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.

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.

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.