Volume 88 Number 3
Letters to the Editor
52 1
Table I. Results of T- a n d B-cell studies o f the p a t i e n t d u r i n g t h e acute phase (15 m o n t h s o f age) a n d after the recovery (25 m o n t h s o f age); m e a n values a n d s t a n d a r d deviations (SD) o f 13 n o r m a l individuals are also included
Age of patient
(mo)
15 25 Normal values Mean _+
% Stained cells with antisera against
Peripheral lymphocyte counts (lOS~ram~) 59.3 4.9
46 61
2 17
2 6
0 13
2 8
0 7
0 1
2 12
2 7
2.5 0.8
60 8
13 6
6 3
5 3
7 6
4 2
1 1
8 5
5 2
REFERENCES
1. Wintrobe MM, editor: Clinical hematology, 7 ed, Philadelphia, 1974, Lea & Febiger, p 1289. 2. Hsu CCS, Marti GE, Schrek R, and Williams RC Jr: Lymphocyte bearing B- and T-cell markers in patient with Iymphosarcoma cell leukemia, Ctin Immunol Immunopathol 3:385, 1975. 3. Pattengale PK, Smith RW, Perlin E: Atypical lymphoeytes in acute infectious mononucleosis, N Engl J Med 291:1145, 1974. 4. Wernet P, Siegal FP, Dickler H, Bentwich Z, Fu S, arid Kunkel HG: B and T cells in various disease states: Immature B cells in the blood of certain patients with immune deficiency, Ado Biosci 12:169, 1974. 5. Scheinberg MA, Cathcart ES, and Goldstein AL: Thymosin-induced reduction of "null cells" in peripheral blood lymphocytes of patients with systemic lupus erythematosus, Lancet 1:424, 1975. 6. Jondal M. Wigzeil H, and Aiuti F: Human lymphocyte subpopulations: classification according to surface markers and/or functional characteristics, Transpl Rev 16:163, 1973.
On admission, inspiration was stridulous and prolonged, but expiration was even more affected and characterized by wheezhag. The infant had two respiratory arrests, which responded to intubation. Radiographs of the lateral neck demonstrated no abnormality. An esophagram obtained to exclude tracheal compression by a vascular ring revealed an intrinsic mass of the intrathoracic trachea almost totally obliterating the tracheal air shadow (Fig. 1). The esophagus was normal. Bronchoscopy was then performed; a cyst was seen approximately 2 cm below the glottis. The cyst was ruptured during the procedure, and cloudy fluid was aspirated. The infant was later extubated without recurrence of respiratory distress. Repeat bronchoscopies four days and six weeks later, respectively, revealed no reaccumulation of fluid; the margins of the old cyst were open. DISCUSSION Airway obstruction in infants characterized predominantly by inspiratory stridor is usually indicative of an extrathoracic lesion
Intraluminal tracheal cyst producing airway obstruction in the newborn infant To the Editor: Laryngeal cysts in the newborn infant have been reported as a cause of airway obstructionr but no reports of intraluminal cysts of the trachea could be found upon review of the literature. CASE REPORT
Patient D. L., a male infant, was born after an uncomplicated pregnancy. Delivery was spontaneous, but the amnionic fluid was meconium stained. Respiratory distress with inspiratory stridor and prolonged expiration was noted immediately after delivery. The infant became hypoxemic and hypercapneie and was initially treated for meconium aspiration. A subsequent bronchoscopy was reported as negative. The infant was then referred to the Arizona Medical Center.
Fig. 1. This view of the esophagram demonstrates the smgoth, rounded contour of the intrathoracic tracheal mass. Note tha'~ the tracheal air shadow is nearly completely obliterated by the intrinsic mass. The trachea is not shifted. The esophagus is normal.
