One-drug versus two-drug antibiotic therapy in pediatric perforated appendicitis: a prospective randomized study.

A prospective randomized study was undertaken to compare the use of the combination of gentamicin and clindamycin with single agent, cefoxitin, in the treatment of perforated appendicitis in the pediatric patient. In a 3-year period from 1986 to 1989, 56 patients with perforated appendicitis were randomized. Twenty-nine patients received cefoxitin, and 27 patients received gentamicin and clindamycin. Antibiotics were started before operation and continued for a minimum of 6 days after operation. Skin and subcutaneous tissues were left open at surgery and closed secondarily after day 3, if they appeared to be clean. Wounds were considered infected if they developed increased purulence with positive wound cultures. Age range was similar in both groups, with a mean of 9 years (range, 1 to 17 years); 28 were boys and 28 were girls. No changes in antibiotics were required for reasons of susceptibility. No adverse drug reactions occurred in either group. The most common organisms were Escherichia coli in 35 cases (62%) and Bacteroides species in 26 cases (46%). No difference was noted in infection complications in the two groups nor in length of hospital stay. Therefore, no difference is evident in the use of cefoxitin versus gentamicin and clindamycin in the treatment of perforated appendicitis in terms of disease or drug-related complications.

Gastroschisis and omphalocele.

The etiology, presentation, and treatment of the abdominal wall defects of omphalocele and gastroschisis are discussed, based on a ten-year clinical experience in an urban medical center.

A variant of the split notochord syndrome.

Split notochord syndrome has been described in several previous case reports; however, we recently treated a patient with a previously undescribed variant of this syndrome. A 2.5-g baby girl was admitted to the neonatal intensive care unit with a skin-covered mass on the back, and a portion of intestine, with meconium being passed, attached to it. The legs were equino varus, and the anus was anteriorly displaced. Radiographs were consistent with a lumbar split notochord, and the patient was taken to the operating room for closure. During surgery, the skin-covered mass was opened and found to contain loops of small intestine leading to an intussuscepted cecum and an open ended duplication of the cecum attached to the end of the spinal cord. The small intestinal loops exited the abdomen from a peritoneal-lined opening to the back. The loops were returned to the abdomen after releasing the duplication from the cord. The peritoneal connection was closed with a purse string suture, and the abdomen was opened. The duplication of the cecum was partially amputated and the rest was used to create a chimney-type stoma. The intraabdominal exploration was significant for nonrotation as well as a normal rectum. The postoperative course was uneventful. This case is unique because most cases of split notochord only involve a fistula to the rectum. In this case, a major part of the small bowel had herniated. Moreover, there was an open duplication similar to that found in extrophy. In approaching such a situation, it is also important to have the neurosurgeon available to provide adequate closure of the back similar to a myelomeningocele.

Surgical and genetic aspects of persistent müllerian duct syndrome.

Persistent müllerian duct syndrome (PMDS) is characterized by the presence of a uterus, cervix, and fallopian tubes in an otherwise normally differentiated 46.XY male. During embryogenesis, regression of müllerian structures in normal males is mediated by antimüllerian hormone (AMH), also called müllerian inhibiting substance (MIS), produced by fetal Sertoli's cells. PMDS has been attributed to deficient AMH activity or to abnormalities in the AMH receptor. The authors report on two patients with PMDS in whom the abnormalities were discovered during surgery for inguinal hernia and cryptorchidism. During the initial operations in each case, testicular biopsies were obtained, and the gonads and müllerian elements were replaced in the pelvis. A second operative procedure, performed several months later, included proximal salpingectomies with dissection of the vasa deferentia on pedicles of myometrium. This permitted excision of the vestigial uterine corpus, leaving a tiny remnant of cervix with the vasa deferentia. The testes were further mobilized so that bilateral orchidopexies could be completed. In the first case, a molecular abnormality was present at position 377 of the first exon of the AMH gene. Thymine replaced cytosine, which altered a CGG arginine codon to a TGG tryptophan codon, rendering the AMH molecule unstable. The molecular abnormality in the first case differs from the first abnormality in AMH reported by Knebelmann et al, thus indicating heterogeneity in this condition. The molecular basis for deficient AMH activity in the second patient has not yet been defined. No molecular abnormalities were found in the exons of this patient's AMH gene.

