1. Hirst AE Jr, Johns VJ Jr, Kime SW: aneurysm of the aorta: A review of 505 cases. Medicine 37:217-279, 1958. 2. Hamilton WF, Abbott ME: Coarctation of the aorta of the adult type: I. Complete obliteration of the descending arch at insertion of the ductus in a boy of 14: Bicuspid aortic valve: Impending rupture of the aorta: Cerebral death: Statistical study and historical retrospect of 200 recorded cases, with autopsy, of stenosis or obliteration of descending arch in subjects above age of 2 years. Am Heart J 3:381-421, 1928. 3. Tobin JR Jr, Bay EG, Humphreys EM: Marfan's syndrome in adult: Dissecting aneurysm associated with arachnodactyly. Arch Intern Med 80:475-490, 1947. 4. Schneider JA, Schulman JD, Seegmiller JE: Cystinosis and the Fanconi syndrome, in Stanbury JB, Wyngaarden JB, Frederickson DS (eds): The Metabolic Basis of Inherited Disease. New York, McGraw-Hill Book Co Inc, 1978. 5. Schulman JD, Wong V, Olson WH, et al: Lysosomal site of crystalline deposits in cystinosis as shown by ferritin uptake. Arch Pathol 90:259-264, 1970. 6. Ueno A: Evaluation of management of dissecting aorta aneurysm. Jap J Thorac Surg 30:219-221, 1977. 7. Schnitker MA, Bayer CA: Dissecting aneurysm of the aorta in young individuals, particularly in association with pregnancy, with report of a case. Ann Intern Med 20:486-511, 1944. 8. Gore I: Dissecting aneurysms of the aorta in persons under 40 years of age. Arch Pathol 55:1-13, 1953. 9. Shennan T, cited by Gore I: Dissecting aneurysms of the aorta in persons under 40 years of age. Arch Pathol 55:1-13, 1953. 10. Bronson E, Sutherland GA: Ruptured aortic aneurysm in childhood, with the report of a case. Br J Child Dis 15:241-258, 1918. 11. Von Rokitansky K: Uber einige der wichtigsten Krankeiten der Arterien. Denkschr Akad Wissench 4:41, 1852. 12. Coleman PN: A case of dissecting aneurysm in a child. J Clin Pathol 8:313-317, 1955. 13. Gelfand M: Giant cell arteritis with aneurysmal formation in an infant. Br Heart J 17:264, 1955. 14. Strauss RG, McAdams AJ: Dissecting aneurysm in childhood. J Pediatr 76:578-584, 1970. 15. Schlafmann JSM, Becker AE: Histologic changes in the normal aging aorta: Implications for dissecting aortic aneurysm. Am J Cardiol 39:13-20, 1977.

Genital Malformations in a Child With VATER Association

Dissecting

of a Circumcision Performed With a Plastic Disposable Circumcision Device:

Complication

Long-term Follow-up

A complication of a circumcision performed with a plastic bell clamp was previously reported.1 Since that report, we have had inquiries concerning the long-term physical effects in that child. To answer these inquiries, we present these photographs of the appearance of the glans penis in that boy at 6 months of age (Fig 1) and again at 7 years of age (Fig 2). Both the ridge and the sulcus have remained after this long period of time. A longer follow-up may be desirable to

determine the effects of sexual matu-

Since the initial description in 1968,1 knowledge of this entity of multiple congenital malformations has steadily expanded. Recently, it was suggested that malformation of external genitalia in males may be part of abnormalities found in this condition.2 The following report describes a girl with vertebral defects, anal atresia, T-E fistula, renal defects, and radical limb dysplasia (VATER) association who, in addition, has severe malformations of the genitalia.

Fig 1.\p=m-\Glanspenis

at 6 months of age.

Fig 2.\p=m-\Glanspenis

at 7 years of age.

ration on these structural changes, but it could be reasonably assumed that these changes will remain throughout life. These interim photographs will be of assistance to physicians who have had similar complications. ROGER E. JOHNSONBAUGH, MD, PHD Endocrine Division Department of Pediatrics National Naval Medical Center and Uniformed Services University of the Health Sciences Bethesda, MD 20014 Supported in part by project CICC 8-06-1146 from the Bureau of Medicine and Surgery, Navy Department, Washington, DC. The opinions or assertions contained herein are the private ones of the author and are not to be construed as official or reflecting the views of the Navy Department, the naval service at large, or the Department of Defense. Johnsonbaugh RE, Meyer BP, Catalano JD: Complication of a circumcision performed with a plastic bell clamp. Am J Dis Child 118:781, 1.

1969.

