Laparoscopic resection of type 1 choledochal cysts in pediatric patients.

BACKGROUND: Choledochal cyst resection and hepaticojejunostomy have historically been performed using an open technique. We describe here the largest single experience with this procedure using laparoscopic techniques in eight consecutive pediatric patients. METHODS: There were six girls and two boys, of ages ranging from 3 months to 13 years. All had type I choledochal cysts. Three were asymptomatic, having been noted on prenatal ultrasonography. Five ports were utilized: one 5-mm telescope port at the umbilicus, two 3-mm operating ports on both sides of the umbilicus, one 5-mm left subcostal port for liver retraction, and one LLQ 5-mm assistant port. RESULTS: The median operating time was 155 min (range 110-250 min), with one conversion to an open procedure due to a high transection of the cyst leading to partial retraction of the left hepatic duct into the liver substance. Mean hospital stay was 3 days. At a mean follow-up of 18.8 months, all patients were anicteric and asymptomatic. CONCLUSIONS: Laparoscopic resection of choledochal cysts can be performed safely in pediatric patients with minimal morbidity and good long-term results.

The lung-to-head ratio and fetoscopic temporary tracheal occlusion: prediction of survival in severe left congenital diaphragmatic hernia.

OBJECTIVES: To evaluate the reliability of sonographic lung-to-head ratio (LHR) measurement as a predictor of survival in fetuses with congenital diaphragmatic hernia (CDH) and to compare the probability of survival in those with temporary tracheal occlusion (TO) or standard care with respect to the LHR. METHODS: Fifty-six fetuses with left CDH with liver herniated into the thorax at complete prenatal evaluation were included in logistic regression analyses of antenatal predictors of survival to hospital discharge. Sixteen subjects underwent TO and 40 received standard care. RESULTS: LHR was a significant predictor of survival, with probability of survival increasing with increasing LHR (odds ratio (OR) 8.5, P = 0.04). When subjects with anomalies were excluded, the LHR effect was similar after adjustment for TO (OR 7.1, P = 0.11). Linear spline models suggested a plateau in survival at an LHR of 1.0 and all models suggested increased odds of survival with TO. Minimum LHR measurements had a high degree of inter- and intraobserver agreement (intraclass correlation coefficients of 0.70 and 0.80, respectively). CONCLUSIONS: Calculation of the LHR in fetuses with CDH is a reliable and powerful predictor of survival to hospital discharge, although improving odds of survival may plateau at an LHR of 1.0. TO may have an independent benefit on survival to hospital discharge.

Reversed latissimus dorsi muscle flap for repair of recurrent congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: Neonates with large congenital diaphragmatic hernias (CDH) require prosthetic patch closure of the defect because of the paucity of native diaphragmatic tissue. As the child grows, patch separation can occur necessitating reoperation. Use of vascularized autologous tissue may decrease the incidence of reherniation as tissue incorporation and growth may be improved. The authors report our early experience using a local muscle advancement flap with microneural anastomosis for those children in whom reherniation develops after prosthetic patch placement. METHODS: Seven patients with CDH (6 left and 1 right) whose synthetic diaphragmatic patch separated from the chest wall resulting in a clinically significant recurrent hernia were followed up with prospectively. After dissecting the ipsilateral latissimus dorsi off the chest wall and dividing the thoracodorsal neurovascular bundle (based on its lumbar blood supply), the synthetic patch was removed via an eighth intercostal incision. The muscle flap was placed into the hemithorax through the bed of the tenth rib and sutured in place over a Vicryl mesh scaffold. The thoracodorsal nerve was anastomosed to the phrenic nerve. Functional analysis of the flap was performed in 4 patients. RESULTS: Age at placement of the muscle graft ranged from 2 months to 48 months (median, 24 months). There has been no evidence of reherniation after placement of the muscle graft. Long-term outcome and functional analysis of the flap was available in 4 patients (mean, 19 months). Two infants had fluoroscopic and sonographic evidence of nonparadoxical neodiaphragmatic motion. In one of these, electromyographic evidence of function was documented with a phrenic nerve conduction velocity of 22 meters per second. The third infant showed no evidence of neodiaphragmatic motion, and the fourth infant had paradoxical motion. CONCLUSIONS: This is the first direct documentation of phrenic nerve function in an infant with CDH. An innervated reversed latissimus dorsi (RLD) flap reconstruction for recurrent CDH provides an alternative to prosthetic patch repair. This technique offers the advantages of autologous vascularized tissue with potential phrenic nerve innervation and physiologic neodiaphragmatic motion.

