Journal of Pediatric Surgery DECEMBER 1990
VOL 25, NO 12
Aplasia Cutis Congenita of the Scalp By Larry A. Sargent Chattanooga.
Tennessee
0 Aplasia cutis congenita is the congenital absence of skin. The majority of these defects involve the vertex of the scalp in the area overlying the sagittal sinus. The larger defects are predisposed to sudden lethal hemorrhage and require urgent closure. Local rotational scalp flaps are recommended for closure of the larger defects because they provide the most reliable coverage, eliminate the risk of infection and/or hemorrhage, and avoid the alopecia of secondarily healed wounds. 0 1990 by W.B. Saunders Company. INDEX WORDS: defect.
Aplasia
cutis congenita:
congenital
scalp
T
HE CONGENITAL absence of skin is a rare lesion that can occur anywhere on the body. These lesions are referred to as aplasia cutis congenita, and predominantly involve the vertex of the scalp. Most of these congenital wounds are small, superficial to the skull, and heal with local wound care. Larger defects, particularly those over the sagittal sinus, are predisposed to sudden lethal hemorrhage, and several authors have reported a 20% perinatal mortality, related to precipitous exsanguinating hemorrhage from the sagittal sinus. l-3 Early definitive closure of defects over the sagittal sinus might reduce these risks. Presented is the case report of a congenital scalp and bone defect overlying the sagittal sinus, which was successfully closed with rotational scalp flaps. CASE REPORT This baby girl was referred at 3 weeks of age with a defect on the vertex of the scalp with no underlying bone. There was a 2 x 3.5 cm elliptical necrotic eschar surrounded by a 2-cm irregular margin of very thin scalp that appeared to represent recent epithelialization (Fig 1). The sagittal sinus could be seen through a thin, shiny translucent membrane in the center of the necrotic eschar. The underlying skull defect palpated to be 2 cm larger around the periphery than the scalp defect. The urgent operative management consisted of excision of the eschar, as well as the 2-cm surrounding border of abnormal scalp tissue. The 3.5 x 5.5 cm defect was closed with two rotational scalp flaps without the use of skin grafts (Fig 2). The postoperative course was uneventful. The scalp flaps healed, providing good skin coverage over the defect. At 6 months the
Fig 1. Preoperative photograph of newborn with a 2 x 3.6 cm scalp defect on the vertex of the skull. Note necrotic eschar overlying sagittal sinus.
reconstructed scalp defect remains well healed with a good cosmetic result (Fig 3). The bony defect is slowly decreasing in size. DISCUSSION
In 1826, Campbell4 was the first to report two cases of congenital cutis aplasia of the scalp. His first patient died of exsanguinating hemorrhage from the sagittal sinus. Since then there have been fewer than 300 cases reported in the literature. In 1933, Ingalls’ review of 60 cases showed that over 80% of the lesions are close to midline near the vertexe3 The majority of these lesions are single and less than 2 cm in diameter.
From The Tennessee Craniofacial Center, and the Department of PlasticSurgery. Chattanooga Unit, University of Tennessee College of Medicine, Chattanooga, TN. Address reprint requests to Larry A. Sargent, MD. Director, The Tennessee Craniofacial Center, Suite 900, Medical Center Plaza North, 979 E Third St, Chattanooga, TN 37403. o 1990 by W.B. Saunders Company. 0022-3468/90/2512-0001%03.00/0
Journal of Pediatric Surgery, Vol 25, No 12 [December), 1990: pp 12 1 l-1 2 13
1211
LARRY A. SARGENT
Fig 2. (A) lntraoperative view after debridement of eschar and abnormal scalp. Note in center translucent membrane overlying sagittal sinus. Dark lines indicate design of two rotational scalp flaps. (B) Defect reconstructed with two large rotational scalp flaps. No skin graft wss needed.
The pathogenesis of aplasia cutis congenita is not clear; however, past clinical experiences aid in decision making as to the need for surgical repair. Small defects (~2 cm) usually heal spontaneously with dressing changes, but can be closed surgically with local rotational flaps. Surgical intervention has the advantage of avoiding the threat of hemorrhage, infection, and subsequent alopecia from secondary healing. Larger defects, especially those over the sagittal sinus, are at risk for infection and potential lethal hemorrhage.5
For extensive defects overlying the sagittal sinus, most authors recommend urgent surgical intervention,5*6 although cases of successful conservative management have been reported. ’ The greatest risk is during the first month of life as the eschar cracks and starts to separate. Classically several small hemorrhages precede the terminal one. 5*8Because of these potential lethal risks, surgical closure is recommended as early as possible. Surgical reconstruction of these defects has the goal of preventing infection, hemorrhage, and getting the best cosmetic result. Large local scalp flaps have proved to be the most reliable coverage.6V9“’They provide good skin coverage and cosmetically are superior to skin grafts. Local flaps also have the advantage of minimizing alopecia as well as scars that are hidden in hair-bearing scalp. REFERENCES
Fig 3. Postoperative result with good coverage and hair growth 6 months after closure with scalp flaps.