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Report of a Case.\p=m-\A4-year-old girl had been born six weeks prematurely and weighed 1,530 g. The mother had been 29 years of age and the father 31 years of age at the time of the patient's birth. The pregnancy had been complicated by spotting at seven or eight weeks. There was no history of exposure to teratogens and no family history of malformations. The mother had not taken contraceptive pills for two years prior to pregnancy. At birth, the patient's malformations consisted of tracheoesophageal fistula with distal esophageal atresia, imperforate anus with low rectal vaginal fistula, hypoplastic right radius, 13 ribs bilaterally, probable agenesis of the left kidney, and six lumbar vertebrae.3 Neurological examination showed hypotonia in the trunk and the extremities. Ophthalmologic evaluation showed her left eye to be severely myopic (-8 diopters). Recently, she

was reevaluated in prepa¬ ration for anal transplantation. She was found to have a completely septate vagina with membranous septum between the two and a cervix identifiable in each vaginal tract. Both cervices appeared to be normal for age, and a single urethra was observed.

Comment.-Apold et al,2 in 1976, reported their observation of genital

malformations in three of seven male patients with VATER association. In two, these consisted of a bifid scrotum and caudally displaced dysplastic pe¬ nis. The third patient had mild defects in the form of a split preputium and a coronal hypospadias. A brief review of the literature disclosed several other instances of hypospadias in males with the VAT¬ ER association.12 One female with

pseudohermaphroditism4 and, more interestingly, another with agenesis of the fallopian tubes, uterus, and vagina5 have also been reported.

We believe it is important that the possibility of genital malformations

be considered in male or female patients with VATER association. We

think that the lack of reports in this regard may merely point out the necessity of more detailed physical examination in such cases. BURHAN SAY, MD NANCY J. CARPENTER, PHD Department of Pediatrics Children's Medical Center PO Box 35648 Tulsa, OK 74135 E. IDE SMITH, MD Health Sciences Center University of Oklahoma Oklahoma City, OK 73190 1. Say B, Gerald PS: A new polydactyly/ imperforate anus/vertebral anomalies syndrome? Lancet 2:688, 1968. 2. Apold J, Dahl E, Aarskog A: The VATER

association: Malformations of the male external

genitalia. Acta Paediatr Scand 65:150-152, 1976. 3. Say B, Greenberg D, Harris R, et al: The anus/vertebral radial dysplasia/imperforate anomalies syndrome (the VATER association): Developmental aspects and eye findings. Acta Paediatr Scand 66:233-235, 1977. 4. Temtamy SA, Miller JD: Extending the

scope of the VATER association: Definition of the VATER syndrome. J Pediatr 85:345-349, 1974. 5. King SL, Ladda RL, Schochat SJ: Monozygotic twins concordant for tracheo-esophageal fistula and discordant for the VATER association. Acta Paediatr Scand 66:783-785, 1977.

Methemoglobinemia Owing

to

Rectal-Probe Lubrication

Methemoglobinemia is cyanosis in the absence

a cause of of lung or cardiac disease. Nursery outbreaks owing to marking ink on diapers1 and 3.25% triclocarban (3-4-41 trichlorocarbanilide) in the diaper rinse2 have been reported. Benzocaine has caused methemoglobinemia when given as a rectal suppository to infants and children,3-5 when used as an endotracheal tube lubricant in an older child,6 and when used topically on skin7-11 or oral mucosa.12,13 We wish to report another potential route of exposure.

Report of

a Case.\p=m-\A 1,030-g female born at Tampa (Fla) General Hospital to a 19-year-old gravida 1 four days after spontaneous rupture of the membranes. The estimated gestational age was 27 weeks, and the mother had received two doses of betamethasone. Delivery was spontaneous, and the Apgar scores were 8 and 9, at 1 and 5 minutes of age, respectively. The initial gastric aspirate showed many polymorphonuclear cells; the patient was given intravenous ampicillin sodium and gentamicin sulfate. The CSF, blood, and urine cultures were sterile, but Escher¬ ichia coli grew from the gastric aspirate and antibiotics were continued. Small feed¬ ings of formula were begun and were tolerated well. Apnea and bradycardia

infant,

was

began on the second day of life and were initially controlled with cutaneous stim¬ ulation. On the eighth day of life, the apnea and bradycardia worsened, abdom¬ inal distention developed, and pneumatosis

intestinalis was seen on abdominal roent¬ genograms. On the ninth day, an abscess was felt in the right lower quadrant, and a cecal

perforation

was

demonstrated at

surgery that was performed under nitrous oxide and pancuronium bromide anesthe¬ sia. The cecum and terminal ileum were

