Thoracoscopic esophagomyotomy for achalasia in a child.
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Biomedical subjects
Publications and source records attributed to T M Crombleholme.
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PURPOSE: To evaluate the usefulness of prenatal magnetic resonance (MR) imaging in determination of the position of the fetal liver and amount of lung tissue in left-sided congenital diaphragmatic hernia. MATERIALS AND METHODS: In three pregnant women, MR imaging was performed with a 1.5-T magnet and fast gradient-echo, half-Fourier single-shot turbo spin-echo, and echo-planar sequences. MR imaging findings were compared with those from ultrasound (US) performed the same day. The fetuses were 20, 23, and 32 weeks gestational age. RESULTS: The fetal liver was demonstrated in the chest in all three fetuses with MR imaging and in only one fetus with US. The best images of the fetal liver were obtained with a T1-weighted gradient-echo sequence. The best images of the entire fetus were obtained with a half-Fourier single-shot turbo spin-echo sequence. CONCLUSION: In these fetuses, MR imaging proved important by clearly demonstrating herniation of fetal liver into the chest, thereby changing family counseling and prenatal care.
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The natural history of fetal ureteroceles is poorly understood. We report two cases of fetal ureterocele which add to our understanding of its natural history. In both cases, the fetal ureterocele 'resolved' later in gestation. The postnatal findings emphasize the importance of an aggressive newborn evaluation despite apparent resolution of the ureterocele on prenatal ultrasound examination.
Unrecognized biliary tract obstruction due to choledochal cysts or biliary atresia can result in significant morbidity and mortality. Diagnosis in utero of choledochal cyst allows prompt postnatal diagnostic evaluation and appropriate surgical therapy to be instituted early in life. This may minimize the potential complications of cholangitis, cirrhosis, and liver failure in infants with choledochal cysts.
Hyaluronic acid (HA)-dependent pericellular matrices (PCM) play a role in embryonic differentiation of mesodermal cells. Fetal fibroblasts have significantly larger PCMs than postnatal fibroblasts. To determine if this property is intrinsic to fetal fibroblasts or induced by factors in the fetal environment, we studied the effect of fetal bovine serum (FBS) of varying gestational age on human fetal, newborn, and adult fibroblast PCM formation. Cultured human fetal, newborn, and adult fibroblasts were plated in triplicate at a density of 1 x 10(5) cells and incubated in medium alone, medium containing 10% pooled FBS, or FBS from the first, second, or third trimesters. The cells were photographed and morphometric analysis of PCM was performed by the erythrocyte exclusion technique. PCM size was expressed as a ratio of the maximal width of the cell matrix to the maximal width of the cell. The unpaired Student's t test was used for statistical analysis. The earlier the gestational age of FBS used, the larger the PCM observed in fetal and newborn fibroblasts. The PCM of fetal fibroblasts was significantly larger (P < 0.001) than that of newborn and adult fibroblasts at each gestational age of FBS tested (fetal >> newborn > adult). Medium containing pooled FBS caused a significant (P < 0.001) increase in PCM size in all cell lines compared with serum-free medium. There are both intrinsic and extrinsic factors which affect PCM size. These factors which affect HA-dependent PCM size may contribute to a permissive microenvironment for cell migration, proliferation, and development which may be important for scarless fetal wound repair.
To avoid the need for a gastrostomy and parenteral nutrition during the 7- to 10-day healing period after esophageal anastomosis, the authors modified their technique for esophageal atresia repair to include placement of a transanastomotic feeding tube. A SILASTIC transanastomotic feeding tube and early enteral nutrition was used for 19 of 23 consecutively treated patients after repair of esophageal atresia and tracheoesophageal fistula. One of the 19 patients had recurrent fistula and another had an anastomotic leak. Five patients had significant gastroesophageal reflux (noted on barium esophagram), and four had strictures that required dilatation. Parenteral nutrition was necessary for only two patients. The authors conclude that transanastomotic feeding tubes and early enteral nutrition are safe and effective, reduce costs, and do not appear to increase the incidence of anastomotic leaks, strictures, or gastroesophageal reflux.
Immune thrombocytopenic purpura (ITP) frequently leads to splenectomy. Accessory spleens maybe found in a variety of locations, and may not be readily apparent. Retained accessory splenic tissue can lead to recurrent ITP, which, this report (involving multiple relapses in a single patient) demonstrates.
