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Biomedical subjects

M M Woolley

Publications and source records attributed to M M Woolley.

At least 19 recordsLinked to original sources

The death of a child--the parent's perspective and advice.

Recognizing the grief of parents after they have lost a child is an integral part of the practice of pediatric surgery. Parents who have experienced the loss of a child suggest the following: (1) The feelings associated with the loss of a child are not understandable unless you have lost a child of your own. (2) If you have not experienced the loss of your own child you should not state "I understand." You can't (3) The death of a child places stress on the marriage. (4) Many marriages do not survive. If the marriage does survive it becomes stronger than before the child's death. (5) During the recovery the parents see themselves as "different people." (6) Mothers and fathers react to the death differently. (7) Some parents have initiated new, beneficial projects that are in some way related to the child's death. (8) Parents react adversely to "pat" explanations and platitudes. (9) Parents want their friends and relatives to remain available. (10) The dead child should remain as an integral part of the family and no attempt should be made to "forget." (11) The self-help group that is referred to most frequently is "The Compassionate Friends, Inc.," with headquarters in Oak Brook, Illinois.

Attitude to Death↗

Tracheoesophageal fistula associated with perforated Meckel's diverticulum.

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.

Abnormalities, Multiple↗

Peripheral congenital arteriovenous fistulae: observe, operate, or obturate?

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.

Adolescent↗

Testicular feminization: the androgen insensitivity syndrome.

Testicular feminization (TF) is a syndrome due to androgen insensitivity. It occurs in a complete (CTF) and an incomplete (ITF) form. We have treated 21 patients with TF over the last 24 years. Eight patients presented because of ambiguous genitalia, seven presented as "females" with inguinal hernia and testes were found at surgery, five were diagnosed by karyotyping performed for a family history of TF, and one presented with an incarcerated hernia and primary amenorrhea. Two patients had prior surgery for inguinal hernia but the diagnosis was not recognized. All patients had a 46,XY karyotype. Patients with CTF were phenotypically female while those with ITF had a variable appearance of the external genitalia depending on the degree of androgen insensitivity. Seventeen patients underwent gonadectomy and one patient planned for delayed gonadectomy was lost to follow-up. Seventeen patients had been raised as females since birth. One patient with ambiguous genitalia, who was initially raised as a male, was reassigned female gender at 1 year of age when the diagnosis of ITF was made. Three patients were raised as males even after the diagnosis of ITF was made. Patients raised as males underwent multiple genital reconstructive procedures with poor results. In view of the poor anatomic and functional results of genital reconstructive surgery and the consequent psychological problems, patients with TF should be raised as female. Careful evaluation of infants with ambiguous genitalia and documentation of absent fallopian tubes in "females" presenting with inguinal hernia will lead to early diagnosis of TF, correct sex assignment, and early gonadectomy.

Adolescent↗

Indirect calorimetry in mechanically ventilated infants and children: measurement accuracy with absence of audible airleak.

OBJECTIVE: To establish the effect of an audible airleak (around an endotracheal tube) on oxygen consumption (VO2) measurements in pediatric ICU patients. DESIGN: Prospective trial comparing VO2 measurements before and after deflation of the endotracheal tube cuff. SETTING: Pediatric ICU in a large pediatric tertiary care center. PATIENTS: Twenty critically ill infants and children receiving mechanical ventilatory support via cuffed endotracheal tube. INTERVENTIONS: Deflation of endotracheal tube cuff. MEASUREMENTS AND MAIN RESULTS: The presence (group 1, n = 9) or absence (group 2, n = 11) of an audible airleak with the cuff deflated was confirmed by two independent observers. The percent difference in VO2 was calculated for both groups using the following formula: ([VO2 cuff up - VO2 cuff down]/VO2 cuff up) x 100. An audible airleak associated with cuff deflation (group 1) caused a significant (p = .0012) reduction of VO2 by 45.6% (mean difference in VO2 = 45.6%). In contrast, with no audible airleak after cuff deflation (group 2), only minimal changes in VO2 (mean difference in VO2 = -0.4%) were observed. CONCLUSIONS: These data suggest that if no audible airleak is detected, VO2 determined by indirect calorimetry may be reliably measured in infants and children with a noncuffed endotracheal tube.

Calorimetry, Indirect↗

Gastroschisis and intestinal atresia.

Controversy exists over the best method of treating gastroschisis with concomitant intestinal atresia because the mortality in such patients is still high. We present our experience of 74 neonates with gastroschisis seen over a 17-year period. Four patients (5.5%) had intestinal atresia; 1 had only one small bowel atresia, 2 had double areas of atresia (one in the mid small bowel and the other in the proximal colon), and the remaining 1 had multiple areas of atresia. Atresia with gastroschisis conventionally has been treated by either primary anastmosis or by exteriorization. Three of the patients were treated by replacing the atretic bowel into the abdomen initially and exploring later (1 to 3 weeks) when the bowel appeared much more healthy, making the anastomosis easier and more secure. One patient with extreme jejunal dilatation was treated by creation of a Mikulicz fistula. All 4 of the patients survived the newborn period, but one of them died at 1 year of age due to total parenteral nutrition-induced chronic liver failure. Our experience is presented to emphasize this uncommonly used approach to this complex problem because it seems to be associated with a lower morbidity and mortality than other conventional approaches.