522
Letters to the Editor
involving the larynx or upper trachea. When obstruction is more severe during expiration, the lesion is usually intrathoracicY The lesion in our patient was in the intrathoracic trachea, and the more severe involvement of expiration is consistent with that location. Appropriate radiographic studies are essential in the evaluation of infants with airway obstruction. Lateral neck radiographs should always be obtained; in the case presented, however, it was the esophagram which demonstrated the lesion. This finding stresses the need for doing barium esophagrams in the evaluation of infants with airway obstruction, not only to demonstrate vascular rings, but also to detect intra-airway lesions. The etiology of the cyst is unclear. A comparison with laryngeal cysts may be appropriate since the lower larynx and the trachea have a common embryologic origin.:' Both have ciliated epithelium with mucous glands. Laryngeal mucous cysts result from obstruction of mucous gland ducts; the tracheal cyst presented may have developed in this fashion. Susan E. Denson, M.D. Lynn M. Taussig, M.D. Neonatology and Pulmonary Divisions Department of Pediatrics Gerald D. Pond, M.D. "Department of Pediatrics Department of Radiology Arizona Medical Center Tucson, Ariz. 85724 REFERENCES
1. Suehs OW, and Powell DB: Congenital cysts of the larynx in infants, Laryngoscope 77"654, 1967. 2. Sackner MA: Physiologic features of upper airway obstruction, Chest 62:414, 1972. 3. Langman J: Medical embryology, ed 2, Baltimore, 1969, The Williams & Wilkins Company.
The Chediak-Higashi syndrome in a Negro infant To the Editor: Since the first patient with the Chediak-Higashi syndrome was described by Bequez-Cesar in 1943,1 approximately 70 cases of the disorder have been reported. We wish to describe the occurrence of the Chediak-Higashi syndrome in a black infant who presented with some features of the accelerated phase of the disease. CASE REPORT
A black male infant was discharged from hospital at 5 days of age with a diagnosis of "albinism." At 2 months of age he was admitted to Rainbow Babies and Childrens Hospital because of fever and irritability. Laboratory data included a hematocrit value of 17%, hemoglobin concentration of 6.1 gm)dl, and white
The Journal of Pediatrics March 1976
blood cell count 3500/mm ~, with 6% neutrophils and 94% lymphocytes. Red blood cell morphology was normal. Platelets numbered 175,000/mm ~. The result of the nitroblue tetrazolium dye test was positive. A direct Coombs test was strongly positive, although no specific incompatibilities of blood group or type were identified. The diagnosis of Chediak-Higashi syndrome was confirmed by the presence of the typical cytoplasmic inclusions in the granulocytes on peripheral blood smear. Shortly after admission the patiem developed thrombocytopenia and prolonged prothrombin and partial thromboplastin times. However, no increase in circulating fibrin degradation products was detected. The infant was transfused with fresh whole blood, and chemotherapy with vincristine and prednisone was instituted. Response to this therapy was dramatic. Platelet count and other measurements of clotting function returned to normal within the next week, and the hematocrit rose following a significant reticulocystosis. The NBT test became negative and was never again positive. Immune globulins were repeatedly within or above the normal ranges for the infant's age during his subsequent course. Recurrent bacterial infections were not a prominent feature of this patient's hospital course. Seven months after his initial admission, the infant again developed a recurrence of the hemorrhagic diathesis with the thrombocytopenia and a Coombs-positive hemolytic anemia. Vincristine, prednisone, and antibiotic therapy were reinstituted, but the infant died. A complete description of autopsy findings will be published elsewhere. DISCUSSION In view of the chronic neutropenia and delayed killing of bacteria by neutrophils in this syndrome, it is somewhat surprising that infections did not appear to play a major role in the course of this infant's disease. He maintained normal or elevated immune globulin levels throughout his illness. The NBT test, initially positive, remained negative throughout the patient's hospital course. The Chediak-Higashi syndrome has been