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Congenital infiltrating lipomatosis of the face related to cytomegalovirus infection
British Journal o/Plastic Surgery ( 1990). 43, I24- I26 0 1990 The Trusteesof British Association of Plastx Surgeons
Case Report Congenital infiltrating lipomatosis of the face related to cytomegalovirus infection L. DONATI,
P. CANDIANI,
S. GRAPPOLINI,
M. KLINGER and M. SIGNORINI
Department of Plastic Surgery, University of Milan Medical School, Italy
Summary-A case of congenital infiltrating congenital cytomegalovirus infection.
lipomatosis
Case report A male child, G.L., presented at birth with a significant subcutaneous mass in the left cheek, producing marked facial asymmetry. There were no relevant episodes during the pregnancy, such as infectious disease, drug abuse or exposure to teratogenic agents. At birth, mild jaundice and slight hepatomegaly were noted. Laboratory data were as follows: total bilirubin 4.56 mg% (direct 1.95, indirect 2.61), SGOT 65 W/l, SGPT 28 IUjl, gamma GT 125 IUjl, alkaline phosphatase 124 IUjl. The clinical and laboratory abnormalities regressed spontaneously to normal by 3 months. The lesion was covered by skin of normal colour and texture; it waselastic, non-tender, with ill-defined borders (Fig. l), and there were no murmurs or palpable thrills. The tumour steadily enlarged and at the age of 4 months the patient was admitted to the paediatric division of our hospital following convulsions. EEG revealed slow and widespread high voltage anomalies of the left hemisphere, of epileptic pattern. These findings and the symptoms improved after the start of anticonvulsant drug treatment. X-rays of the skull revealed asymmetry of the cranial vault which presented greater convexity on the left. No signs of intracranial hypertension or pathological calcification were detected. CT scans (Fig. 2) confirmed that the left cerebral hemisphere was significantly larger than the right, associated with overdevelopment of the left hemicranial vault. The left lateral ventricle was enlarged. A generalised hypodensity of the white matter of the right hemisphere was detected. Also evident was the asymmetry of the soft tissues of the face but no abnormalities were seen within them. A biopsy of the subcutaneous mass reported the presence of normal adipose tissue only. Because of the increase in facial asymmetry, surgery was carried out at the age of 5 months. Through a rhytidectomy incision and with the help of an electrostimulator to preserve the facial nerve, the non-encapsulated adipose mass was excised as radically as possible. 124
of the face is presented,
associated
with a
Histological examination (Fig. 3) revealed the presence of parotid gland tissue, and large epithelial cells with intracellular and intranuclear inclusions typical of cytomegalovirus infection were present in the intralobular ducts. A search for specific anti-CMV antibodies (IgG and IgM) was also positive. After surgery the residual mass showed no tendency to recur but the patient failed to thrive and died at the age of 20 months of a pulmonary infection.
Fig. Figurel-The
I
patient aged 3 months
125
CASE REPORT
Discussion
Fig. 2 Figure 2--Cranial CT scan. aged 3 months. asymmetry and the enlarged left ventricle
Note
the brain
Infiltrating congenital lipomatosis of the face is a rare but well defined clinical entity (Slavin et al., 1983; Baker, 1984). More frequently, it first appears later in life and in other parts of the body [80x of lipomatoses affect the lower limbs (Regan et al., 1946; Gonzale-Crussi et al., 1966)]. Lipomatosis of the face is localised in the cheek and physical examination reveals a poorly defined elastic tumour that may reach a remarkable size, with overlying skin of normal colour and texture. Microscopic examination reveals non-capsulated fatty tissue formed by mature adipocytes infiltrating the neighbouring structures, such as peripheral nerves, salivary glands (parotid), muscles and vessels, thus minimising the possibility of a radical excision. The presence of mature adipocytes and the absence of lipoblastic proliferation, pleomorph-
Fig. 3 Figure 3-Histological sections of part of the excised specimen, & E x 37). (B) Higher magnification; note the parotid glandular bodies (H & E x 300).
(A) Low power view showing adipose cells and glandular tissue (H tissue with typical intranuclear and cytoplasmatic CMV inclusion
126 ism and mitoses are extremely important for the differential diagnosis from other lipomatous masses (Hoehn et al., 1984) in particular liposarcoma (Wurlitzer et al., 1973; Dionne and Seemayer, 1974). Although they are benign, treatment of these neoplasms is difficult as radical excision often implies the sacrifice of important anatomical structures. This explains the high recurrence rates, estimated at 50-60x (Dionne and Seemayer, 1974). Congenital CMV infection is transmitted transplacentally. It is a common disease [about 2% of newborns are immunologically positive (Krugman and Katz, 1981)], but fortunately in only 5% of positive subjects do clinically evident lesions arise. Symptoms frequently do not arise until several months after birth; these can vary to a great degree and can involve any anatomical area. However, the central nervous system is by far the most frequent target, with microcephaly, enlarged ventricles, convulsions, spasticity etc. Other clinical findings such as hepatosplenomegaly, jaundice, chorioretinitis, coagulation defects and skin rashes are common. Localisation in the salivary glands (parotid in particular) is typical. In such cases intranuclear and cytoplasmatic inclusion bodies, which can at times be identified in other parts of the body also, are diagnostic. In this case report most of the typical symptoms of a congenital CMV infection were present : jaundice, hepatomegaly, complex neurological alterations, and death following a respiratory infection (these patients are highly susceptible to infections). The diagnosis was confirmed by histology and CMV positive serology. Hanshaw (1970) has listed a large series of abnormalities associated with CMV congenital infection, among which lipomas are included. However, we have not traced reports in the literature relating a congenital infiltrating lipomatosis of the face to a CMV infection. The finding of glandular tissue within the lipomatosis, with the typical CMV inclusions, confirmed involvement of the region with the viral
BRITISH JOURNAL
OF PLASTIC SURGERY
infection but we cannot say whether there was a causal relationship between the two conditions. References Baker, D. C. (1984). $oft tissue tumors of the head and neck. In Serafin D. and Georgiade, N. G. Pediatric Plastic Surgery. St Louis: C. V. Mosby Co., p. 674. Diotme, G. P. and Seemayer, T. A. (1974). Infiltrating lipomas and angiolipomas revisited. Cancer, 33,732.
