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Oligohydramnios syndrome and intra-abdominal pregnancy
15 8
Letters to the Editor
The Journal of Pediatrics January 1977
pectus carinatum deformity; and a wide anterior fontanelle. The patient's mother and an older female sibling have apparent hypertelorism. The cleft was surgically repaired. At age five months, the infant was doing well r the aid of a tracheostomy. Chromosome studies were reported as normal. COMMENT
Fig 1. Lateral film of chest following barium esophagram through a feeding tube. Note barium outlining esophagus, trachea, and common esophagotrachea (arrows).
the occurrence of these entities together has not been reported. Varying in severity from a small posterior cleft to gross tracheoesophageal communication, laryngotracheoesophageal cleft is due to arrest of rostral advancement of the tracheoesophageal septum preventing fusion of the cricoid cartilages. Because aspiration can be massive and lethal, early detection is essential. The defect may be detected by laryngoscopy and esophagram, although the first recorded case was diagnosed by palpation with the index finger. CASE REPORT
Patient B. W. was born at term by a normal vaginal delivery. Apgar score at one minute was 8, and five minutes 9. Birth weight was 2,670 gin. He required suctioning. Hypertelorism was present. Interpupillary distance measured 4.7 cm (greater than the ninty-seventh percentile for age). Hypospadias with chordee were also noted at birth. One hour after birth, the baby was noted to have noisy respirations and a large volume of mucus that required frequent suctioning. At the first feeding, the baby aspirated and became cyanotic. He was tachypneic and had retractions. Chest roentgenogram showed left lower lobe pneumonia. A nasogastric tube was passed into the stomach. Antibiotic therapy was instituted for the treatment of aspiration pneumonia. "H" type tracheoesophageal fistula was suspected. Barium esophagram demonstrated a large communication between the trachea and esophagus at the cricoid level (Fig 1). A laryngotracheoesophageal cleft was seen by direct laryngoscopy. Other anomalies noted were minor notching of the upper lip in the midline; a nonfunctioning, probably absent, left kidney by excretory urography; bony anomaly of the lower sternum with
In the diagnostic appraisal of an infant suspected of having laryngotracheoesophageal cleft, Felman and Talbert '~ recommended laryngoscopy and insertion of an endotracheal tube prior to barium studies through a nasogastric tube. The endotracheal tube facilitates suctioning of the trachea and assisted ventilation, if necessary. The possibility of laryngotracheoesophageal cleft should be kept in mind in a newborn infant with hypertelorism-hypospadias syndrome, and the first feeding should be given with caution. If feeding difficulty is encountered, the child should be studied as described. Peter R. Miller, M.D. Resident in Radiology Rochester General Hospital Richard M. Bernstein, M.D. Department of Radiology Rochester General Hospital 1425 Portland Ave. Rochester, N Y 14621 Ambadas Pathak, M.D. Assistant Professor of Pediatrics University of Rochester School of Medicine and Dentistry and Chief Section of Neonatology Rochester General Hospital H. George Decancq, Jr., M.D. Clinical Instructor in Pediatrics University of Rochester School of Medicine and Dentistry REFERENCES
1. Christian JC: Hypertelorism-hypospadias syndrome, in Bergsma D, editor: Birth defects: Atlas and compendium, National Foundation-March of Dimes, New York, 1973, The Williams & Wilkins Company, p 509. 2. Burroughs N, and Leape LL: Laryngotracheoesophageal cleft: Report of a case successfully treated and review of the literature, Pediatrics 53:516, 1974. 3. Felman AH, and Talbert JL: Laryngotracheoesophageal cleft: Description of a combined laryngoscopic and roentgenographic diagnostic technique and report of two patients, Radiology 103:641, 1972.
