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Association of supernumerary nipples with other anomalies
274
Brief clinical and laboratory observations
ability to produce chemotactic factors are normal in patients with clinically stable cystic fibrosis. It is unlikely that a primary congenital deficiency of systemic immunity is involved in the pathogenesis of cystic fibrosis. The authors gratefully acknowledge the expert technical assistance of Ms. Judy Faust and Ms. Carol Nye.
The Journal of Pediatrics August 1979
4.
5.
REFERENCES
1. Talamo RC, Stiehm ER, and Schwartz RH: Immunologic aspects of cystic fibrosis, in Mangos JA, and Talamo RC, editors: Cystic fibrosis: Projections into the future, New York, 1976, Stratton Intercontinental, pp 195-217. 2. Gallin JI: Abnormal chemotaxis: cellular and humoral components, in Bellanti JA, and Dayton DH, editors: The phagocytic cell in host resistance, New York, 1975, Raven Press, pp 227-243. 3. Lett-Brown MA, Boetcher DA, and Leonard EJ: Chemotac-
6
7. 8.
tic responses of normal human basophils to C5a and to lymphocyte derived chemotactic factor, J Immunol 117:246, 1976. Klein RB, Fischer TJ, Gard S, et al: Decreased mononuclear and polymorphonuclear chemotaxis in human newborns, infants and young children, Pediatrics 60:467, 1977. Orr W, and Ward PA: Quantitation of leukotaxis in agarose by three different methods, J Immunol Methods 20:95, 1978. Sorensen RU, Stern RC, and Polmar SH: Lymphocyte responsiveness to Pseudomonas aeruginosa in cystic fibrosis: relationship to status of pulmonary disease in sibling pairs, J PEDIATR93:201, 1978. Conover JH, Conod EJ, and Hirschhorn K: Complement components in cystic fibrosis, Lancet 2:1501, 1973. Hill HR, Warwick WJ, DettloffJ, and Quie PG: Neutrophil granulocyte function in patients with pulmonary infection, J PEDIATR84:55, 1974.
Association of supernumerary nipples with other anomalies K. Mrhes, M.D~, Gyg)r, Hungary
As PART of a longitudinal study to determine the significance of given minor anomalies/we evaluated our findings in children with supernumerary nipples. MATERIALS
AND METHODS
During a newborn screening program for detection of minor malformations, 4,100 babies were consecutively investigated in the neonatal unit of our hospital Every infant was examined specifically for supernumerary nipple. In addition, without any other selection 1,689 children admitted to our pediatric department in 1976 were examined to determine whether they had accessory nipples. In a third group, 129 patients seen for genetic counseling because of various familial malformations or mental retardation or both were investigated. A supernumerary nipple was scored when a small pigmented or pearl colored mark or concave spot was seen below and somewhat medial to the mammilla. Patients with such lesions were followed for at least one year, the majority for more than two years. RESULTS Among 4,100 neonates, nine had an accessory nipple, an incidence of 0.22%. Three examples were discovered Reprint address: Department of Pediatrics, County Hospital, Pf 92. H-9002 GyOr, Hungary.
during genetic counseling, and another nine in hospitalized patients. Their ages varied from 2 weeks to 16 months. Thus, 21 babies with supernumerary nipples were identified. At the first examination, only threcWof these 21 infants were noted to have a congenital defect: bilateral hydronephrosis in two and pyloric stenosis in one infant. Of the asymptomatic children, 17 could be followed; in seven of them, obscure major abnormalities were ascertained, including six with major anomalies of the kidney or renal collecting system, and another infant with pyloric stenosis. One additional child had epilepsy (Table). DISCUSSION The frequency of accessory nipples was estimated to be about 1/500 in a review by Leichtenstern~ one hundred years ago. This agrees with our findings in screening unselected newborn infants. Supernumerary nipples or breasts may occur at any site on the body 3. 4; however, in neonates and infants we found them below the normally placed mamillae only, on the line extending from the nipple to the symphysis pubis. The anomaly may easily be overlooked in young infants, in whom it often appears as a small spot with a diameter of only 2 to 3 mm. A wide range of abnormalities associated with supernumerary nipples has been described? -7 The most convinc-
0022-3476/79/080274+02500.20/0 9 1979 The C. V. Mosby Co.