Abdominal Muscles↗

Transanal mucosal sleeve resection for the treatment of rectal prolapse in children.

This is a report of a simple transanal operation performed on six patients (age range, 19 months to 18 years), who underwent unsuccessful nonoperative management of complete rectal prolapse for at least 1 month (range, 1 month to 13 years). All patients had normal sweat chloride levels, normal chest radiographs, and normal barium enemas. None of the patients were neurologically compromised. At the time of surgery, all but one patient had occurrence of reducible prolapse with minor straining or with every bowel movement. No severe mucosal ulcerations were present. Surgical therapy consisted of the transanal mucosal sleeve resection described herein. In this series, there were no anastomotic leaks, no clinically evident strictures and no recurrence of prolapse in 1.5- to 19-year follow-up. Surgical therapy for rectal prolapse in infants and children is rarely necessary. Various complicated or ineffective operations for the treatment of this condition have been recommended in the past. This technique offers a simple, safe, and effective method of treating complete, medically intractable rectal prolapse in children.

Adolescent↗

Pericardial flap aortopexy for tracheomalacia.

Tracheomalacia is frequently associated with esophageal atresia and vascular compression of the thoracic viscera. Suture aortopexy, as first described by Gross, has become the most commonly used surgical procedure for alleviating the symptoms of tracheomalacia. External stenting, implantation of cartilage graft, and tracheal resection have been tried in severe cases not amenable to aortopexy. A standard aortopexy was attempted in an infant who had undergone division of the posterior portion of a double aortic arch. Because of very tight posterior attachments of the mediastinal structures, the aortic arch could not be brought up to the sternum without undue tension. A 3 x 2 cm flap of pericardium was formed, based at the aortic root. The free end was then sutured to the undersurface of the sternum, thereby pulling the aorta toward the sternum under gentle, controlled tension. The patient was easily extubated and has remained symptom free. Pericardial flap aortopexy is a relatively simple procedure with minimal risk to the aorta or trachea. It may be the preferred initial procedure in the surgical management of tracheomalacia.

Aorta↗

Evolution in the management of splenic injury in children.

To assess the changes in the management of splenic trauma, the charts of all patients with splenic injury, documented either roentgenographically or at operation, treated at the Childrens Hospital of Los Angeles from 1965 through 1985 were reviewed. One hundred and eleven patients with confirmed splenic injury were treated. Forty-one patients were seen during the first decade (1965 to 1975) and 70 during the second decade (1976 to 1985). The majority of patients were boys with a mean age of seven years. All of the patients sustained blunt abdominal trauma. During the first decade, all of the patients who had a recognized splenic injury underwent splenectomy. In the second decade, 44 patients were treated without surgical intervention. Twenty-six of the patients required surgical treatment. Splenorrhaphy was successful in 15; however, splenectomy was required in 11 patients. The patients who required surgical therapy had more complications and required more blood products than those patients who were treated nonsurgically. The overall survival rate was 95 per cent and was similar in both decades. Associated injuries, not the splenic injury, were responsible for the lethal outcome of the nonsurvivors. Although the majority of children with splenic injury can be managed nonoperatively, surgical intervention continues to be necessary in our experience in approximately 38 per cent of the patients with an over-all splenic salvage rate of 85 per cent.

Abdominal Injuries↗

Pseudocysts of the pancreas in children.

Sixteen children with pancreatic pseudocysts were treated from 1965-1988. Blunt trauma was the etiology of pseudocyst formation in 69 per cent of children with 50 per cent resulting from the abdomen impacting bicycle handlebars. Chronic pancreatitis is an uncommon cause of pseudocyst formation in children. Medical therapy is directed towards reduction of pancreatic stimulation and nutritional support, which are maintained through pseudocyst resolution or maturation. Pseudocysts spontaneously resolved in 25 per cent of patients. Complications occurred in 25 per cent during nonoperative management. Children may safely undergo internal drainage earlier than adults (3-4 weeks vs 6 weeks). Internal drainage by cystoenterostomy was curative in eight patients. Persistent fistula drainage developed for five weeks in one patient who had surgical external pseudocyst drainage. One patient required distal pancreatectomy for a transected pancreatic duct. Spontaneous resolution of psseudocysts while on medical therapy is more frequent in children than in adults, and major complications (abscess formation, hemorrhage, and fistula formation) are usually not encountered. Pseudocyst rupture is the major complication of conservative management. We had no pseudocyst recurrences and 11 of 12 children treated surgically were discharged home within ten days of operation.

Abdominal Injuries↗

Use of magnetic resonance imaging in planning the separation of omphalopagus conjoined twins.