Oligohydramnios syndrome and intra-abdominal pregnancy To the Editor: The oligohydramnios syndrome 1 -' may be secondary to fetal anuria 1 or chronic amniotic fluid leakage.:' Oligohydramnios
Volume 90 Number 1
Letters to the Editor
15 9
Fig. 1. Photomicrograph of the lung with a line drawn from a terminal bronchiole perpendicular to the pleural surface demonstrating paucity of alveoli (H & E; • 64).
commonly accompanies intra-abdominal pregnancy, and an infant with the oligohydramnios syndrome following intraabdominal pregnancy is described. CASE REPORT The infant was delivered by laparotomy performed because of abdominal pain and concern for the fetus after hysterography had confirmed an intra-abdominal pregnancy. Amniotic fluid was absent. Apgar scores were 2 (1 minute) and 5 (5 minutes): the infant required assisted ventilation. The chest was small, retractions slight, breath sounds poorly heard, and tachypnea of 70/ minute was maintained independently of the Baby Bird ventilator. Clinical gestational age was 32 weeks, weight 1,220 gm (tenth percentile), length 39 cm (tenth to twenty-fifth percentile) and FOC 26.0 cm (
surfaces and lobular septa. The mean radial alveolar count of 20 measurements was 3.2 (normal 6.2) (Fig. 1). Hyaline membranes lined, and recent hemorrhage filled, several air spaces. The membrane adhering to the skin was amnion with island clusters of desquamated epithelial cells and fibrin characteristic of amnion nodosum. The kidneys and urinary tracts were normal. DISCUSSION The syndrome of bilateral pulmonary hypoplasia, limb malposition, altered facies, and intrauterine growth retardation, as observed in this infant, has been attributed to prolonged oligohydramnios. Here oligohydramnios was due to intra-abdominal pregnancy, in which compression of head and limbs is recognized. Hypothetically, compression could also impair lung growth directly, paralleling the pulmonary hypoplasia accompanying the rigid chest wall in Jeune syndrome, or indirectly by neck flexion and micrognathia rendering the laryngeal sphincter incompetent with facilitated outflow of lung fluidr Intrauterine growth retardation may result from limited placental growth spread and absent ingestion of protein-containing amniotic fluid (normally 0.2-0.3 gm protein/kg/day). John Williams, M.B.B.Chir., M.R.C.P. Lawrence P. Gordon, M.D. Harvey S. Rosenberg, M.D. Arnold J. Rudolph, M.D. Departments of Pediatrics and Pathology Texas Children's Hospital and Baylor College of Medicine Houston, Texas 77030
16 0
Letters to the Editor
The Journal of Pediatrics Januar)~ 1977
REFERENCES
I.
Perlman M, and Levin M: Fetal pulmonary hypoplasia, anuria, and oligohydramnios: clinicopathologic observations and review of the literature, Am J Obstet Gynecol 118:1119, 1974. 2. Thomas IT, and Smith DW: Oligohydramnios, cause of the non-renal features of Potter's syndrome, including pulmonary hypoplasia, J PED]Aa-R84:811, 1974. 3. Perlman M, Williams J, and Hirsch M: Neonatal pulmonary hypoplasia after prolonged leakage of amniotic fluid, Arch Dis Child 51:359, 1976. 4. Adams FH, Desilets DT, and Towers 13: Control of flow of fetal lung fluid at the laryngeal outlet, Respir Physiol 2:302, 1967.
Relationship between weight gain and serum level of alkaline phosphatase in malnourished infants treated by total parenteral nutrition To the Editor: In the last decade total parenteral nutrition (TPN) has been largely used in the treatment of severe nutritional failure in infancy. During TPN, the growth rates of infants with proteincalorie malnutrition (PCM) are high when compared to normal infants.' In the absence of clinical signs of water retention, weight gain is considered a reliable index of growth. ~In the first weeks of treatment, however, the increase of the body weight is often poor or completely absent, despite positive nitrogen balance and availability of adequate calories. ~By water retention, on the other hand, which is a complication of TPN, the weight gain may simulate nonexistent growth. In order to evaluate, by simple biochemical tests, the changes in the nutritional status of malnourished infants during TPN, we studied the behavior of some biochemical values in 27 infants aged four to 24 weeks, who had intractable diarrhea and severe malnutrition and were successfully treated with TPN. The technique of parenteral nutrition as described by Filler and Eraklis 3was used. The average duration of treatment was 42 days (18 to 120 days); the average weight gain was 930 gm (450-2,250 gm). During TPN all infants remained in good fluid and electrolyte balance. We determined, at the beginning of TPN and once weekly thereafter, the serum concentrations of total protein, urea nitrogen (BUN) and alkaline phosphatase; these are characteristically decreased in PCM. 4- ~ Blood samples were obtained before the infusion of plasma, which was given in an amount of 20 ml/kg once a week. No statistically significant differences were found in the serum levels of protein, albumin, and BUN determined at the beginning of TPN and one and two weeks later. The alkaline phosphatase, on the contrary, increased remarkably from 47 _+ 6 IU/1 to 85 _+ 9 (p < 0.01) after one week and to 115 _+ 8 IU/I after two weeks of treatment. Fig. 1
3
~.