A unique chest wall splint for unilateral agenesis of ribs.

Unilateral agenesis of ribs with a "lung hernia," may cause severe respiratory distress in a neonate. Our experience with the use of a temporary external chest splint for stabilization of the congenital flail chest in one patient is presented. The device facilitated extubation and allowed the infant to grow prior to future surgical reconstruction.

The outcome for children with blunt trauma is best at a pediatric trauma center.

PURPOSE: The mortality rate for pediatric trauma patients cared for in adult trauma centers has been shown, by means of TRISS methodology, not to differ significantly from that of the Major Trauma Outcome Study (MTOS). The question remains, however, whether the outcome of injured children is better in a designated pediatric trauma center (DPTC). The authors' hypothesis is that outcome is better at a DPTC. METHODS: The records of 1,797 children (0 to 15 years of age) admitted to a DPTC between 1987 and 1993 were reviewed. TRISS methodology was used to calculate probability of survival for outcome comparison with the MTOS. The data also was compared with outcome in relation to the admitting Glasgow Coma Score (GCS) reported in the National Pediatric Trauma Registry (NPTR). RESULTS: The outcome of all children at this DPTC had a Z score of +1.4199 (P > .1). The Z score of children admitted because of penetrating trauma (PT, n = 460) did not differ significantly from that of the MTOS. However, the children admitted because of blunt trauma (BT, n = 1,337) had a Z score of +3.3501 (M score = .90), which is significantly better than that of the MTOS (P < .001). The BT population with an ISS of > or = 9 (n = 149) had a Z score of +2.8686 (P < .005) (M = .95). By GCS comparison, the BT group had a outcome similar to that reported in the NPTR. Head injury was the cause of death for 26 (84%) of the 31 PT deaths and 20 (83%) of the 24 BT deaths (three of the remaining four had associated severe head injury). Only 1 of 24 (4%) BT liver injuries and 5 (21%) of 24 BT splenic injuries required surgical intervention. This low incidence of liver and splenic surgical invention is similar to that reported by other DPTCs, but for children treated at adult centers the rates are 37% to 58% and 43% to 53% for liver & splenic surgical intervention, respectively. CONCLUSION: Children with BT have a significantly better outcome at a DPTC; the outcome for children with PT does not differ. Successful nonoperative treatment of blunt abdominal injuries is more likely to occur at a DPTC than at adult trauma centers "with pediatric committment." Thus, children with blunt injuries should be taken to a DPTC, when available.

Congenital tracheal stenosis: a review of 22 patients from 1965 to 1987.

Twenty-two infants with congenital tracheal stenosis (CTS) were treated in our hospital between 1965 and 1987. Diagnosis was confirmed by endoscopic and radiographic methods. Patients had a spectrum of tracheobronchial lesions from localized stenosis to more complex deformities involving the carina and bronchi. Other anomalies were found in all patients with the occurrence of vascular slings or rings in 11 patients (50%). Six infants treated nonoperatively died from severe CTS and other lethal anomalies. Five of 16 patients (31%) treated surgically survived. Localized CTS in four cases was treated by dilatation, tracheostomy, or tracheal resection with primary anastomosis (two survivors, 2 non-survivors). Funnel-shaped deformities and extensive tracheobroncial stenosis were treated by tracheal reconstruction using a variety of autogenous tissue and prosthetic grafts (three survivors, nine non-survivors). The overall mortality was 77%. A new intratracheal stent was used in two patients. The stent was a flexible steel spring covered with a silicone rubber sheath. In one patient, it was placed within the trachea at the time of repair and removed later with the bronchoscope. A stent was used in a second patient with intermittent airway obstruction following an esophageal tracheoplasty. In this case, the device failed to alleviate the obstruction, and the infant expired from progressive respiratory failure. Issues of importance in the management of infants with CTS are: (1) adequate evaluation of the tracheobronchial tree, (2) awareness that tracheobronchography may precipitate further respiratory decompensation, (3) assessment of vascular and other anomalies requiring surgical correction, and (4) selection of an appropriate therapeutic approach.