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.

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.

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.

[Fetal surgery for severe congenital abnormalities]. / Fetale Chirurgie für schwere kongenitale Fehlbildungen.

BACKGROUND: Over the past two decades the diagnosis of life threatening congenital malformations has evolved rapidly. Sophisticated and powerful new imaging and sampling techniques have stripped the veil of mystery from the once secretive fetus. Early detection and close follow-up of the fetus with congenital malformations have allowed us to define their natural history, determine the clinical features that affect clinical outcome, and plan management approaches to improve prognosis. Fetal surgical intervention is the logical culmination of the progress in fetal diagnosis. The purpose of this article is to describe the current techniques and recent advances in prenatal diagnosis and fetal intervention of severe congenital malformation. MATERIAL AND METHODS: A complete review of the literature and our own experience concerning fetal surgery was performed. RESULTS: Although most prenatally diagnosed malformations are best managed by appropriate medical and surgical therapy after maternal transport and planned delivery at a tertiary care center, an expanding number of simple anatomical abnormalities with predictable, lethal consequences have been successfully corrected before birth. A malformation amenable to prenatal surgical intervention must fulfill a number of conditions. It must be severe enough to warrant the risks associated with in utero treatment and must be reliably detectable before birth. Additionally, the pathophysiology must be reversible by fetal surgery, significantly improving the prognosis over post-natal treatment. Many technical intricacies of open fetal surgery have been solved, but pre-term labor and premature rupture of membranes remain a omnipresent risks to both the mother and the fetus. To reduce maternal morbidity and the risk of prematurity we developed minimally invasive techniques to treat the fetus prenatally. Current indications of fetal surgery include the treatment of congenital diaphragmatic hernia, cystic adenomatoid malformation of the lung, sacrococcygeal teratoma, obstructive uropathy, twin-to-twin-transfusion-syndrome and myelomeningocele. Minimally invasive surgical techniques (FETENDO) have significantly lessened the incidence of preterm labor and promise to extend the indications for fetal surgical intervention. CONCLUSIONS: Fetal surgical therapy for severe congenital malformations may improve the outcome of selected patients. The development of FETENDO will in all probability reduce the importance of open fetal surgery in the future.

Endoscopic techniques in fetal surgery.

Fetal endoscopic surgery (FETENDO) involves many techniques that allow surgical procedures to be performed inside the uterus without an hysterotomy. The impetus for developing these minimal access techniques for fetal surgery is the unusual occurrence with an open hysterotomy of preterm labor, premature rupture of membranes, and maternal complications resulting from tocolytic therapy. The unique requirements of this approach necessitated a modification of existing endoscopic techniques, the development of novel fetoscopic instruments, and the inclusion of a wide variety of specialists. Technical expertise in the field and a natural evolution of techniques have given rise to innovative repairs previously not envisioned. Severe congenital diaphragmatic hernia, diseases of monochorionic twins, and obstructive uropathy have already been successfully treated using fetoscopic surgical techniques. Fetoscopic correction of many other non-life threatening anomalies continues to evolve. The future of fetoscopic surgical intervention depends on the continual evolution of novel approaches to disease, the elucidation of the pathophysiology and treatment of other fetal disorders, and a better understanding of treatment of complications of such intervention.

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.

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.

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.

Laparoscopic Heller myotomy and Dor fundoplication for esophageal achalasia in children.

