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Perforation of the scrotum complicating nephrotic syndrome
336
9. 10.
11. 12.
Clinical and laboratory observations
subdural hemorrhage in a neonate with hemophilia A. Am J Pediatr Hematol Oncol 1985;7:384-7. Bray GL, Luban NLC. Hemophilia presenting with intraeranial hemorrhage. Am J Dis Child 1987;141:1215-7. Gilchrist GS, Piepgras DG, Roskos RR. Neurologic complications in hemophilia. In: Hilgartner MW, ed. Hemophilia in the child and adult. Chicago: Masson USA, 1982:99-120. Silverstein A. Intracranial bleeding in hemophilia. Arch Neurol 1960;3:141-57. Hilgartner MW, McMillan CW. Coagulation disorders. In: Miller DR, Baehner RL, McMillan CW, eds. Blood diseases
The Journal of Pediatrics August 1988
of infancy and childhood. 5th ed. St. Louis: CV Mosby, 1984;858-921. 13. Schmidt B, Zipursky A. Disseminated intravascular coagulation masking neonatal hemophilia. J PEDIATR 1986;109: 886-8. 14. Buchanan GR. Hemorrhagic diseases. In: Nathan DG, Oski FA, eds. Hematology of infancy and childhood. 3rd ed. Philadelphia: WB Saunders, 1987:104-27. 15. Andrew M, Paes B, Milner R, et al. Development of the human coagulation system in the full-term infant. Blood 1987;70:165-72.
Perforation of the scrotum complicating nephrotic
syndrome Thomas R. Welch, MD, John Gianis, MD, a n d Curtis A. Sheldon, MD From the Departments of Pediatrics and Surgery (Urology), University of Cincinnati, and the Divisions of Nephrology and Pediatric Urology, Children's Hospital Medical Center and Children's Hospital Research Foundation, Cincinnati, Ohio
Scrotal e d e m a often accompanies the a n a s a r c a of childhood nephrotic syndrome. A l t h o u g h this feature is often dramatic, it rarely presents a significant problem. W e report on a child in whom nephrotic syndrome was accompanied by the complication of spontaneous scrotal rupture.
CASE R E P O R T A 2-year-old boy was well until 19 months of age, when he developed nephrotic syndrome. He was treated with prednisone, 30 mg/day, for presumed minimal change disease. His nephrosis resolved, and his corticosteroid therapy was changed to an alternate-day regimen. Two recurrences of proteinuria and edema occurred while he was receiving alternate-day corticosteroid therapy, each managed with a brief course of steroids given daily. When the boy was 22 months of age, recurrent proteinuria and edema necessitated a resumption of daily therapy. Nevertheless, he developed progressive anasarca and was admitted to the hospital. Examination at that time showed an acutely distressed boy whose weight of 15.5 kg represented a recent gain of 3.0 kg. Tense ascites and generalized pitting edema were present. The scrotum was massively enlarged, with a diameter of 12 cm. The child was treated with intravenous albumin and methylprednisolone. His weight increased to 18.4 kg, scrotal swelling progressed, and proteinuria and hypoproteinemia continued. After
Submitted for publication Feb. 19, 1988; accepted Feb. 24, 1988. Reprint requests: Thomas R. Welch, MD, Children's Hospital Research Foundation, Division of Nephrology, Elland and Bethesda Avenues, Cincinnati, OH 45229-2899.