Magnetic resonance imaging (MRI) was used for the first time in the preoperative planning for separation of conjoined twins. In these omphalopagus infants, MRI showed normal biliary and cardiovascular structures and demonstrated, in detail, a relatively avascular plane through the liver bridge, which enabled safe separation with minimal blood loss at 3 1/2 months of age. A single MRI study supplied information superior to that obtained with multiple previously available imaging studies. MRI should be an essential part of the preoperative workup of all types of conjoined twins.

Abdomen↗

Required surgical therapy in the pediatric patient with dermatomyositis.

A review of 96 patients with the established diagnosis of childhood dermatomyositis revealed that 15 patients required surgical therapy in addition to diagnostic biopsy. Four of the patients had perforation of the esophagointestinal tract, and 11 required surgical therapy for abscesses, calcific deposits, or treatment of pneumothorax. The life-threatening lesions were perforations of the esophagus and intestine. The best surgical therapy is closure, resection, and adequate drainage. Prognosis depends on treatment of the underlying medical disease.

Abscess↗

Measured energy expenditure in critically ill infants and young children.

Technological limitations have impeded accurate energy expenditure assessment in critically ill infants and young children. Instead, a predicted energy expenditure (PEE) is derived based on weight, heat loss, activity, growth requirements, and degree of stress. This study compared actual measured energy expenditure (MEE) with conventional predicted values in 20 critically ill infants and children using a validated metabolic cart designed for use in this age group. All patients were studied either within 4 days of major surgery or during an acute disease process necessitating intensive care. All were severely stressed clinically and were studied while mechanically ventilated in a temperature-controlled environment. The study interval ranged from 1 to 12 hr and averaged 4 hr after a stabilization period of 30 min. The mean MEE was significantly lower than the mean PEE (52.2 +/- 16 kcal/kg/day vs 101.8 +/- 17 kcal/kg/day, P less than 0.001) with a mean MEE/PEE of 52.6 +/- 17% (range 26 to 92%). In a subgroup of 7 paralyzed patients, the mean MEE was significantly lower than in the 13 nonparalyzed patients when compared with PEE and predicted basal metabolic rate (PBMR). The coefficient of variance, conventionally recognized to be approximately 15% for PEE, averaged 6.35% for MEE in this study. These data indicate that if PEE is used as the sole guide for caloric repletion in the stressed infant or child, these patients will be substantially overfed.

Basal Metabolism↗

Postoperative functional and manometric evaluation of patients with Hirschsprung's disease.

Sixty-two patients with Hirschsprung's disease (operated on according to Duhamel, Swenson, or Soave) were evaluated for quality of defecation and studied manometrically. Follow-up was 30 years. Stools were normal in 30% to 50% of patients, while the rest had either constipation, loose, or increased frequency of stools per day. Continence was complete in 30% to 60%. Enterocolitis was seen in 12 patients preoperatively and in 20 patients postoperatively, regardless of type of operative procedure. This complication improved with age. To correct some of these problems, 18 had post pull-through internal sphincter myectomy with 50% improvement. Manometrically, 50% to 70% experienced a sensation of fullness and an urge to defecate following rectal balloon inflation. The resting external sphincter pressure was high in the majority of patients and further increased following rectal balloon inflation. The internal sphincter pressure showed a normal anorectal reflex in only 10% of patients postoperatively and did not seem to be related to clinical fecal continence. We concluded from the study that an appreciable number of patients in this select group with Hirschsprung's disease suffered from a variety of stooling disorders postoperatively. The majority of these complications were managed medically and/or socially but some were quite incapacitating.

Anal Canal↗

Hydrops of the gallbladder associated with Kawasaki syndrome.

Hydrops of the gallbladder is recognized as a major component of the abdominal crisis occurring in children with Kawasaki syndrome. Sixteen patients with hydrops of the gallbladder secondary to Kawasaki syndrome have been diagnosed and treated at the Childrens Hospital of Los Angeles. One patient was treated by cholecystectomy and 15 nonoperatively without untoward sequelae. Nonoperative management with serial ultrasonic evaluation and close clinical monitoring is a safe method of treatment for this entity. Pathologic and clinical data are presented and discussed. Review of diagnosis and treatment of 41 reported cases of hydrops of the gallbladder in Kawasaki syndrome from the English language literature is also presented.

Child↗

Esophageal atresia and tracheoesophageal fistula in the twin. Anatomic variants.

Recent experience with a twin who had esophageal atresia and tracheoesophageal fistula revealed complex anatomy not suspected before operation. The experience in patients with esophageal atresia and tracheoesophageal fistula who were twins was reviewed at the Children's Hospital of Los Angeles. Of 245 patients seen in the past 23 years, 16 were twins (only two of whom were siblings). Six of the 16 patients (38%) had other than the most frequent anatomy, i.e., proximal atresia of the esophagus and distal fistula. Two of these patients had extremely complex anatomy. Twenty-five per cent of the patients had a right aortic arch compared with 5% of all patients with tracheoesophageal fistula. The surgeon who encounters a twin with esophageal atresia or tracheoesophageal fistula is appropriately cautioned that significant anatomic complexity may be encountered.

Abnormalities, Multiple↗