A new intratracheal stent for tracheobronchial reconstruction: experimental and clinical studies.

An intraluminal tracheal stent (ITS) was used experimentally in rabbits and piglets, as well as clinically in infants with tracheal stenosis, to facilitate airway reconstruction. The ITSs were constructed of stainless-steel springs covered with silicone rubber. They were implanted in seven piglets (6 to 8 kg), five rabbits (3 to 5 kg) and three infants. No animals developed severe respiratory distress and all appeared to tolerate the ITS. Postmortem examinations 1 to 8 weeks after surgery showed (1) loss of stent fixation (one pig), (2) increased tracheal secretions, (3) pneumonia (one pig, two rabbits), and (4) focal squamous metaplasia of tracheal mucosa. Stents used to treat three infants (2 to 5 months of age) with complex tracheobronchial stenosis were placed at the time of periosteal tracheoplasty in two. Recurrent stenosis necessitated a second tracheoplasty and stenting in one, and a long tracheostomy tube and balloon dilatations in the other. The third child had endoscopic stent insertion to alleviate severe airway collapse after esophageal tracheoplasty. The child died from progressive respiratory failure after stent dislodgment. Although the stents were well tolerated in animals and they enhanced critical ventilation of all pulmonary lobes in infants after tracheal reconstruction, certain modifications such as alternative methods of fixation, accommodation for tracheal growth, and reduction in tissue reactivity are necessary before further use of the ITS can be advocated.

Cyclic neutropenia with colonic perforation and nonhealing colocutaneous fistula.

Cyclic neutropenia is an hereditary disorder of white blood cells, characterized by profound neutropenic episodes approximately every three weeks. Septic complications are usually limited to cutaneous and oropharyngeal infections. A 4-year-old boy with known cyclic neutropenia was in shock with neutropenia, clostridial septicemia, and right lower quadrant peritonitis when he was admitted. At the time of laparotomy, inflammation of the cecum, with no gross perforation, was found; no resection or appendectomy was done. He subsequently developed a right lower quadrant abscess that was drained, resulting in a colocutaneous fistula. For the next 8 months his fistula persisted, with intermittent episodes of fever, increased fistula output, and abdominal pain during his neutropenic periods. Standard nonoperative approaches to healing the fistula failed (ie, elemental feeds, total parenteral nutrition, irrigations, antibiotics, and drains). Attempts to medically abolish his neutropenic episodes using lithium, gammaglobulin, and steroids also failed. Ultimately, he underwent an ileocecal resection with primary anastamosis; the operation was done immediately following a neutropenic episode, in order to allow adequate healing of his anastamosis before his next period of neutropenia. Postoperative course was satisfactory, and he remains well after 8 months follow-up. This case, and several similar previously reported cases, illustrate that cyclic neutropenia may present with serious surgical complications. They also underlines the important role that neutrophils play in the healing of enteric fistulae.

Insertion of a small central venous catheter in neonates and young infants.

Total parenteral nutrition (TPN) administered through a central venous catheter in low-birthweight neonates and infants has been complicated by mechanical catheter malfunctions and catheter-associated infections. A retrospective survey of catheter complications 66 infants with 90 pediatric Broviac (1.3 mm o.d.) and large-diameter (French size 3, 4, and 5) Silastic catheters revealed 17 mechanical malfunctions (27%) and 16 cases (26%) of catheter infections. The current study presents our experience using 58 small-diameter (0.635 mm o.d.) Silastic catheters for TPN in 53 neonates and infants. There were 13 episodes (22%) of mechanical problems such as accidental dislodgement, occlusion of the catheter, and perforation of the tubing. Only four cases (7%) of catheter-associated sepsis occurred, a significant reduction (p = 0.008) in this serious problem compared to the previous large catheter study. We have compared clinical features of both large- and small-diameter catheters and suggest specific guidelines for their use. The small-diameter Silastic catheter is safe, easily inserted, and effective in the critically ill, low-birthweight neonate and in young infants weighing less than 6 kg. The pediatric Broviac catheter is recommended for administration of long-term or home TPN to infants and children greater than 6 kg. These catheters are useful for multiple purposes such as blood drawing, chemotherapy, and nutritional support while the small catheter is not as versatile.