1. Anderson NP, Novy FG Jr: Congenital defects of the scalp. Arch Dermatol Syph 46:257, 1942 2. Demmel U: Clinical aspects of congenital skin defects. I. Congenital skin defects on the head of the newborn. Em J Pediatr 121:21,1975 3. Ingalls NW: Congenital defects of the scalp. III. Studies in the pathology of development. Am J Obstet Gynecol25:861,1933 4. Campbell W: Case of congenital ulcer on the cranium of a fetus, terminating in fatal hemorrhage on the 18th day after birth. J Med Sci (Edinb) 2:82, 1826 5. Peer LA, Duyn JV: Congenital defect of the scalp: Report of a case with fatal termination. Plast Reconstr Surg 3:722, 1948 6. Kosnik EJ, Sayers MP: Congenital scalp defects: Aplasia cutis congenita. J Neurosurg 42:32, 1975
APLASIA
CUTIS CONGENITA
7. Six EG, Kelly DL: Conservative management of aplasia cutis congenita: Case report. Neurosurgery 8:233, 1981 8. Irons GB, Olson RM: Aplasia cutis congenita. Plast Reconstr Surg 66: 199, 1980 9. Farmer AW, Maxmen MD: Congenital absence of skin. Plast Reconstr Surg 25:291, 1960
1213
10. Kahn EA, Olmedo L: Congenital defect of the scalp with a note on the closure of large scalp defects in general. Plast Reconstr Surg 6:435, 1950 11. Lynch PJ, Kahn EA: Congenital defects of the scalp: A surgical approach to aplasia cutis congenita. J Neurosurg 33:198, 1970
VOL 25, NO 12
Aplasia Cutis Congenita of the Scalp By Larry A. Sargent Chattanooga.
Tennessee
0 Aplasia cutis congenita is the congenital absence of skin. The majority of these defects involve the vertex of the scalp in the area overlying the sagittal sinus. The larger defects are predisposed to sudden lethal hemorrhage and require urgent closure. Local rotational scalp flaps are recommended for closure of the larger defects because they provide the most reliable coverage, eliminate the risk of infection and/or hemorrhage, and avoid the alopecia of secondarily healed wounds. 0 1990 by W.B. Saunders Company. INDEX WORDS: defect.
Aplasia
cutis congenita:
congenital
scalp
T
HE CONGENITAL absence of skin is a rare lesion that can occur anywhere on the body. These lesions are referred to as aplasia cutis congenita, and predominantly involve the vertex of the scalp. Most of these congenital wounds are small, superficial to the skull, and heal with local wound care. Larger defects, particularly those over the sagittal sinus, are predisposed to sudden lethal hemorrhage, and several authors have reported a 20% perinatal mortality, related to precipitous exsanguinating hemorrhage from the sagittal sinus. l-3 Early definitive closure of defects over the sagittal sinus might reduce these risks. Presented is the case report of a congenital scalp and bone defect overlying the sagittal sinus, which was successfully closed with rotational scalp flaps. CASE REPORT This baby girl was referred at 3 weeks of age with a defect on the vertex of the scalp with no underlying bone. There was a 2 x 3.5 cm elliptical necrotic eschar surrounded by a 2-cm irregular margin of very thin scalp that appeared to represent recent epithelialization (Fig 1). The sagittal sinus could be seen through a thin, shiny translucent membrane in the center of the necrotic eschar. The underlying skull defect palpated to be 2 cm larger around the periphery than the scalp defect. The urgent operative management consisted of excision of the eschar, as well as the 2-cm surrounding border of abnormal scalp tissue. The 3.5 x 5.5 cm defect was closed with two rotational scalp flaps without the use of skin grafts (Fig 2). The postoperative course was uneventful. The scalp flaps healed, providing good skin coverage over the defect. At 6 months the
Fig 1. Preoperative photograph of newborn with a 2 x 3.6 cm scalp defect on the vertex of the skull. Note necrotic eschar overlying sagittal sinus.