25
o;;,~
4
5
6
7
8
9
10 11 12 13 14 15
WEEKS OF TREATMENT
Fig. 1. Relationship between weight gain and serum level of alkaline phosphatase in a four-month-old infant treated by total parenteral feeding for malnutrition, intractable diarrhea and cow milk and soy milk intolerance.
shows the relationship between weight gain and serum level of alkaline phosphatase in a four-month-old infant treated by TPN for malnutrition, intractable diarrhea, and cow milk and soy milk intolerance, During total parenteral feeding, body weight as well as the serum alkaline phosphatase concentration increased. When oral feeding with cow milk and a soy milk formula (Isomil) was instituted, diarrhea appeared and a progressive fall in weight occurred with a corresponding decrease in the serum level of alkaline phosphatase. Slight changes of weight and of alkaline phosphatase were observed when the oral feeding with a meatbased formula was tolerated and TPN was discontinued. During TPN, vitamin D was given in physiologic amounts to all infants. Clinical, biochemical, or radiologic signs of rickets were never observed. COMMENT Marked alterations of some biochemical measurements, such as those of serum protein, albumin, BUN, and alkaline phosphatase have been described in PCM. During TPN, malnourished infants show rapid growth and increase of the above mentioned biochemical concentrations in serum. However, only the increase of the alkaline phosphatase reflects the rapid changes in the nutritional status; it can be used as indirect evidence of the efficiency of the parenteral hyperalimentation. Domenico Della Pietra, M.D. Antonio Correra, M.D. Francesco Tancredi, M.D. Divisione Scientifica di Pediatria E.O. SS Annunziala Napoli via Annunziata 34 Napoli, Italia
Letters to the Editor
The Journal of Pediatrics January 1977
pectus carinatum deformity; and a wide anterior fontanelle. The patient's mother and an older female sibling have apparent hypertelorism. The cleft was surgically repaired. At age five months, the infant was doing well r the aid of a tracheostomy. Chromosome studies were reported as normal. COMMENT
Fig 1. Lateral film of chest following barium esophagram through a feeding tube. Note barium outlining esophagus, trachea, and common esophagotrachea (arrows).
the occurrence of these entities together has not been reported. Varying in severity from a small posterior cleft to gross tracheoesophageal communication, laryngotracheoesophageal cleft is due to arrest of rostral advancement of the tracheoesophageal septum preventing fusion of the cricoid cartilages. Because aspiration can be massive and lethal, early detection is essential. The defect may be detected by laryngoscopy and esophagram, although the first recorded case was diagnosed by palpation with the index finger. CASE REPORT
Patient B. W. was born at term by a normal vaginal delivery. Apgar score at one minute was 8, and five minutes 9. Birth weight was 2,670 gin. He required suctioning. Hypertelorism was present. Interpupillary distance measured 4.7 cm (greater than the ninty-seventh percentile for age). Hypospadias with chordee were also noted at birth. One hour after birth, the baby was noted to have noisy respirations and a large volume of mucus that required frequent suctioning. At the first feeding, the baby aspirated and became cyanotic. He was tachypneic and had retractions. Chest roentgenogram showed left lower lobe pneumonia. A nasogastric tube was passed into the stomach. Antibiotic therapy was instituted for the treatment of aspiration pneumonia. "H" type tracheoesophageal fistula was suspected. Barium esophagram demonstrated a large communication between the trachea and esophagus at the cricoid level (Fig 1). A laryngotracheoesophageal cleft was seen by direct laryngoscopy. Other anomalies noted were minor notching of the upper lip in the midline; a nonfunctioning, probably absent, left kidney by excretory urography; bony anomaly of the lower sternum with