BACKGROUND/PURPOSE: In the past, surgical treatment in achalasia usually has been reserved for patients whose dysphagia does not respond to pneumatic dilatation. The success of minimally invasive myotomy, however, has resulted in a shift in practice in adult patients, whereby laparoscopic surgery is becoming preferred as primary treatment by most gastroenterologists and surgeons. The aim of this study was to assess the efficacy of laparoscopic Heller myotomy and Dor fundoplication for esophageal achalasia in children. METHODS: Thirteen patients with esophageal achalasia (median age, 15 years; 6 boys and 7 girls; median duration of symptoms, 24 months) underwent laparoscopic Heller myotomy and Dor fundoplication between 1996 and 1999. Two patients had been treated previously by pneumatic dilatation, and 1 patient had received intrasphincteric Botulinum toxin injections. RESULTS: Median duration of the operation was 130 minutes. The patients were fed after an average of 33 hours, and they all left the hospital within 2 days. At a median follow-up of 19 months, there was no residual dysphagia in any patient. CONCLUSIONS: Laparoscopic Heller myotomy and Dor fundoplication were effective and safe for children with esophageal achalasia. Hospital stay and recovery time was short, and the functional results were excellent. These data support the notion that laparoscopic Heller myotomy should become the primary treatment of esophageal achalasia in children.

Coccidioides immitis in the gallbladder and biliary tree.

Intraabdominal coccidioidomycosis is a very rare entity and usually responds to medical therapy. Operative intervention is reserved for diagnosis or drainage of localized collections. With biliary coccidioidomycosis, medical treatment appears to be ineffective, and biliary tract drainage is necessary for optimal management. A case of coccidioidomycosis in the gallbladder and biliary tree is described and the literature reviewed.

Congenital diaphragmatic hernia: prenatal evaluation with MR lung volumetry--preliminary experience.

PURPOSE: To determine the interobserver variability of prenatal magnetic resonance (MR) lung volumetry and to assess the value of MR lung volumetric findings as predictors of outcome in fetuses with congenital diaphragmatic hernia. MATERIALS AND METHODS: Prenatal MR imaging was performed in 26 fetuses with unilateral congenital diaphragmatic hernia. Two independent observers performed planimetric measurement of lung volume. Relative lung volume was calculated as the observed total lung volume expressed as a percentage of the total lung volume predicted from fetal size. Relative lung volume was correlated with the ultrasonographic lung-head ratio in left-sided congenital diaphragmatic hernias evaluated before 27 weeks gestation (n = 21) and with pregnancy outcome in all cases of isolated left-sided congenital diaphragmatic hernia without prenatal intervention (n = 11). RESULTS: Observers demonstrated excellent agreement in total lung volume measurements at MR imaging, with an intraclass correlation coefficient of 0.95. Relative lung volume was positively correlated with lung-head ratio (r = 0.78, P <.001). By using rank order analysis in the pregnancy outcome group, relative lung volume was predictive of prognosis (P <.05) when adjusted for gestational age at delivery and birth weight. Three of four fetuses with a relative lung volume of less than 40% died. CONCLUSION: Interobserver agreement is high at MR lung volumetry, and its findings are predictive of outcome in fetuses with isolated left-sided congenital diaphragmatic hernia.

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.

Transplantation of a fetus with paternal Thy-1(+)CD34(+)cells for chronic granulomatous disease.