reported to be associated with a false positive NBT testy Cytotoxic therapy has been used in the "accelerated" phase of the syndrome, ~and our experience suggests that this mode of therapy may be beneficial in inducing prompt (if temporary) remission. The age of onset of the "accelerated" phase of the disease in this patient and the near fatal outcome during the first episode indicate that the disease may be terminal at an extremely early age and consequently may not be diagnosed, especially in Caucasians, prior to death. Sean O'Regan, M.B., B.Ch. Department of Nephrology Montreal Children's Hospital 2300 Tupper St. Montreal, P. Q., Canada H3H 1P3 Arthur J. Newman, M.A., M.D. Rafael Valenzuela, M.D. Sudesh P. Makker, M.D. Richard C. Graham, M.D. Department of Pediatrics & Medicine Case Western University School of Medicine Cleveland, Ohio 44106
Letters to the Editor
52 1
Table I. Results of T- a n d B-cell studies o f the p a t i e n t d u r i n g t h e acute phase (15 m o n t h s o f age) a n d after the recovery (25 m o n t h s o f age); m e a n values a n d s t a n d a r d deviations (SD) o f 13 n o r m a l individuals are also included
Age of patient
(mo)
15 25 Normal values Mean _+
% Stained cells with antisera against
Peripheral lymphocyte counts (lOS~ram~) 59.3 4.9
46 61
2 17
2 6
0 13
2 8
0 7
0 1
2 12
2 7
2.5 0.8
60 8
13 6
6 3
5 3
7 6
4 2
1 1
8 5
5 2
REFERENCES
1. Wintrobe MM, editor: Clinical hematology, 7 ed, Philadelphia, 1974, Lea & Febiger, p 1289. 2. Hsu CCS, Marti GE, Schrek R, and Williams RC Jr: Lymphocyte bearing B- and T-cell markers in patient with Iymphosarcoma cell leukemia, Ctin Immunol Immunopathol 3:385, 1975. 3. Pattengale PK, Smith RW, Perlin E: Atypical lymphoeytes in acute infectious mononucleosis, N Engl J Med 291:1145, 1974. 4. Wernet P, Siegal FP, Dickler H, Bentwich Z, Fu S, arid Kunkel HG: B and T cells in various disease states: Immature B cells in the blood of certain patients with immune deficiency, Ado Biosci 12:169, 1974. 5. Scheinberg MA, Cathcart ES, and Goldstein AL: Thymosin-induced reduction of "null cells" in peripheral blood lymphocytes of patients with systemic lupus erythematosus, Lancet 1:424, 1975. 6. Jondal M. Wigzeil H, and Aiuti F: Human lymphocyte subpopulations: classification according to surface markers and/or functional characteristics, Transpl Rev 16:163, 1973.
On admission, inspiration was stridulous and prolonged, but expiration was even more affected and characterized by wheezhag. The infant had two respiratory arrests, which responded to intubation. Radiographs of the lateral neck demonstrated no abnormality. An esophagram obtained to exclude tracheal compression by a vascular ring revealed an intrinsic mass of the intrathoracic trachea almost totally obliterating the tracheal air shadow (Fig. 1). The esophagus was normal. Bronchoscopy was then performed; a cyst was seen approximately 2 cm below the glottis. The cyst was ruptured during the procedure, and cloudy fluid was aspirated. The infant was later extubated without recurrence of respiratory distress. Repeat bronchoscopies four days and six weeks later, respectively, revealed no reaccumulation of fluid; the margins of the old cyst were open. DISCUSSION Airway obstruction in infants characterized predominantly by inspiratory stridor is usually indicative of an extrathoracic lesion
Intraluminal tracheal cyst producing airway obstruction in the newborn infant To the Editor: Laryngeal cysts in the newborn infant have been reported as a cause of airway obstructionr but no reports of intraluminal cysts of the trachea could be found upon review of the literature. CASE REPORT
Patient D. L., a male infant, was born after an uncomplicated pregnancy. Delivery was spontaneous, but the amnionic fluid was meconium stained. Respiratory distress with inspiratory stridor and prolonged expiration was noted immediately after delivery. The infant became hypoxemic and hypercapneie and was initially treated for meconium aspiration. A subsequent bronchoscopy was reported as negative. The infant was then referred to the Arizona Medical Center.
Fig. 1. This view of the esophagram demonstrates the smgoth, rounded contour of the intrathoracic tracheal mass. Note tha'~ the tracheal air shadow is nearly completely obliterated by the intrinsic mass. The trachea is not shifted. The esophagus is normal.