a week without improvement, chlorambucil was added to the regimen. Examination of the child's scrotum revealed a few small areas of what was thought to be a monilial dermatitis, which was treated with topical nystatin. Although no full-thickness cutaneous injury was present, resolution of the dermatitis left an area of epidermal thinning. After 6 more days, the patient's proteinuria decreased and a diuresis began. Coincident with his improvementl the child began walking for the first time since admission to the hospital. On the day that discharge was planned, he fell while walking and was noted to have a large amount of fluid leaking from his diaper. Examination at that time revealed a 2 cm scrotal laceration. Urologic consultation was obtained, and the child was taken to the operating room for exploration and repair of the wound. The entire wound was excised, irrigated copiously with an antimicrobial solution, and closed in layers. Antibiotics for the treatment of potential infection with hospital-acquired pathogens were administered. The child's nephrotic syndrome continues to be in remission, and his scrotal wound has completely healed. DISCUSSION Scrotal swelling has been recognized as a feature of childhood nephrosis since the early descriptions of the condition. 1 This fluid accumulation sometimes represents hydrocele or c o m m u n i c a t i o n with the fluid-filled peritoneu m t h r o u g h a patent processus vaginalis. M o r e often, as in our patient, tense ascites impairs venous a n d lymphatic r e t u r n from the scrotum, producing lymphedema. T h e degree of scrotal e n l a r g e m e n t can be massive, especially in children with a large a m o u n t of ascites. A l t h o u g h older
Volume t 13 Number 2
patients often complain of some discomfort with walking, it has been our experience that significant complaints are rare. The scrotum in these children may be huge and the skin extraordinarily thin, but we had never seen a patient develop an injury such as the one reported here. The major current pediatric nephrology texts 2"5 do not mention this complication, and we could find no similar published reports. The principles of surgical management include preoperative examination and intraoperative inspection to ensure that the massive fluid seepage does not represent leakage of ascitic fluid from a ruptured communicating hydrocele. In this instance, there was massive soft-tissue edema of the scrotum without evidence of scrotal or inguinal mass by history or examination. The absence of ascitic drainage was further confirmed by careful scrotal exploration during which an intact tunica vaginalis was found. Physicians caring for children with nephrotic syndrome should be aware of this potential, albeit rare, complication. Presumably, toddlers whose gait is still somewhat immature would be more likelty to develop such an injury than
Clinical and laboratory observations
337
would older children. Furthermore, the presence of a seemingly trivial dermatitis, as in our patient, may further compromise the integrity of the already-attenuated epidermis. Enforced bed rest is i m p r a c t i c a l - - a n d perhaps even ill advised in nephrotic syndrome unless dermatitis or focal attenuation is encountered. Perhaps, however, children with this condition would benefit from bulky support in the form of heavy diapers, until scrotal swelling lessens. REFERENCES
1. Ratehford BK. Diseases of children. New York: D Appleton, 1912:576. 2. Royer P, Habib R, Mathieu H, Buoyer M. Pediatric nephrology. Philadelphia: WB Saunders, 1974:258-71. 3. Arneil GC. Management of the nephrotic syndrome in the child. In" Liebermann E, ed. Clinical pediatric nephrology. Philadelphia: JB Lippincott, 1976:146-72. 4. Barnett HL, Schoeneman M, Bernstein J, Edelmann C M . The nephrotic syndrome. In: Edelmann CM, ed. Pediatric kidney disease. Boston: Little, Brown, 1978:679-95. 5. Vernier RL. Primary (idiopathic) nephrotic sy+~:lrome. In: Holliday MA, Banatt TM, Vernier RL, eds. Pediatric nephrology. Baltimore: Williams & Wilkins, 1987:445-56.
Occurrence of prolactinoma after estrogen treatment in a girl with constitutional tall stature E. Panteon, MD, E. Loumaye, MD, M. Maes, MD, a n d P. Malvaux, MD From the Departments of Pediatrics, and Gynecology and Obstetrics, Cliniques Universitaires Saint-Luc, Universit~ Catholique de Louvain, Brussels, Belgium
Galactorrhea is an infrequent complication of estrogen therapy for constitutional tall stature in girls. ~'2 The following case history indicates that high-dose estrogen administration in some tall girls may be associated with prolactinoma. CASE R E P O R T S A 12~f2-year-oldgirl was referred to our clinic for evaluation of tall stature. Her parents were tall: the father 192.6 era, and the mother 189.1 cm. Although the girls did not appear to have major psychologic problems, the mother was concerned that she wmild have difficulties in relating to peers and in engaging in professional iife, as had the mother. Her height was 171.5 era (above the 97th percentile on the Tanner growth curves), and her weight 44.8
Submitted for publicationSept. 14, 1987; accepted March 22, 1988. Reprint requests: E. Panteon, MD, Department of Pediatrics, Cliniques Universitaires Saint-Luc, Universite Catholique de Louvain, Brussels, Belgium.
kg (75th percentile). She was in early puberty, according to the Tanner pubertal ratings, with onset of breast development (B2) and of pubic and axillary hair (P2, A2). Visual fields were normal by frontal confrontation. Findings of physical examination were normal. Her bone age was 11)/2 years according to the Greulich and Pyle atlas, and her predicted mature height was estimated at 192.5 cm, using the Bayley and Pinneau tables. Because of normal history and physical findings and her parents' heights, she was considered to be constitutionally tall. Because of the consequences of her tallness on her present and future psychologic well-being, the patient and her parents agreed to a reduction of final height. She was given 300 t~g ethinyt estradiol daily, and 10 mg lynestrenol per day, seven days a month. Before onset of therapy serum prolactin, measured by radioimmunoassay, was 1.7 ng/ml (normal <20 ng/ml). The hormonal treatment was welt tolerated, with expected decelerated growth velocity and'accelerated pubertal development and bone maturation (Table). Therapy was stopped after 25 months, when the patients was I4y2 years of age and her bone age had reached 16 years.