resected and a primary anastomosis was made. On arrival back in the Neonatal Inten¬ sive Care Unit, the infant was noted to be dusky and hypotensive. The administra¬ tion of 10 mL of 5% plasma protein solution returned the blood pressure to normal, but the child remained dusky and tachypneic, with a Pao, of 195 mm Hg. The infant's blood was chocolate colored despite expo¬ sure to 100% oxygen. The child rapidly regained a pink color when 2 mg of méthy¬ lène blue were given intravenously. Methemoglobin levels were not measured because a 5-mL sample of blood is required by our laboratory, and the parent's religion for¬ bids the transfusion that might then have been necessary. It was subsequently discovered that the rectal, automatic tem¬ perature probe used during surgery had been lubricated with Américaine Lubricant and Ointment, which is a mixture of benzo¬ caine (20%) and benzethonium chloride. The application of ointment to the rectal probe deposits about 0.2 mL of ointment or 40 mg of benzocaine. The package insert contains the warning against use in infants less than 1 year of age. Methemo¬ globinemia has not recurred in this infant.

Comment.—Methemoglobin is nor¬ mally present in concentrations of less than 2% of the total hemoglobin in adults, 0% to 2.8% in full-term infants, and as high as 4.7% in the prematurely born." Acquired methemoglobinemia is more commonly seen in infants, presumably because of a transient deficiency of nicotinamide adenine dinucleotide (reduced form)-dependent methemoglobin reducíase.'~'~17 As little as 1.5 to 2 g/dL of methemoglo¬ bin, or 10% of the normal hemoglo¬

methemoglobin

to

hemoglobin.

Causative agents may be classified as direct oxidants, such as nitrites, or indirect oxidants that do not form methemoglobin from hemoglobin in vitro but presumably are metabolized within the body to active agents. Benzocaine is this latter type of

compound. In the patient reported here, only a presumptive diagnosis of methemo¬ globinemia can be made since methe¬ moglobin was not measured. The clin¬ ical history, color of the blood, and response to méthylène blue make methemoglobinemia a likely diagno¬ sis, however. The danger of benzo¬ caine in infants is reemphasized and its

lubricant is condemned. JAMES M. SHERMAN, JR, MD Department of Pediatrics Rainbow Babies and Childrens

use as a

Hospital

2101 Adelbert Rd Cleveland, OH 44106 Kim SMITH, MD Department of Pediatrics

Tampa General Hospital Davis Island FL 33606

Tampa,

Names and Trademarks of Drugs

Nonproprietary

Ampicillin sodium—Alpen-N, Amcill-S, Pen A/N, Penbritin-Sfor Injection, Polycillin-Nfor Injection, Principen/N, Omnipen-N, SK-Ampicillin-N. Gentamicin sulfate—Garamycin. Pancuronium bromide—Pavulon. 1. Ramsay DHE, Harvey CC: Marking poisoning. Lancet 1:910-912, 1959.

ink

bin concentration in the newborn, may produce cyanosis. A level of 25%

2. Johnson RR, Navone R, Larson EL: An unusual epidemic of methemoglobinemia. Pediatrics 31:222-225, 1963. 3. Huges JR: Infantile methemoglobinemia due to benzocaine suppository. J Pediatr 66:797\x=req-\ 799, 1965. 4. Peterson, HD: Acquired methemoglobinemia in an infant due to benzocaine suppository. N Engl J Med 263:454-455, 1960. 5. Bloch A: More on infantile methemoglobinemia due to benzocaine suppositories. J Pediatr 67:509-510, 1965. 6. Steinberg JB, Zepernick RG: Methemoglobinemia during anesthesia. J Pediatr 61:885-886,

levels of 40%, whereas lethargy and stupor occur at levels of approximate¬ ly 60%.1R The degree of methemoglobi¬ nemia produced by any agent depends on the activity of the compound or its intermediates, the rates of absorption and excretion of the compound, and the endogenous rate of reduction of

benzocaine. Pediatrics 21:340-341, 1958. 10. Haggerty RJ: Blue baby due to methemoglobinemia. N Engl J Med 267:1303, 1962. 11. Townes PL, Geertsma MA, White MR: Benzocaine-induced methemoglobinemia. Am J Dis Child 131:697-698, 1977.

methemoglobin

may produce no symptoms other than cyanosis. Tachy¬ cardia and hyperpnea may be seen at

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1962. 7. Goluboff N, MacFadyen DJ: Methemoglobinemia in an infant: Associated with application of a tar-benzocaine ointment. J Pediatr 47:222\x=req-\ 226, 1955. 8. Wolff JR: Methemoglobinemia due to benzocaine. Pediatrics 20:915-916, 1957. 9. Goluboff N: Methemoglobinemia due to