Diagnosis and incidence of cardiac injury in children with blunt thoracic trauma.

Over a 20-month period, we prospectively studied 41 children with blunt thoracic trauma (BTT) to determine the incidence and morbidity of cardiac injury in this population. Four patients died, and the data were incomplete in 10, leaving 27 for analysis. Serial electrocardiograms (ECG) were abnormal in 36% of the patients studied, serial creatine phosphokinase isoenzyme (CK-MB) ratios were elevated in 31%, echocardiogram showed septal dysfunction in 14%, and pyrophosphate scan showed grade 1 uptake in 14%. There was poor correlation among tests, since only four patients had more than one abnormal test. There was no significant difference in Injury Severity Score or Thoracic Abbreviated Injury Scale between patients with and without abnormal investigations. No patient in the study developed arrhythmias or cardiac failure. In 13 trauma-related deaths undergoing autopsy over the same period, including the four with thoracic trauma, none had evidence of cardiac injury. These results indicate a striking lack of consistency in the diagnosis of posttraumatic cardiac injury in children using standard investigations. The absence of adverse cardiac events in surviving patients and the lack of autopsy evidence of cardiac injury in the trauma deaths suggest that the actual incidence and clinical significance of these lesions in children is lower than generally reported. Children with BTT should be followed clinically, and reliance on screening tests should be avoided.

Lipoid proteinosis: case report.

A 20-month-old Kuwaiti girl had manifestations of lipoid proteinosis, a rare autosomal recessive disorder seen more commonly in Caucasians. This condition is diagnosed based on clinical, histopathologic, and ultrastructural criteria. Its biochemical and genetic aspects are still poorly understood.

The new epidemic in children: penetrating injuries.

OBJECTIVE: It is our hypothesis that there has been a dramatic increase in penetrating injuries in children. The purpose of this study is to verify this "new" epidemic in children and to note some of its characteristics. METHODS: We performed a 7-year retrospective review of the trauma registry at our urban pediatric (< 16 years of age) trauma center (UPTC). RESULTS: The percentage of admissions due to penetrating injuries at our UPTC has gradually risen over the past 7 years primarily due to gunshot wounds. In 1992 and 1993, compared to 1987 and 1988, the incidence of penetrating injuries has increased from 20 to 36% in all children and from 45 to 66% in the 12-to-15-year-old age group. CONCLUSION: Injuries due to penetrating trauma have, indeed, increased to epidemic proportions.

Short-term pulmonary effects of total parenteral nutrition in children with cystic fibrosis.

Indices of respiratory muscle strength, pulmonary function, and pulmonary diffusing capacity were measured in 11 malnourished children (age 10 to 17 years) with cystic fibrosis, before and after improvement of nutritional status with supplemental parenteral nutrients for 1 month. During this time, the children received 120% of estimated energy requirements (either 3.75% or 22.5% as lipid) and amino acids 2.5 gm/120 kcal by central venous catheter, plus as much of their usual diet as desired. With nutritional supplementation, body weight, triceps skinfold thickness, and mid-arm muscle circumference increased (mean 15%, 62%, and 95%, respectively). Maximum inspiratory airway pressure also increased (mean 29%; P less than 0.01), suggesting improvement in respiratory muscle strength. However, none of the indices of pulmonary function improved. Pulmonary diffusing capacity did not change during parenteral nutrition regardless of the amount of parenteral energy intake supplied by lipid, but arterial oxygen saturation decreased (mean of 93.5% to 91.5%; P less than 0.005). During the month following parenteral nutrition, weight, skinfold thickness, and mid-arm muscle circumference, but not MIP, decreased and arterial oxygen saturation returned to the initial value (P less than 0.01).