Vohone 95 Number 2
B r i e f clinical and laboratory observations
Table. Association of supernumerary nipples with other anomalies
.o,i
Mode o f discovery
Routine newborn examination Unselected hospitalized patients Genetic counseling Total
infants followed
)
Renal anomalies
9
3
8
4
3
1
20
8
Other anomalies
1 Pyloric stenosis 1 Pyloric stenosis 1 Epilepsy
ing connection exists between accessory nipples and renal anomalies. This is confirmed by our study: in eight of 20 children with polythelia, significant renal abnormalities could be identified. These were mostly obstructive. In contrast to earlier assumptions, 7 no supernumerary kidneys were found. Two infants in our series had the hitherto not described association of accessory nipple and congenital pyloric stenosis. Thus, half of the apparently normal infants with accessory mammillae had serious hidden malformations. As previously noted, ~ mammillary abnormalities, and especially accessory nipples, are potentially significant
275
minor malformations. They should be sought in the routine examination o f the newborn infant, and if identified, indicate the need for careful follow-up of the child a n d , at the first suspicion of renal disease, radiographic studies o f the kidneys. REFERENCES
1. M6hes K: A follow-up study of infants with minor malformations, in Szab6 G, and Papp Z, editors: Medical genetics, Budapest-Amsterdam, 1977; Akad6miai Kiad6-Excerpta Medica, pp 483-486. 2, Leichtenstern D: Ueber das Vorkommen und Bedeutung supernumer~rer (accessorischer) BriJste und Brustwarzen, Arch Pathol Anat Physiol Klin Med 73:222, 1878. 3. John C: lJber akzessorische Milchdrfisen und Warzen, insbesondere fiber milchdrOsen~ihnliche Bildungen in der Aehselh6hle, Arch Gyn~ikol 126:689, 1925. 4. Eckert M, and Hammann HJ: Mammaektopie am Rt~cken, Dtsch Med Woehenschr 100:1395, 1975. 5. Carella A: Supernumerary breast associated with multiple vertebral malformations. Case report, Acta Neurol (Napoli) 26:136, 1971. 6. M~tt6, K., Horwith, J., and Schmidt, J.: Association of polythelia and aberrant ventricular conduction (Hungarian), Orv Hetilap 117:2863, 1976. 7. Goeminne L.: Synopsis of mammo-renal syndromes, Humangenetik 14:171, t972. 8. M6hes, K.: A simple score to facilitate detection of congenital disorders, Acta Paediatrs Acad Sci Hung 18:61, 1977.
Intracranial hypertension due to eosinophilic granuloma of the temporal bone Alberto Leiberman, M.D., Asher Tal, M.D., and Jacob Bar Ziv, M.D., Beer-Sheba, Israel
G R A N U L O M A is t h e benign form of histiocytosis-X. 1 Involvement of the skull, ribs, pelvis, and long bones are common. W h e n the disease occurs in the temporal bone, the patient may present with acute or chronic otitis media. We report an unusual case of eosinophilic granuloma of the temporal bone presenting with signs o f intracranial hypertension. EOSINOPHILIC
CASE REPORT
A 589 boy was referred because of severe headaches and a five-day history of vomiting and high fever. This was From the Departments of Ear, Nose and Throat, Pediatrics "B," and Pediatric Radiology, The Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev. Reprint address: Department of Ear, Nose and Throat, The Soroka Medical Center, P.O. Box 151, Beer-Sheba, Israel.
0022-3476/79/080275 +02500.20/0 9 1979 The C. V. Mosby Co.
attributed to acute otitis media and antibiotics were administered. At this time neither earache nor discharge was present. The physical examination on admission revealed a normal body temperature. Signs of right abducens nerve palsy were evident, as well as mild paresis of the right facial muscles. The Kernig sign was present and the Babinski sign was positive on the right side. Funduscopic examination revealed bilateral papilledema with hemorrhage. The diagnosis of increased intracranial pressure was established and substantiated by separated sutures on skull radiograph. The right external auditory canal was blocked by crust formation; upon its removal purulent material was also removed revealing granulation tissue filling the external auditory canal. Radiologic examination of the right ear including tomographic sectioning showed destruction of the mastoid process, extending to the area of the jugular foramen, the posterior wall of external auditory canal, and the canal of the descending facial nerve. Pu~:e tone aadiometry showed a conductive hearing loss of 60 db on
Brief clinical and laboratory observations
ability to produce chemotactic factors are normal in patients with clinically stable cystic fibrosis. It is unlikely that a primary congenital deficiency of systemic immunity is involved in the pathogenesis of cystic fibrosis. The authors gratefully acknowledge the expert technical assistance of Ms. Judy Faust and Ms. Carol Nye.
