonary function has been shown to be excellent. Children with bilateral recurrent nerve paralysis may not fully recover voice or airway.

Airway Obstruction↗
PubMed2001

Outcome of prenatally diagnosed solid fetal tumors.

BACKGROUND/PURPOSE: In the last 10 years, the ability to diagnose fetal tumors in the prenatal period has improved greatly because of technical advances in imaging. Early diagnosis and determination of tumor may affect prognosis. METHODS: The authors retrospectively reviewed the records of 1316 fetuses who underwent sonographic evaluation for congenital defects at University of California-San Francisco over a 6-year period. Of these, 16 had fetal tumors and were followed up at our institution. There were solid or predominantely solid with small cystic component masses in one of 3 locations: cervical, mediastinal, or abdominal. Excluded from our study were those fetuses with either sacrococcygeal teratoma, congenital cystic adenomatoid malformation of the lung, or ovarian cyst, because these defects have been extensively reviewed elsewhere. In addition, masses that were primarily cystic also were excluded. Data collected included diagnosis, gestational age at diagnosis and at delivery, mode of delivery, fetal and neonatal survival, and disease confirmation. RESULTS: Of the 16 fetuses, 4 had mediastinal tumors: 2 with pericardial teratomas (both of whom died in utero) and 2 with cardiac rhabdomyomas (1 died; the other presented tuberous sclerosis and is alive at 2 years of age). Four patients had cervical tumors (3 died; 1 survived and is alive and well), and 8 had abdominal tumors (3 with liver tumors, 4 with a left adrenal mass, and 1 with retroperitoneal teratoma). All eight patients with an abdominal tumor are alive and well. CONCLUSIONS: Fetal tumors are rare, and the prognosis seems to depend on their location and size. Although easier to detect, cervical and mediastinal tumors have a worse prognosis. Abdominal masses are more difficult to detect but have a better prognosis.

Abdominal Neoplasms↗
PubMed2001

Pylephlebitis, portal-mesenteric thrombosis, and multiple liver abscesses owing to perforated appendicitis.

The authors report a case of a ruptured retrocecal appendix presenting with pylephlebitis, portal-mesenteric thrombosis, and multiple liver abscesses in an 8-year-old boy. A right hemicolectomy was performed, and liver abscesses were drained. The patient was treated with long-term antibiotics and anticoagulation. This the only case of perforated appendicitis associated with these 3 morbid complications in the modern literature.

Anti-Bacterial Agents↗
PubMed2001

Radiofrequency ablation of human fetal sacrococcygeal teratoma.

OBJECTIVE: Fetuses with solid, highly vascularized sacrococcygeal teratomas can die as a result of the vascular steal syndrome. This is the first report in which a percutaneous technique, radiofrequency ablation, was used to interrupt blood flow to a sacrococcygeal teratoma in 4 human fetuses. STUDY DESIGN: A radiofrequency ablation probe was percutaneously inserted into the fetal tumor under ultrasonographic guidance. In 2 fetuses a significant portion of the tumor mass was ablated, whereas in the other 2 fetuses only the major feeding vessels were targeted. RESULTS: Two infants were delivered at 28 and 31 weeks' gestation, respectively, and are doing well. In 2 other cases hemorrhage into the tumor led to an unfavorable fetal outcome. CONCLUSION: Ablation of a majority of the tumor tissue in sacrococcygeal teratoma is not necessary and proved fatal in two instances. Targeted ablation of the feeding tumor vessels diminishes blood flow sufficiently to reverse high-output fetal heart failure.

Adult↗
PubMed2001

Fetoscopic temporary tracheal occlusion by means of detachable balloon for congenital diaphragmatic hernia.

Occlusion of the fetal trachea blocks the egress of fetal lung fluid and stimulates the growth of hypoplastic lungs in fetuses with diaphragmatic hernia. Accomplishing temporary and reversible occlusion of the fetal trachea has proven difficult without invasive fetal surgery. Using simultaneous real-time ultrasonography and fetal bronchoscopy through a single uterine port, we placed a detachable balloon in the trachea of 2 fetuses with severe diaphragmatic hernia. In both fetuses the fetal lung subsequently enlarged, allowing survival after birth.

Adult↗
PubMed2001

Fetal surgery for myelomeningocele.

Myelomeningocele is a devastating birth defect affecting a significant number of live births worldwide. Prenatal repair of myelomeningocele has been performed in the United States for 5 years with mixed results. The initial intent was to preserve distal neurological function by covering the exposed spinal cord. Although there has been relatively little effect on distal sensorimotor function, prenatal repair serendipitously led to an apparent reduction in hindbrain herniation and a possible decreased need for ventriculoperitoneal shunting. The long-term clinical consequences of these findings are not clear. What is clear, however, is that further study in the form of a prospective, randomized trial is mandatory.

Animals↗
PubMed2000

Intraoperative radiation therapy for high-risk pediatric neuroblastoma.