In the diagnostic appraisal of an infant suspected of having laryngotracheoesophageal cleft, Felman and Talbert '~ recommended laryngoscopy and insertion of an endotracheal tube prior to barium studies through a nasogastric tube. The endotracheal tube facilitates suctioning of the trachea and assisted ventilation, if necessary. The possibility of laryngotracheoesophageal cleft should be kept in mind in a newborn infant with hypertelorism-hypospadias syndrome, and the first feeding should be given with caution. If feeding difficulty is encountered, the child should be studied as described. Peter R. Miller, M.D. Resident in Radiology Rochester General Hospital Richard M. Bernstein, M.D. Department of Radiology Rochester General Hospital 1425 Portland Ave. Rochester, N Y 14621 Ambadas Pathak, M.D. Assistant Professor of Pediatrics University of Rochester School of Medicine and Dentistry and Chief Section of Neonatology Rochester General Hospital H. George Decancq, Jr., M.D. Clinical Instructor in Pediatrics University of Rochester School of Medicine and Dentistry REFERENCES
1. Christian JC: Hypertelorism-hypospadias syndrome, in Bergsma D, editor: Birth defects: Atlas and compendium, National Foundation-March of Dimes, New York, 1973, The Williams & Wilkins Company, p 509. 2. Burroughs N, and Leape LL: Laryngotracheoesophageal cleft: Report of a case successfully treated and review of the literature, Pediatrics 53:516, 1974. 3. Felman AH, and Talbert JL: Laryngotracheoesophageal cleft: Description of a combined laryngoscopic and roentgenographic diagnostic technique and report of two patients, Radiology 103:641, 1972.
Oligohydramnios syndrome and intra-abdominal pregnancy To the Editor: The oligohydramnios syndrome 1 -' may be secondary to fetal anuria 1 or chronic amniotic fluid leakage.:' Oligohydramnios
Volume 90 Number 1
Letters to the Editor
15 9
Fig. 1. Photomicrograph of the lung with a line drawn from a terminal bronchiole perpendicular to the pleural surface demonstrating paucity of alveoli (H & E; • 64).
commonly accompanies intra-abdominal pregnancy, and an infant with the oligohydramnios syndrome following intraabdominal pregnancy is described. CASE REPORT The infant was delivered by laparotomy performed because of abdominal pain and concern for the fetus after hysterography had confirmed an intra-abdominal pregnancy. Amniotic fluid was absent. Apgar scores were 2 (1 minute) and 5 (5 minutes): the infant required assisted ventilation. The chest was small, retractions slight, breath sounds poorly heard, and tachypnea of 70/ minute was maintained independently of the Baby Bird ventilator. Clinical gestational age was 32 weeks, weight 1,220 gm (tenth percentile), length 39 cm (tenth to twenty-fifth percentile) and FOC 26.0 cm (
surfaces and lobular septa. The mean radial alveolar count of 20 measurements was 3.2 (normal 6.2) (Fig. 1). Hyaline membranes lined, and recent hemorrhage filled, several air spaces. The membrane adhering to the skin was amnion with island clusters of desquamated epithelial cells and fibrin characteristic of amnion nodosum. The kidneys and urinary tracts were normal. DISCUSSION The syndrome of bilateral pulmonary hypoplasia, limb malposition, altered facies, and intrauterine growth retardation, as observed in this infant, has been attributed to prolonged oligohydramnios. Here oligohydramnios was due to intra-abdominal pregnancy, in which compression of head and limbs is recognized. Hypothetically, compression could also impair lung growth directly, paralleling the pulmonary hypoplasia accompanying the rigid chest wall in Jeune syndrome, or indirectly by neck flexion and micrognathia rendering the laryngeal sphincter incompetent with facilitated outflow of lung fluidr Intrauterine growth retardation may result from limited placental growth spread and absent ingestion of protein-containing amniotic fluid (normally 0.2-0.3 gm protein/kg/day). John Williams, M.B.B.Chir., M.R.C.P. Lawrence P. Gordon, M.D. Harvey S. Rosenberg, M.D. Arnold J. Rudolph, M.D. Departments of Pediatrics and Pathology Texas Children's Hospital and Baylor College of Medicine Houston, Texas 77030
16 0
Letters to the Editor
The Journal of Pediatrics Januar)~ 1977
REFERENCES
I.