The Journal of Pediatrics August 1979
4.
5.
REFERENCES
1. Talamo RC, Stiehm ER, and Schwartz RH: Immunologic aspects of cystic fibrosis, in Mangos JA, and Talamo RC, editors: Cystic fibrosis: Projections into the future, New York, 1976, Stratton Intercontinental, pp 195-217. 2. Gallin JI: Abnormal chemotaxis: cellular and humoral components, in Bellanti JA, and Dayton DH, editors: The phagocytic cell in host resistance, New York, 1975, Raven Press, pp 227-243. 3. Lett-Brown MA, Boetcher DA, and Leonard EJ: Chemotac-
6
7. 8.
tic responses of normal human basophils to C5a and to lymphocyte derived chemotactic factor, J Immunol 117:246, 1976. Klein RB, Fischer TJ, Gard S, et al: Decreased mononuclear and polymorphonuclear chemotaxis in human newborns, infants and young children, Pediatrics 60:467, 1977. Orr W, and Ward PA: Quantitation of leukotaxis in agarose by three different methods, J Immunol Methods 20:95, 1978. Sorensen RU, Stern RC, and Polmar SH: Lymphocyte responsiveness to Pseudomonas aeruginosa in cystic fibrosis: relationship to status of pulmonary disease in sibling pairs, J PEDIATR93:201, 1978. Conover JH, Conod EJ, and Hirschhorn K: Complement components in cystic fibrosis, Lancet 2:1501, 1973. Hill HR, Warwick WJ, DettloffJ, and Quie PG: Neutrophil granulocyte function in patients with pulmonary infection, J PEDIATR84:55, 1974.
Association of supernumerary nipples with other anomalies K. Mrhes, M.D~, Gyg)r, Hungary
As PART of a longitudinal study to determine the significance of given minor anomalies/we evaluated our findings in children with supernumerary nipples. MATERIALS
AND METHODS
During a newborn screening program for detection of minor malformations, 4,100 babies were consecutively investigated in the neonatal unit of our hospital Every infant was examined specifically for supernumerary nipple. In addition, without any other selection 1,689 children admitted to our pediatric department in 1976 were examined to determine whether they had accessory nipples. In a third group, 129 patients seen for genetic counseling because of various familial malformations or mental retardation or both were investigated. A supernumerary nipple was scored when a small pigmented or pearl colored mark or concave spot was seen below and somewhat medial to the mammilla. Patients with such lesions were followed for at least one year, the majority for more than two years. RESULTS Among 4,100 neonates, nine had an accessory nipple, an incidence of 0.22%. Three examples were discovered Reprint address: Department of Pediatrics, County Hospital, Pf 92. H-9002 GyOr, Hungary.
during genetic counseling, and another nine in hospitalized patients. Their ages varied from 2 weeks to 16 months. Thus, 21 babies with supernumerary nipples were identified. At the first examination, only threcWof these 21 infants were noted to have a congenital defect: bilateral hydronephrosis in two and pyloric stenosis in one infant. Of the asymptomatic children, 17 could be followed; in seven of them, obscure major abnormalities were ascertained, including six with major anomalies of the kidney or renal collecting system, and another infant with pyloric stenosis. One additional child had epilepsy (Table). DISCUSSION The frequency of accessory nipples was estimated to be about 1/500 in a review by Leichtenstern~ one hundred years ago. This agrees with our findings in screening unselected newborn infants. Supernumerary nipples or breasts may occur at any site on the body 3. 4; however, in neonates and infants we found them below the normally placed mamillae only, on the line extending from the nipple to the symphysis pubis. The anomaly may easily be overlooked in young infants, in whom it often appears as a small spot with a diameter of only 2 to 3 mm. A wide range of abnormalities associated with supernumerary nipples has been described? -7 The most convinc-
0022-3476/79/080274+02500.20/0 9 1979 The C. V. Mosby Co.