PURPOSE: To evaluate the efficacy of intraoperative radiation therapy (IORT) in the treatment of high-risk pediatric neuroblastoma. METHODS AND MATERIALS: Between 1986 and 1998, 23 children received IORT for pediatric neuroblastoma. Electron beam energies ranged from 4 MeV to 16 MeV and median dose was 10 Gy (7-16 Gy). RESULTS: Twenty-one of 23 patients were classified as high-risk. A gross total resection (GTR) was achieved in 18 patients, of whom 6 experienced disease recurrence, 2 of which included a locoregional relapse as a component of failure. Fourteen of 18 patients receiving IORT after a GTR are disease-free survivors. A second subset of 5 patients had a subtotal resection (STR), with gross residual disease remaining after surgery. All 5 patients recurred locally, and all died of their disease. IORT was extremely well-tolerated in our cohort. Surgical resection and IORT resulted in the narrowing of the abdominal aorta and an atrophic kidney in 1 patient. CONCLUSIONS: For high-risk neuroblastoma patients, IORT as the only radiotherapy to the primary, produced excellent local control after a GTR. However, IORT as the sole radiotherapy to the primary was inadequate for patients with extensive adenopathy or an STR. In this setting, we are exploring the use of IORT as a boost in conjunction with external beam radiation therapy.

Adolescent↗
PubMed2000

Balloon tracheal occlusion for congenital diaphragmatic hernia: experimental studies.

PURPOSE: Temporary tracheal occlusion is an effective strategy to enlarge fetal lungs, but the optimal technique to accomplish occlusion is unknown. External clips are effective when applied fetoscopically (Fetendo clip), but require a difficult fetal neck dissection. This study was undertaken to assess the feasibility of intratracheal balloon occlusion, revisiting the internal occlusion strategy. METHODS: (1) The internal diameter (ID) of human fetal trachea (53 fetuses; 14 to 41 weeks' gestation) was compared using a computer-assisted image analyzer and sonography, ex vivo. (2) Volume to diameter relationship of the balloon (balloon configuration curve) was defined using an image analyzing computer. (3) Using the trachea of fetal sheep, pressures that break balloon tracheal seal (seal pressure) were investigated. RESULTS: (1) Between 16 and 41 weeks' gestation, tracheal ID (range, 0.7 to 5.4 mm) correlates significantly with gestational age. (2) Balloon volume required to achieve tracheal seal could be determined based on the tracheal growth curve and the balloon configuration curve. (3) Tracheal seal breaking points varied depending on the tracheal specimen tested. CONCLUSION: Internal tracheal occlusion using a balloon is feasible with minimal tracheal damage if the balloon volume is adjusted to fetal tracheal growth.

Balloon Occlusion↗
PubMed2000

Tracheal stenosis: the long and the short of it.

PURPOSE: The aim of this study was to present slide tracheoplasty as the procedure of choice for tracheal stenosis. From 1990 through 1997, patients referred to University of California, San Francisco for tracheal stenosis were managed by resection and anastomosis. During this period, other centers reported successful use of slide tracheoplasty and, from 1998 to 1999, we performed 3 slide tracheoplasties in addition to 3 more resection and anastomosis procedures. METHODS: Between 1990 and 1999, 9 patients with tracheal stenosis of varying lengths underwent surgery at the University of California, San Francisco. They were treated surgically with either resection and anastomosis or with slide tracheoplasty. RESULTS: Two of the 6 patients treated by resection and anastomosis had an anastomotic breakdown; all 3 patients undergoing slide tracheoplasty did not have anastomotic problems. Technically, a slide tracheoplasty has only one half the tension distributed over an oblique anastomosis that is more than the circumferential length of a resection and anastomosis approach. CONCLUSION: Based on these results, a slide tracheoplasty may be the procedure of choice for tracheal stenosis, whether long or short.

Adolescent↗
PubMed2000

Urinary tract masses.

Disorders of the urinary tract account for the majority of abdominal masses in infants. The most common causes of a flank mass in a neonate are a hydronephrotic kidney followed by a multicystic kidney. An increasing number of disorders resulting in hydronephrosis are diagnosed by prenatal ultrasound scan, and their natural history continues to be elucidated. This review will examine the common anatomic and functional causes of fetal and neonatal hydronephrosis as well as the diagnosis and treatment of solid and cystic urinary tract masses.

Abdomen↗
PubMed2000

Hindbrain herniation develops in surgically created myelomeningocele but is absent after repair in fetal lambs.

OBJECTIVE: The purpose of our study was to determine whether prenatal repair of myelomeningocele prevents or reverses hindbrain herniation in the sheep model. STUDY DESIGN: A myelomeningocele was surgically created in fetal sheep. One group was repaired later in utero; the others were delivered without repair. After delivery, lambs were assessed for the presence of hindbrain herniation. RESULTS: In all lambs that had not undergone repair of the myelomeningocele, severe hindbrain herniation developed, whereas the brains of all lambs that had undergone fetal repair were normal. CONCLUSION: Prenatal repair of myelomeningocele prevents or reverses development of hindbrain herniation in the fetal lamb model.

Animals↗
PubMed2000

Hepatic pulmonary fusion in neonates.

OBJECTIVE: This report discusses the relationship of supradiaphragmatic hepatic tissue that is fused to the lung (through a diaphragmatic defect) with pulmonary hypoplasia-a new constellation of findings. CONCLUSION: Hepatic pulmonary fusion should be suspected in instances of apparent diaphragmatic hernia characterized by mediastinal shift towards the hypoplastic lung or when the mediastinum does not shift away from the mass.

Female↗