522
Letters to the Editor
involving the larynx or upper trachea. When obstruction is more severe during expiration, the lesion is usually intrathoracicY The lesion in our patient was in the intrathoracic trachea, and the more severe involvement of expiration is consistent with that location. Appropriate radiographic studies are essential in the evaluation of infants with airway obstruction. Lateral neck radiographs should always be obtained; in the case presented, however, it was the esophagram which demonstrated the lesion. This finding stresses the need for doing barium esophagrams in the evaluation of infants with airway obstruction, not only to demonstrate vascular rings, but also to detect intra-airway lesions. The etiology of the cyst is unclear. A comparison with laryngeal cysts may be appropriate since the lower larynx and the trachea have a common embryologic origin.:' Both have ciliated epithelium with mucous glands. Laryngeal mucous cysts result from obstruction of mucous gland ducts; the tracheal cyst presented may have developed in this fashion. Susan E. Denson, M.D. Lynn M. Taussig, M.D. Neonatology and Pulmonary Divisions Department of Pediatrics Gerald D. Pond, M.D. "Department of Pediatrics Department of Radiology Arizona Medical Center Tucson, Ariz. 85724 REFERENCES
1. Suehs OW, and Powell DB: Congenital cysts of the larynx in infants, Laryngoscope 77"654, 1967. 2. Sackner MA: Physiologic features of upper airway obstruction, Chest 62:414, 1972. 3. Langman J: Medical embryology, ed 2, Baltimore, 1969, The Williams & Wilkins Company.
The Chediak-Higashi syndrome in a Negro infant To the Editor: Since the first patient with the Chediak-Higashi syndrome was described by Bequez-Cesar in 1943,1 approximately 70 cases of the disorder have been reported. We wish to describe the occurrence of the Chediak-Higashi syndrome in a black infant who presented with some features of the accelerated phase of the disease. CASE REPORT
A black male infant was discharged from hospital at 5 days of age with a diagnosis of "albinism." At 2 months of age he was admitted to Rainbow Babies and Childrens Hospital because of fever and irritability. Laboratory data included a hematocrit value of 17%, hemoglobin concentration of 6.1 gm)dl, and white
The Journal of Pediatrics March 1976
blood cell count 3500/mm ~, with 6% neutrophils and 94% lymphocytes. Red blood cell morphology was normal. Platelets numbered 175,000/mm ~. The result of the nitroblue tetrazolium dye test was positive. A direct Coombs test was strongly positive, although no specific incompatibilities of blood group or type were identified. The diagnosis of Chediak-Higashi syndrome was confirmed by the presence of the typical cytoplasmic inclusions in the granulocytes on peripheral blood smear. Shortly after admission the patiem developed thrombocytopenia and prolonged prothrombin and partial thromboplastin times. However, no increase in circulating fibrin degradation products was detected. The infant was transfused with fresh whole blood, and chemotherapy with vincristine and prednisone was instituted. Response to this therapy was dramatic. Platelet count and other measurements of clotting function returned to normal within the next week, and the hematocrit rose following a significant reticulocystosis. The NBT test became negative and was never again positive. Immune globulins were repeatedly within or above the normal ranges for the infant's age during his subsequent course. Recurrent bacterial infections were not a prominent feature of this patient's hospital course. Seven months after his initial admission, the infant again developed a recurrence of the hemorrhagic diathesis with the thrombocytopenia and a Coombs-positive hemolytic anemia. Vincristine, prednisone, and antibiotic therapy were reinstituted, but the infant died. A complete description of autopsy findings will be published elsewhere. DISCUSSION In view of the chronic neutropenia and delayed killing of bacteria by neutrophils in this syndrome, it is somewhat surprising that infections did not appear to play a major role in the course of this infant's disease. He maintained normal or elevated immune globulin levels throughout his illness. The NBT test, initially positive, remained negative throughout the patient's hospital course. The Chediak-Higashi syndrome has been reported to be associated with a false positive NBT testy Cytotoxic therapy has been used in the "accelerated" phase of the syndrome, ~and our experience suggests that this mode of therapy may be beneficial in inducing prompt (if temporary) remission. The age of onset of the "accelerated" phase of the disease in this patient and the near fatal outcome during the first episode indicate that the disease may be terminal at an extremely early age and consequently may not be diagnosed, especially in Caucasians, prior to death. Sean O'Regan, M.B., B.Ch. Department of Nephrology Montreal Children's Hospital 2300 Tupper St. Montreal, P. Q., Canada H3H 1P3 Arthur J. Newman, M.A., M.D. Rafael Valenzuela, M.D. Sudesh P. Makker, M.D. Richard C. Graham, M.D. Department of Pediatrics & Medicine Case Western University School of Medicine Cleveland, Ohio 44106