9. 10.
11. 12.
Clinical and laboratory observations
subdural hemorrhage in a neonate with hemophilia A. Am J Pediatr Hematol Oncol 1985;7:384-7. Bray GL, Luban NLC. Hemophilia presenting with intraeranial hemorrhage. Am J Dis Child 1987;141:1215-7. Gilchrist GS, Piepgras DG, Roskos RR. Neurologic complications in hemophilia. In: Hilgartner MW, ed. Hemophilia in the child and adult. Chicago: Masson USA, 1982:99-120. Silverstein A. Intracranial bleeding in hemophilia. Arch Neurol 1960;3:141-57. Hilgartner MW, McMillan CW. Coagulation disorders. In: Miller DR, Baehner RL, McMillan CW, eds. Blood diseases
The Journal of Pediatrics August 1988
of infancy and childhood. 5th ed. St. Louis: CV Mosby, 1984;858-921. 13. Schmidt B, Zipursky A. Disseminated intravascular coagulation masking neonatal hemophilia. J PEDIATR 1986;109: 886-8. 14. Buchanan GR. Hemorrhagic diseases. In: Nathan DG, Oski FA, eds. Hematology of infancy and childhood. 3rd ed. Philadelphia: WB Saunders, 1987:104-27. 15. Andrew M, Paes B, Milner R, et al. Development of the human coagulation system in the full-term infant. Blood 1987;70:165-72.
Perforation of the scrotum complicating nephrotic
syndrome Thomas R. Welch, MD, John Gianis, MD, a n d Curtis A. Sheldon, MD From the Departments of Pediatrics and Surgery (Urology), University of Cincinnati, and the Divisions of Nephrology and Pediatric Urology, Children's Hospital Medical Center and Children's Hospital Research Foundation, Cincinnati, Ohio
Scrotal e d e m a often accompanies the a n a s a r c a of childhood nephrotic syndrome. A l t h o u g h this feature is often dramatic, it rarely presents a significant problem. W e report on a child in whom nephrotic syndrome was accompanied by the complication of spontaneous scrotal rupture.
CASE R E P O R T A 2-year-old boy was well until 19 months of age, when he developed nephrotic syndrome. He was treated with prednisone, 30 mg/day, for presumed minimal change disease. His nephrosis resolved, and his corticosteroid therapy was changed to an alternate-day regimen. Two recurrences of proteinuria and edema occurred while he was receiving alternate-day corticosteroid therapy, each managed with a brief course of steroids given daily. When the boy was 22 months of age, recurrent proteinuria and edema necessitated a resumption of daily therapy. Nevertheless, he developed progressive anasarca and was admitted to the hospital. Examination at that time showed an acutely distressed boy whose weight of 15.5 kg represented a recent gain of 3.0 kg. Tense ascites and generalized pitting edema were present. The scrotum was massively enlarged, with a diameter of 12 cm. The child was treated with intravenous albumin and methylprednisolone. His weight increased to 18.4 kg, scrotal swelling progressed, and proteinuria and hypoproteinemia continued. After
Submitted for publication Feb. 19, 1988; accepted Feb. 24, 1988. Reprint requests: Thomas R. Welch, MD, Children's Hospital Research Foundation, Division of Nephrology, Elland and Bethesda Avenues, Cincinnati, OH 45229-2899.
