Edward G. Ford’s research while affiliated with Children's Hospital Los Angeles and other places

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is characterized by hypoperistalsis in the presence of ganglion cells, malrotation, microcolon, bladder distension, and female predilection. We draw attention to the long-term management of a patient with MMIHS and propose a pathogenetic mechanism to account for this syndrome. We propose that the initial event in the pathogenesis of MMIHS is an intramural inflammatory process that affects the gastrointestinal and urinary tracts. This leads to extensive fibrosis which destroys the intestinal neural network, producing hypoperistalsis. The same process causes neuromuscular incoordination in the bladder wall, resulting in irregular bladder contractions against a "closed sphincter" leading to bladder distension. The enlarged bladder then interferes with the rotation of the intestine causing malrotation.

The incidence of significant intraabdominal injuries from blunt trauma in children is low (1-8%); however, a missed intestinal laceration may result in a devastating, if not morbid, consequence. We present retrospective case studies of eight children with intestinal lacerations following blunt trauma. Each patient has a unique anatomic defect with misleading initial laboratory and radiographic studies. We did not find a single laboratory or roentgenographic study to be consistently reliable for diagnosis of hollow viscus laceration. The combination of a high index of suspicion and serial abdominal examinations, coupled with subsequent comparative laboratory and radiographic evaluations, proved most reliable in identifying patients with intestinal injury.

Symptomatic Salmonella infections usually manifest as self-limited gastrointestinal distress. Patients with chronic systemic illnesses or those who are immunosuppressed may rarely present with Salmonella infection as distant suppurative abscesses. We present a previously healthy Armenian boy who came to medical attention with abdominal pain, fever, and anemia. Abdominal computed tomography (CT) scan showed a cystic suprarenal mass that was surgically explored and found to be a retroperitoneal Salmonella abscess. Postoperative CT scan showed resolving inflammation. A 6-month follow-up CT showed a large suprarenal tumor, which at exploration was found to be neuroblastoma. To our knowledge, Salmonella has never been reported presenting as a solitary retroperitoneal abscess, and neuroblastoma has not been described presenting as a Salmonella abscess. The patient is also unusual because the abscess contained a species unusual for suppurative salmonellosis.

Neonates with esophageal atresia and tracheoesophageal fistula (TEF) may present with respiratory distress. Intubation and mechanical ventilation may force air from the tracheobronchial tree, through a distal fistula and into the gastrointestinal tract. We present a newborn with TEF who became moribund during mechanical ventilation. High ventilator pressures transmitted via the TEF caused over distention of the gastrointestinal tract and perforation of a Meckel's diverticulum.

Extracorporeal membrane oxygenation (ECMO) is lifesaving for infants with severe respiratory distress but is complicated by severe intracranial hemorrhage in 10% to 30% of patients. Intracranial venous hypertension, as a result of ligation of the internal jugular vein (IJV), has been hypothesized as a contributing factor to cerebral edema and subsequent hemorrhage. Accessory cephalad IJV cannulation may serve as a means of additional venous drainage to the pump as well as protection against intracranial venous hypertension. Proximal and distal cannulation of the IJV were studied in a primate model. The parameters monitored included sagittal sinus, right and left ventricular pressures as well as venous pressure in the ECMO circuit. The cephalad venous cannula was clamped and unclamped at 30-minute intervals. There was no significant difference in sagittal sinus or intracranial pressures during periods of cephalad cannula clamping or unclamping. Venous return was augmented when the cephalad cannula was unclamped. Cephalad cannulation has no demonstrable protective effect on intracranial, subarachnoid or venous pressures but does improve venous return to the ECMO circuit. It is concluded that cephalad venous cannulation is not necessary in all cases and should be reserved for those patients requiring additional venous drainage to support pump flow.

In 1976, we presented our experience in the surgical management of patients with peripheral congenital arteriovenous fistulae (CAVF). This report updates our experience and specifically describes subsequent experience with intraarterial embolization therapy. Twenty-six infants and children with CAVFs have been treated at Children's Hospital of Los Angeles from 1966 to 1990. The majority of lesions were lower extremity (12), followed by upper extremity (8), shoulder girdle (3), and thorax/neck (3). Seventeen patients had lesions in multiple locations. All patients under 1 year of age had upper extremity lesions, all in the 5- to 10-year age group had lower extremity lesions, and all over 15 years of age at presentation required amputation. Twelve patients had initial embolization therapy and 12 had surgery. Seven of eight patients followed after embolization have improved. Embolization is not effective in large or ulcerated lesions. All patients with embolization alone have residual disease and three of eight have discrepancy in extremity length. Surgery has a 50% complication rate and 7 of 10 followed surgery patients have residual disease. Radiologic catheter techniques and embolization provide a valuable alternative therapy for CAVFs and should be used as the primary form of treatment in most cases.

Communicating bronchopulmonary foregut malformations (CBPFMs) are characterized by a fistula between an isolated portion of respiratory tissue (ie, a lung, a lung lobe, or a segment) and esophagus or stomach. We combine our 30-year (1959 to 1989) experience of 6 cases with 51 reported patients to propose a CBPFM classification supported by a proposed embryogenesis theory. Group I (16%): anomaly is associated with esophageal atresia and tracheoesophageal fistula. Group II (33%): one lung originates from the lower esophagus. Group III (46%): an isolated anatomic lung lobe or segment communicates with the esophagus or stomach. Group IV (5%): A portion of the normal bronchial system communicates with the esophagus. The portion of the lung served by the communicating bronchus receives systemic blood supply. The right and left lung sacs curve dorsally to embrace the lower esophagus during normal lung development. At this stage a part of the lung bud joins the esophagus. This segment then breaks away from the main pulmonary anlage to form a CBPFM. CBPFMs should be considered in the workup of infants with respiratory distress and/or recurrent pneumonias. Patients with suspected pulmonary sequestration should undergo contrast studies to exclude a gastrointestinal communication.

Several congenital anomalies of the portal vein and inferior vena cava (IVC) have been reported; however, reports of a congenital communication between the portal vein and IVC are few. We report a patient who was found to have a natural shunt between the extrahepatic portal vein and the IVC that was discovered when she underwent a right hepatic lobectomy for an undifferentiated liver sarcoma. The patient also had agenesis of the right kidney. We have not been able to find a similar case reported in English literature.

Extracorporeal membrane oxygenation (ECMO) provides an adjunct to support of neonates with respiratory distress. The rate of venous drainage is the limiting step to the amount of arterial flow that may be generated during ECMO. We present a 3-kg, 37-week gestation female with congenital diaphragmatic hernia (CDH) whose anatomic venous drainage was insufficient to allow total extracorporeal support. The internal jugular vein was of insufficient size to allow placement of a cannula large enough to support adequate bypass flow. We established minimally acceptable flow by simultaneous caudad and cephalad catheterization of the internal vein. We performed a right lower quadrant retroperitoneal dissection and cannulated the common iliac vein when systemic pressures deteriorated at 24 hours with marginal venous drainage. After triple cannulation the bypass flows were normal. The right leg developed mild cyanosis and edema immediately following cannulation, but these changes resolved over 24 hours with elevation. The common iliac vein provides a desirable route for augmentation of venous drainage in the patient with difficult standard, or cephalad, jugular venous access for extracorporeal support.