PURPOSE: Pediatric surgeons are increasingly called on by obstetrical colleagues to counsel parents about the implications of a prenatal ultrasound finding. Our understanding of the natural history of many prenatally diagnosed surgical conditions has grown significantly in recent years. Whether prenatal surgical consultation can influence perinatal course had not been investigated. METHODS: During an 21-month period, 12,865 prenatal ultrasound studies were performed on a total of 4,551 patients, and 221 prenatal surgical consultations were obtained through a newly established fetal treatment program at a tertiary care prenatal diagnostic center. To evaluate the impact of prenatal pediatric surgical consultation on perinatal course, the authors reviewed changes in management including termination of pregnancy, in utero intervention, and altered site, mode, or timing of delivery. RESULTS: Two hundred twenty-one fetuses were referred for consultation; their 234 congenital anomalies included genitourinary (36%), thoracic (16%), intraabdominal (14.5%), abdominal wall (10.6%), neurological (9%), skeletal (6%), and head and neck (2.5%) defects; 2.5% had tumors and 2.5% were twin pregnancies. Pregnancy was terminated in 9.5% of cases, because of patient request, chromosomal abnormality, or dismal prognosis. In 3.6%, the decision to terminate was changed as a result of consultation. Site of delivery was changed as a result of consultation in 37% to facilitate postnatal evaluation and initiate immediate treatment. Mode of delivery was changed in 6.8% to prevent dystocia, hemorrhage into a tumor, as in sacrococcygeal teratoma, or to provide an emergency airway, as in cervical teratoma. The timing of delivery was changed in 4.5% to avoid further damage to fetal organs in cases of obstructive uropathy, gastroschisis, sacrococcygeal teratoma with high-output failure, and hydrocephalus. Five percent (11) underwent treatment in utero for fetal hydrothorax, obstructive uropathy, twin-twin transfusion syndrome, or lymphangioma. The overall perinatal mortality rate was 2.5%. CONCLUSION: Prenatal pediatric surgical consultation may have a significant impact on the perinatal management of the fetus with a surgically correctable congenital anomaly. Providing obstetric colleagues and families with valuable insight into the surgical management of anomalies allows fetal intervention when appropriate, and delivery in an appropriate setting, by the safest mode of delivery, and at the gestational age appropriate to minimize effects of the anomaly.
PURPOSE: We further define the natural history and management of minimal fetal hydronephrosis. MATERIALS AND METHODS: Experience with minimal fetal hydronephrosis (renal pelvic diameter 4 to 10 mm. before 20 weeks of gestation and 5 to 10 mm. from 20 to 24 weeks) was reviewed for 18 months at New England Medical Center. RESULTS: The incidence of minimal fetal hydronephrosis was 2.2% in the 5,900 fetuses screened by ultrasound. In 63% of cases prenatal ultrasound was done more than once, and revealed that dilatation of the urinary tract was stable during gestation in 31 (25%), and became normal in 35 (29%) and worse in 11 (9%). A total of 63 patients (103 renal units) followed for up to 1 year underwent postnatal sonography. Those with abnormalities were screened with a voiding cystourethrogram and renal scan, including 40 with normal postnatal ultrasound and no changes on subsequent evaluation. Of the 23 patients with abnormal findings 4 had functionally significant ureteropelvic junction obstruction and 1 had severe vesicoureteral reflux. A retrospective analysis of the sonograms of patients with minimal fetal hydronephrosis and persistence or progression of hydronephrosis revealed calicectasis, progression of minimal fetal hydronephrosis in utero and/or abnormal renal echogenicity in each fetus. CONCLUSIONS: Our study shows that in a small but significant number of fetuses minimal fetal hydronephrosis will progress, whereas in most it will resolve.
PURPOSE: Desmoplastic round cell tumor (DSCT) is a highly malignant abdominal tumor first described in 1991, with subsequent cases predominantly noted in pathologic case reports. The authors evaluated response to alternating, intensive chemotherapy in three patients with DSCT, and reviewed the clinical experience with this newly described tumor as reported in the literature. PATIENTS AND METHODS: Three adolescent boys with DSCT were treated intravenously with vincristine 2 mg/m2, doxorubicin 75 mg/m2, cyclophosphamide 1.8 g/m2, alternating with 5-day cycles of etoposide 100 mg/m2/day, ifosfamide 1.8 g/m2/day for a total of 11-15 courses. RESULTS: Each patient showed initial tumor regression during chemotherapy, but developed progressive disease within 8-18 months. One patient subsequently showed a transient response to doxorubicin 45 mg/m2 plus 5-fluorouracil 500-600 mg/m2. All three patients died of disease within 20 months of diagnosis. A comprehensive literature review of clinical data on 101 reported cases of DSCT is presented. The median age was 21 years (range 6-38 years) with 78 male patients and 23 female patients. Ninety-nine cases involved tumor mass in the abdominal-pelvic cavity in proximity to the mesentery. Metastatic seeding to the omentum was most common, followed by spread of disease to liver, distant lymph nodes, lung, and occasionally to scrotum or to ovary. Tumor response to chemotherapy was noted in approximately 50% of 40 patients who received combinations of doxorubicin, cisplatin, cyclophosphamide, etoposide, and/or 5-fluorouracil. Four of 13 patients who received additional radiotherapy were alive at 24-48 months. Median survival was 17 months (range: 3-72 months), with only two patients reported disease free beyond 2 years at 40 and 48 months. CONCLUSION: DSCT should be included in the differential diagnosis of small round cell tumors in children and young adults. Tumor regression has been noted during multiagent chemotherapy, but prolonged survival is rare with current therapies.