a week without improvement, chlorambucil was added to the regimen. Examination of the child's scrotum revealed a few small areas of what was thought to be a monilial dermatitis, which was treated with topical nystatin. Although no full-thickness cutaneous injury was present, resolution of the dermatitis left an area of epidermal thinning. After 6 more days, the patient's proteinuria decreased and a diuresis began. Coincident with his improvementl the child began walking for the first time since admission to the hospital. On the day that discharge was planned, he fell while walking and was noted to have a large amount of fluid leaking from his diaper. Examination at that time revealed a 2 cm scrotal laceration. Urologic consultation was obtained, and the child was taken to the operating room for exploration and repair of the wound. The entire wound was excised, irrigated copiously with an antimicrobial solution, and closed in layers. Antibiotics for the treatment of potential infection with hospital-acquired pathogens were administered. The child's nephrotic syndrome continues to be in remission, and his scrotal wound has completely healed. DISCUSSION Scrotal swelling has been recognized as a feature of childhood nephrosis since the early descriptions of the condition. 1 This fluid accumulation sometimes represents hydrocele or c o m m u n i c a t i o n with the fluid-filled peritoneu m t h r o u g h a patent processus vaginalis. M o r e often, as in our patient, tense ascites impairs venous a n d lymphatic r e t u r n from the scrotum, producing lymphedema. T h e degree of scrotal e n l a r g e m e n t can be massive, especially in children with a large a m o u n t of ascites. A l t h o u g h older
Volume t 13 Number 2
patients often complain of some discomfort with walking, it has been our experience that significant complaints are rare. The scrotum in these children may be huge and the skin extraordinarily thin, but we had never seen a patient develop an injury such as the one reported here. The major current pediatric nephrology texts 2"5 do not mention this complication, and we could find no similar published reports. The principles of surgical management include preoperative examination and intraoperative inspection to ensure that the massive fluid seepage does not represent leakage of ascitic fluid from a ruptured communicating hydrocele. In this instance, there was massive soft-tissue edema of the scrotum without evidence of scrotal or inguinal mass by history or examination. The absence of ascitic drainage was further confirmed by careful scrotal exploration during which an intact tunica vaginalis was found. Physicians caring for children with nephrotic syndrome should be aware of this potential, albeit rare, complication. Presumably, toddlers whose gait is still somewhat immature would be more likelty to develop such an injury than
Clinical and laboratory observations
337
would older children. Furthermore, the presence of a seemingly trivial dermatitis, as in our patient, may further compromise the integrity of the already-attenuated epidermis. Enforced bed rest is i m p r a c t i c a l - - a n d perhaps even ill advised in nephrotic syndrome unless dermatitis or focal attenuation is encountered. Perhaps, however, children with this condition would benefit from bulky support in the form of heavy diapers, until scrotal swelling lessens. REFERENCES
1. Ratehford BK. Diseases of children. New York: D Appleton, 1912:576. 2. Royer P, Habib R, Mathieu H, Buoyer M. Pediatric nephrology. Philadelphia: WB Saunders, 1974:258-71. 3. Arneil GC. Management of the nephrotic syndrome in the child. In" Liebermann E, ed. Clinical pediatric nephrology. Philadelphia: JB Lippincott, 1976:146-72. 4. Barnett HL, Schoeneman M, Bernstein J, Edelmann C M . The nephrotic syndrome. In: Edelmann CM, ed. Pediatric kidney disease. Boston: Little, Brown, 1978:679-95. 5. Vernier RL. Primary (idiopathic) nephrotic sy+~:lrome. In: Holliday MA, Banatt TM, Vernier RL, eds. Pediatric nephrology. Baltimore: Williams & Wilkins, 1987:445-56.
Occurrence of prolactinoma after estrogen treatment in a girl with constitutional tall stature E. Panteon, MD, E. Loumaye, MD, M. Maes, MD, a n d P. Malvaux, MD From the Departments of Pediatrics, and Gynecology and Obstetrics, Cliniques Universitaires Saint-Luc, Universit~ Catholique de Louvain, Brussels, Belgium
Galactorrhea is an infrequent complication of estrogen therapy for constitutional tall stature in girls. ~'2 The following case history indicates that high-dose estrogen administration in some tall girls may be associated with prolactinoma. CASE R E P O R T S A 12~f2-year-oldgirl was referred to our clinic for evaluation of tall stature. Her parents were tall: the father 192.6 era, and the mother 189.1 cm. Although the girls did not appear to have major psychologic problems, the mother was concerned that she wmild have difficulties in relating to peers and in engaging in professional iife, as had the mother. Her height was 171.5 era (above the 97th percentile on the Tanner growth curves), and her weight 44.8
Submitted for publicationSept. 14, 1987; accepted March 22, 1988. Reprint requests: E. Panteon, MD, Department of Pediatrics, Cliniques Universitaires Saint-Luc, Universite Catholique de Louvain, Brussels, Belgium.
kg (75th percentile). She was in early puberty, according to the Tanner pubertal ratings, with onset of breast development (B2) and of pubic and axillary hair (P2, A2). Visual fields were normal by frontal confrontation. Findings of physical examination were normal. Her bone age was 11)/2 years according to the Greulich and Pyle atlas, and her predicted mature height was estimated at 192.5 cm, using the Bayley and Pinneau tables. Because of normal history and physical findings and her parents' heights, she was considered to be constitutionally tall. Because of the consequences of her tallness on her present and future psychologic well-being, the patient and her parents agreed to a reduction of final height. She was given 300 t~g ethinyt estradiol daily, and 10 mg lynestrenol per day, seven days a month. Before onset of therapy serum prolactin, measured by radioimmunoassay, was 1.7 ng/ml (normal <20 ng/ml). The hormonal treatment was welt tolerated, with expected decelerated growth velocity and'accelerated pubertal development and bone maturation (Table). Therapy was stopped after 25 months, when the patients was I4y2 years of age and her bone age had reached 16 years.