Perlman M, and Levin M: Fetal pulmonary hypoplasia, anuria, and oligohydramnios: clinicopathologic observations and review of the literature, Am J Obstet Gynecol 118:1119, 1974. 2. Thomas IT, and Smith DW: Oligohydramnios, cause of the non-renal features of Potter's syndrome, including pulmonary hypoplasia, J PED]Aa-R84:811, 1974. 3. Perlman M, Williams J, and Hirsch M: Neonatal pulmonary hypoplasia after prolonged leakage of amniotic fluid, Arch Dis Child 51:359, 1976. 4. Adams FH, Desilets DT, and Towers 13: Control of flow of fetal lung fluid at the laryngeal outlet, Respir Physiol 2:302, 1967.
Relationship between weight gain and serum level of alkaline phosphatase in malnourished infants treated by total parenteral nutrition To the Editor: In the last decade total parenteral nutrition (TPN) has been largely used in the treatment of severe nutritional failure in infancy. During TPN, the growth rates of infants with proteincalorie malnutrition (PCM) are high when compared to normal infants.' In the absence of clinical signs of water retention, weight gain is considered a reliable index of growth. ~In the first weeks of treatment, however, the increase of the body weight is often poor or completely absent, despite positive nitrogen balance and availability of adequate calories. ~By water retention, on the other hand, which is a complication of TPN, the weight gain may simulate nonexistent growth. In order to evaluate, by simple biochemical tests, the changes in the nutritional status of malnourished infants during TPN, we studied the behavior of some biochemical values in 27 infants aged four to 24 weeks, who had intractable diarrhea and severe malnutrition and were successfully treated with TPN. The technique of parenteral nutrition as described by Filler and Eraklis 3was used. The average duration of treatment was 42 days (18 to 120 days); the average weight gain was 930 gm (450-2,250 gm). During TPN all infants remained in good fluid and electrolyte balance. We determined, at the beginning of TPN and once weekly thereafter, the serum concentrations of total protein, urea nitrogen (BUN) and alkaline phosphatase; these are characteristically decreased in PCM. 4- ~ Blood samples were obtained before the infusion of plasma, which was given in an amount of 20 ml/kg once a week. No statistically significant differences were found in the serum levels of protein, albumin, and BUN determined at the beginning of TPN and one and two weeks later. The alkaline phosphatase, on the contrary, increased remarkably from 47 _+ 6 IU/1 to 85 _+ 9 (p < 0.01) after one week and to 115 _+ 8 IU/I after two weeks of treatment. Fig. 1
3
~.
25
o;;,~
4
5
6
7
8
9
10 11 12 13 14 15
WEEKS OF TREATMENT
Fig. 1. Relationship between weight gain and serum level of alkaline phosphatase in a four-month-old infant treated by total parenteral feeding for malnutrition, intractable diarrhea and cow milk and soy milk intolerance.
shows the relationship between weight gain and serum level of alkaline phosphatase in a four-month-old infant treated by TPN for malnutrition, intractable diarrhea, and cow milk and soy milk intolerance, During total parenteral feeding, body weight as well as the serum alkaline phosphatase concentration increased. When oral feeding with cow milk and a soy milk formula (Isomil) was instituted, diarrhea appeared and a progressive fall in weight occurred with a corresponding decrease in the serum level of alkaline phosphatase. Slight changes of weight and of alkaline phosphatase were observed when the oral feeding with a meatbased formula was tolerated and TPN was discontinued. During TPN, vitamin D was given in physiologic amounts to all infants. Clinical, biochemical, or radiologic signs of rickets were never observed. COMMENT Marked alterations of some biochemical measurements, such as those of serum protein, albumin, BUN, and alkaline phosphatase have been described in PCM. During TPN, malnourished infants show rapid growth and increase of the above mentioned biochemical concentrations in serum. However, only the increase of the alkaline phosphatase reflects the rapid changes in the nutritional status; it can be used as indirect evidence of the efficiency of the parenteral hyperalimentation. Domenico Della Pietra, M.D. Antonio Correra, M.D. Francesco Tancredi, M.D. Divisione Scientifica di Pediatria E.O. SS Annunziala Napoli via Annunziata 34 Napoli, Italia