BACKGROUND: Percutaneous endoscopic gastrostomy (PEG) by the "push" technique avoids peri-catheter infection, repeated insertion of the endoscope, potential esophageal injury from the catheter, and the possible need for another endoscopy for catheter removal associated with the "pull" technique. In small infants, however, the "push" technique could result in loss of gastric insufflation and pneumoperitoneum during tract dilatation. A simple modification of the "push" technique has eliminated this problem. STUDY DESIGN: During a 16-month period, 22 infants and children underwent PEG insertion using our modified "push" technique. These cases were reviewed for patient characteristics including age, weight, indication for the procedure, duration of the procedure, cost, conversion to open technique, and complications. RESULTS: We have used the modified "push" technique to place PEG tubes in 20 infants and children aged four weeks to 15 years (mean, 13 months), weighing 2.7 to 36 kg (median, 6.0 kg), indicated for failure to thrive due to cystic fibrosis (n=3) or neurologic impairment (n=10). These patients have had follow-up examination from nine to 30 months after the procedure. Operative time averaged 15 minutes. The "push" technique was successful in 95 percent of patients with one failure caused by loss of gastric insufflation when Fogarty balloons failed. All PEGs were used within 24 hours. There were no deaths and no peri-catheter infections. CONCLUSIONS: A simple modification of the "push" technique of PEG insertion eliminated problems with loss of gastric insufflation previously encountered in small infants. The modified "push" technique is safe, simple, and quick, obviating potential risks inherent in the "pull" technique when applied in infants.
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A fetal lamb model of amniotic band syndrome (ABS) was developed to study the pathophysiology of banded extremities and evaluate the possibility of in utero treatment with the potential for functional recovery. Eight fetal lambs underwent banding of their extremities with umbilical tape at 100 days' gestation. Two lambs aborted after the open fetal surgery. The limbs of two unoperated newborn lambs served as controls in addition to five sham-operated control limbs that had no bands applied. Nine limbs were banded without reducing blood flow assessed by laser doppler (group 1), and 6 limbs were similarly banded and released fetoscopically at 125 days' gestation. Four limbs were banded, with a mean reduction in blood flow to the limb of 18.7% (group 2) by laser doppler flowmeter. Shortly after birth the lambs were killed, and segmental limb length, circumference, joint range of motion, and histology were evaluated. At birth, banded limbs showed marked brawny edema and absence of wool distal to the band. Segmental limb measurements showed shorter distal forelimbs in banded limbs compared with controls (10.97 +/- 0.59 versus 12.98 +/- 0.69, P < .05). Banded limbs were associated with progressive increase in hoof circumference (P < .03) and a decrease in joint range of motion (P < .003). In sharp contrast, there were no differences between fetoscopically released limbs and control limbs in any of the parameters measured. Histology of banded extremities showed edema, venous and lymphatic congestion, and fibrosis compared with controls. This model of ABS in fetal lambs is simple, reproducible, and replicates all the clinical features of extremity ABS.(ABSTRACT TRUNCATED AT 250 WORDS)
The authors reviewed their experience with meconium peritonitis (MP) diagnosed in utero to define criteria for prenatal and postnatal management. Prenatal diagnosis was made by identifying abdominal calcification on serial ultrasound examinations in nine fetuses, between 18 and 37 weeks' gestation. Cases without associated bowel abnormalities were considered "simple MP" and those with bowel abnormalities were considered "complex MP." Five cases of simple MP were identified at 18, 23, 30, 34, and 37 weeks' gestation. These five fetuses were delivered at term and had normal abdominal examinations. Abdominal radiographs were obtained in three showing normal bowel gas patterns, and abdominal calcifications in only two. All five patients were fed uneventfully. Four cases of complex MP were identified at 26, 26, 31, and 31 weeks' gestation. All four fetuses had dilated loops of bowel. Two of the four had meconium cysts, one of which was associated with ascites and the other with polyhydramnios. Shortly after birth both infants with meconium cysts required ileal resection and ileostomy for ileal atresia and ileal perforation, respectively. The remaining two infants had no evidence of dilated bowel, meconium cyst, or ascites on postnatal radiograph and were fed uneventfully. These data suggest that only 22% of fetuses with a prenatal diagnosis of MP develop complications that require postnatal operation. Gestational age at diagnosis does not correlate with postnatal outcome. Fetuses with complex MP are at increased risk for postnatal bowel obstruction and perforation.
Sacrococcygeal teratoma identified in utero is associated with 50% fetal demise, which is caused by hyperdynamic cardiac failure, hemorrhage, and polyhydramnios-induced preterm labor. A premature infant (26 weeks' gestation) with prenatally diagnosed sacrococcygeal teratoma was managed successfully with initial devascularization to control the hyperdynamic state, followed by staged resection.
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Extension of Wilms' tumor into the renal vein and vena cava complicates the operative treatment of a small but significant number of patients. Two children presented with Wilms' tumor and intracaval tumor extension. After biopsy, both children were given chemotherapy. In one case the tumor completely disappeared from the vena cava, and in the other the tumor regressed substantially, and proximal control became possible at the level of the infrahepatic vena cava. Both children then had an uneventful nephrectomy that was greatly simplified. We conclude that preoperative chemotherapy can induce resolution of intravascular tumor, and may be a useful option in cases of extension of Wilms' tumor into the vena cava.