reconstructed scalp defect remains well healed with a good cosmetic result (Fig 3). The bony defect is slowly decreasing in size. DISCUSSION
In 1826, Campbell4 was the first to report two cases of congenital cutis aplasia of the scalp. His first patient died of exsanguinating hemorrhage from the sagittal sinus. Since then there have been fewer than 300 cases reported in the literature. In 1933, Ingalls’ review of 60 cases showed that over 80% of the lesions are close to midline near the vertexe3 The majority of these lesions are single and less than 2 cm in diameter.
From The Tennessee Craniofacial Center, and the Department of PlasticSurgery. Chattanooga Unit, University of Tennessee College of Medicine, Chattanooga, TN. Address reprint requests to Larry A. Sargent, MD. Director, The Tennessee Craniofacial Center, Suite 900, Medical Center Plaza North, 979 E Third St, Chattanooga, TN 37403. o 1990 by W.B. Saunders Company. 0022-3468/90/2512-0001%03.00/0
Journal of Pediatric Surgery, Vol 25, No 12 [December), 1990: pp 12 1 l-1 2 13
1211
LARRY A. SARGENT
Fig 2. (A) lntraoperative view after debridement of eschar and abnormal scalp. Note in center translucent membrane overlying sagittal sinus. Dark lines indicate design of two rotational scalp flaps. (B) Defect reconstructed with two large rotational scalp flaps. No skin graft wss needed.
The pathogenesis of aplasia cutis congenita is not clear; however, past clinical experiences aid in decision making as to the need for surgical repair. Small defects (~2 cm) usually heal spontaneously with dressing changes, but can be closed surgically with local rotational flaps. Surgical intervention has the advantage of avoiding the threat of hemorrhage, infection, and subsequent alopecia from secondary healing. Larger defects, especially those over the sagittal sinus, are at risk for infection and potential lethal hemorrhage.5
For extensive defects overlying the sagittal sinus, most authors recommend urgent surgical intervention,5*6 although cases of successful conservative management have been reported. ’ The greatest risk is during the first month of life as the eschar cracks and starts to separate. Classically several small hemorrhages precede the terminal one. 5*8Because of these potential lethal risks, surgical closure is recommended as early as possible. Surgical reconstruction of these defects has the goal of preventing infection, hemorrhage, and getting the best cosmetic result. Large local scalp flaps have proved to be the most reliable coverage.6V9“’They provide good skin coverage and cosmetically are superior to skin grafts. Local flaps also have the advantage of minimizing alopecia as well as scars that are hidden in hair-bearing scalp. REFERENCES
Fig 3. Postoperative result with good coverage and hair growth 6 months after closure with scalp flaps.
1. Anderson NP, Novy FG Jr: Congenital defects of the scalp. Arch Dermatol Syph 46:257, 1942 2. Demmel U: Clinical aspects of congenital skin defects. I. Congenital skin defects on the head of the newborn. Em J Pediatr 121:21,1975 3. Ingalls NW: Congenital defects of the scalp. III. Studies in the pathology of development. Am J Obstet Gynecol25:861,1933 4. Campbell W: Case of congenital ulcer on the cranium of a fetus, terminating in fatal hemorrhage on the 18th day after birth. J Med Sci (Edinb) 2:82, 1826 5. Peer LA, Duyn JV: Congenital defect of the scalp: Report of a case with fatal termination. Plast Reconstr Surg 3:722, 1948 6. Kosnik EJ, Sayers MP: Congenital scalp defects: Aplasia cutis congenita. J Neurosurg 42:32, 1975
APLASIA
CUTIS CONGENITA
7. Six EG, Kelly DL: Conservative management of aplasia cutis congenita: Case report. Neurosurgery 8:233, 1981 8. Irons GB, Olson RM: Aplasia cutis congenita. Plast Reconstr Surg 66: 199, 1980 9. Farmer AW, Maxmen MD: Congenital absence of skin. Plast Reconstr Surg 25:291, 1960
1213
10. Kahn EA, Olmedo L: Congenital defect of the scalp with a note on the closure of large scalp defects in general. Plast Reconstr Surg 6:435, 1950 11. Lynch PJ, Kahn EA: Congenital defects of the scalp: A surgical approach to aplasia cutis congenita. J Neurosurg 33:198, 1970