Vohone 95 Number 2
B r i e f clinical and laboratory observations
Table. Association of supernumerary nipples with other anomalies
.o,i
Mode o f discovery
Routine newborn examination Unselected hospitalized patients Genetic counseling Total
infants followed
)
Renal anomalies
9
3
8
4
3
1
20
8
Other anomalies
1 Pyloric stenosis 1 Pyloric stenosis 1 Epilepsy
ing connection exists between accessory nipples and renal anomalies. This is confirmed by our study: in eight of 20 children with polythelia, significant renal abnormalities could be identified. These were mostly obstructive. In contrast to earlier assumptions, 7 no supernumerary kidneys were found. Two infants in our series had the hitherto not described association of accessory nipple and congenital pyloric stenosis. Thus, half of the apparently normal infants with accessory mammillae had serious hidden malformations. As previously noted, ~ mammillary abnormalities, and especially accessory nipples, are potentially significant
275
minor malformations. They should be sought in the routine examination o f the newborn infant, and if identified, indicate the need for careful follow-up of the child a n d , at the first suspicion of renal disease, radiographic studies o f the kidneys. REFERENCES
1. M6hes K: A follow-up study of infants with minor malformations, in Szab6 G, and Papp Z, editors: Medical genetics, Budapest-Amsterdam, 1977; Akad6miai Kiad6-Excerpta Medica, pp 483-486. 2, Leichtenstern D: Ueber das Vorkommen und Bedeutung supernumer~rer (accessorischer) BriJste und Brustwarzen, Arch Pathol Anat Physiol Klin Med 73:222, 1878. 3. John C: lJber akzessorische Milchdrfisen und Warzen, insbesondere fiber milchdrOsen~ihnliche Bildungen in der Aehselh6hle, Arch Gyn~ikol 126:689, 1925. 4. Eckert M, and Hammann HJ: Mammaektopie am Rt~cken, Dtsch Med Woehenschr 100:1395, 1975. 5. Carella A: Supernumerary breast associated with multiple vertebral malformations. Case report, Acta Neurol (Napoli) 26:136, 1971. 6. M~tt6, K., Horwith, J., and Schmidt, J.: Association of polythelia and aberrant ventricular conduction (Hungarian), Orv Hetilap 117:2863, 1976. 7. Goeminne L.: Synopsis of mammo-renal syndromes, Humangenetik 14:171, t972. 8. M6hes, K.: A simple score to facilitate detection of congenital disorders, Acta Paediatrs Acad Sci Hung 18:61, 1977.
Intracranial hypertension due to eosinophilic granuloma of the temporal bone Alberto Leiberman, M.D., Asher Tal, M.D., and Jacob Bar Ziv, M.D., Beer-Sheba, Israel
G R A N U L O M A is t h e benign form of histiocytosis-X. 1 Involvement of the skull, ribs, pelvis, and long bones are common. W h e n the disease occurs in the temporal bone, the patient may present with acute or chronic otitis media. We report an unusual case of eosinophilic granuloma of the temporal bone presenting with signs o f intracranial hypertension. EOSINOPHILIC
CASE REPORT
A 589 boy was referred because of severe headaches and a five-day history of vomiting and high fever. This was From the Departments of Ear, Nose and Throat, Pediatrics "B," and Pediatric Radiology, The Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev. Reprint address: Department of Ear, Nose and Throat, The Soroka Medical Center, P.O. Box 151, Beer-Sheba, Israel.
0022-3476/79/080275 +02500.20/0 9 1979 The C. V. Mosby Co.
attributed to acute otitis media and antibiotics were administered. At this time neither earache nor discharge was present. The physical examination on admission revealed a normal body temperature. Signs of right abducens nerve palsy were evident, as well as mild paresis of the right facial muscles. The Kernig sign was present and the Babinski sign was positive on the right side. Funduscopic examination revealed bilateral papilledema with hemorrhage. The diagnosis of increased intracranial pressure was established and substantiated by separated sutures on skull radiograph. The right external auditory canal was blocked by crust formation; upon its removal purulent material was also removed revealing granulation tissue filling the external auditory canal. Radiologic examination of the right ear including tomographic sectioning showed destruction of the mastoid process, extending to the area of the jugular foramen, the posterior wall of external auditory canal, and the canal of the descending facial nerve. Pu~:e tone aadiometry showed a conductive hearing loss of 60 db on