A fetus diagnosed with X-linked chronic granulomatous disease was transplanted with Thy-1(+)CD34(+) cells of paternal origin. The transplant was performed at 14 weeks gestation by ultrasound guided injection into the peritoneal cavity. The fetus was delivered at 38 weeks gestation after an otherwise uneventful pregnancy. Umbilical cord blood was collected and used to determine the level of peripheral blood chimerism as well as levels of functional engrafted cells. Flow cytometry was used to detect donor leukocytes identified as HLA-A2(-)B7(+) cells, whereas recipient cells were identified as HLA-A2(+)B7(-) cells. No evidence of donor cell engraftment above a level of 0.01% was found. PCR was used to detect HLA-DRB1*15(+) donor cells among the recipient's HLA-DRB1*15(-) cells, but no engraftment was seen with a sensitivity of 1:1000. The presence of functional, donor-derived neutrophils was assessed by flow cytometry using two different fluorescent dyes that measure reactive oxygen species generated by the phagocyte NADPH oxidase. No evidence of paternal-derived functional neutrophils above a level of 0.15% was observed. Peripheral blood and bone marrow samples were collected at 6 months of age. Neither sample showed engraftment by HLA typing using both flow cytometry and PCR. Functional phagocytes were also not observed. Furthermore, no indication of immunological tolerance specific for the donor cells was indicated by a mixed lymphocyte reaction assay performed at 6 months of age. While there appears to be no engraftment of the donor stem cells, the transplant caused no harm to the fetus and the child was healthy at 6 months of age. Analyses of fetal tissues, obtained from elective abortions, revealed that CD3(+) T cells and CD56(+)CD3(-) NK cells are present in the liver at 8 weeks gestation and in the blood by 9 weeks gestation. The presence of these lymphocytes may contribute to the lack of donor cell engraftment in the human fetus.

Congenital diaphragmatic hernia associated with a gastroesophageal duplication cyst: a case report.

Severe left congenital diaphragmatic hernia was diagnosed in a baby prenatally, and she underwent hernia repair on the sixth postnatal day of life. She was found to have a huge symptomatic gastroesophageal duplication cyst on day 24 of life. A thoracoabdominal dissection allowed successful cyst excision. J Pediatr Surg 36:626-628.

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.

Safety of chronic fetal vascular access in the sheep model.

OBJECTIVE: Long-term access to the fetal circulation has the potential to open up new perspectives in the treatment of numerous fetal anomalies. The purpose of this study was to investigate the safety of long-term catheterization of fetal placental vessels. METHODS: A midline laparotomy was performed in 4 time-mated pregnant ewes at 125 days' gestation (term 145 days). Placental vessels were exposed by a small uterine incision. A specially designed catheter was inserted into a placental vessel over a length of 3 cm, the distal end of the catheter was tunneled underneath the maternal skin and attached to a subcutaneous port implanted in the maternal flank. All pregnancies were allowed to go to term. RESULTS: Ewes and fetuses tolerated the placement of the catheter and port without complications. The catheter remained patent in all cases. All lambs were delivered vaginally at term and did not require resuscitation after birth. No fetal anomalies or growth restriction were noted. CONCLUSION: The ovine placental vessel can be accessed long term without complication.

Major complications after minimally invasive repair of pectus excavatum: case reports.

BACKGROUND/PURPOSE: A recently introduced technique allows for minimally invasive repair of pectus excavatum deformity. Successful application of the procedure has been reported by several centers. The purpose of this report is to describe the occurrence of 3 major complications in 5 patients. METHODS: These cases are taken from the combined experience of 3 surgeons at different institutions. Operative technique and postoperative management was not uniform. RESULTS: The first complication was cardiac perforation requiring repair. This occurred in an 8-year-old boy who had hemorrhage immediately after transthoracic placement of the clamp. He required urgent sternotomy with right atrial, and right ventricle repair followed by tricuspid valve repair on cardiopulmonary bypass. The second complication is staphylococcal sepsis, bilateral empyema thoracis, and bacterial pericarditis. This 13-year-old boy required bilateral pleural debridement followed 2 days later by open debridement of his heart. The final complication is thoracic outlet syndrome. These patients, age 12, 14, and 15, experienced persistent parasthesias in one upper extremity. One case was further complicated by instability of the bar requiring removal. In the other 2 patients, the symptoms resolved within 4 weeks with the bar in place. CONCLUSIONS: Minimally invasive pectus repair is a new surgical procedure. The spectrum and rate of complications is still emerging. Thorough and critical evaluation of the combined experience from many centers is essential to evaluate fully this novel approach to pectus repair.