Gonzale-Crussi, F., Kurteking, W. F. and Arean, V. M. (1966). Infiltrating
angiolipoma.
Journal of Bone and Joint Surgery,
4SA, 1111. Hat&raw, J. B. (1970). Developmental with congenital tology, 4,64.
cytomegalovirus
abnormalities associated infection. Advances in Tera-
Hoeku, J. G., Yalamancki, B. A. and Pilon, V. (1984). Benign lipoblastomatosis: Report of a case involving the face and neck. Plastic and Reconstructive Surgery, 73,455.
Krugman, S. and Katz, S. L. (1981). Cytomegalovirus infections. In Infectious Diseases of Children. St Louis: C. V. Mosby Co., p. 1. Regan, J. M., Buckel, W. H. and Brokers, A. C. (1946). Infiltrating benign lipomas of the extremities. Western Journal of Surgery,Obstetrics and Gynecology, J4,87.
Slaviu, S. A., Baker, D. C., McCarthy, J. G. and Mufarrij, A. (1983). Congenital infiltrating lipomatosis of the face: clinicopathologic evaluation and treatment. Plasticand Reconstructive Surgery, 12, 158. Wurlitzer, F., Bedrosaian, C., Ayala, A. and McBride, C. (1973). Problems of diagnosing and treating infiltrating hpomas. American Surgeon, 39,240.
The Authors L. Donati, MD, Professor and Head of Department P. Candiaui, MD, Associate Professor S. Grappoiiai, MD, Clinical Assistant M. Klinger, MD, Clinical Assistant M. Sigaoriui, MD, Senior Registrar Department of Plastic School, Milan, Italy.
Surgery,
University
of Milan
Medical
Requests for reprints to: Professor L. Donati, Cattedra di Chirurgia Plastica Ricostruttiva, Universita degli Studi di Milano,Ospedale NiguardaCa’Granda P.zaOspedale Maggiore 3.20100 - Milano, Italy. Paper received 2 June 1988. Accepted 9 March 1989 after revision.
Case Report Congenital infiltrating lipomatosis of the face related to cytomegalovirus infection L. DONATI,
P. CANDIANI,
S. GRAPPOLINI,
M. KLINGER and M. SIGNORINI
Department of Plastic Surgery, University of Milan Medical School, Italy
Summary-A case of congenital infiltrating congenital cytomegalovirus infection.
lipomatosis
Case report A male child, G.L., presented at birth with a significant subcutaneous mass in the left cheek, producing marked facial asymmetry. There were no relevant episodes during the pregnancy, such as infectious disease, drug abuse or exposure to teratogenic agents. At birth, mild jaundice and slight hepatomegaly were noted. Laboratory data were as follows: total bilirubin 4.56 mg% (direct 1.95, indirect 2.61), SGOT 65 W/l, SGPT 28 IUjl, gamma GT 125 IUjl, alkaline phosphatase 124 IUjl. The clinical and laboratory abnormalities regressed spontaneously to normal by 3 months. The lesion was covered by skin of normal colour and texture; it waselastic, non-tender, with ill-defined borders (Fig. l), and there were no murmurs or palpable thrills. The tumour steadily enlarged and at the age of 4 months the patient was admitted to the paediatric division of our hospital following convulsions. EEG revealed slow and widespread high voltage anomalies of the left hemisphere, of epileptic pattern. These findings and the symptoms improved after the start of anticonvulsant drug treatment. X-rays of the skull revealed asymmetry of the cranial vault which presented greater convexity on the left. No signs of intracranial hypertension or pathological calcification were detected. CT scans (Fig. 2) confirmed that the left cerebral hemisphere was significantly larger than the right, associated with overdevelopment of the left hemicranial vault. The left lateral ventricle was enlarged. A generalised hypodensity of the white matter of the right hemisphere was detected. Also evident was the asymmetry of the soft tissues of the face but no abnormalities were seen within them. A biopsy of the subcutaneous mass reported the presence of normal adipose tissue only. Because of the increase in facial asymmetry, surgery was carried out at the age of 5 months. Through a rhytidectomy incision and with the help of an electrostimulator to preserve the facial nerve, the non-encapsulated adipose mass was excised as radically as possible. 124
of the face is presented,
associated
with a
Histological examination (Fig. 3) revealed the presence of parotid gland tissue, and large epithelial cells with intracellular and intranuclear inclusions typical of cytomegalovirus infection were present in the intralobular ducts. A search for specific anti-CMV antibodies (IgG and IgM) was also positive. After surgery the residual mass showed no tendency to recur but the patient failed to thrive and died at the age of 20 months of a pulmonary infection.
Fig. Figurel-The
I
patient aged 3 months
125
CASE REPORT
Discussion
Fig. 2 Figure 2--Cranial CT scan. aged 3 months. asymmetry and the enlarged left ventricle
Note
the brain
Infiltrating congenital lipomatosis of the face is a rare but well defined clinical entity (Slavin et al., 1983; Baker, 1984). More frequently, it first appears later in life and in other parts of the body [80x of lipomatoses affect the lower limbs (Regan et al., 1946; Gonzale-Crussi et al., 1966)]. Lipomatosis of the face is localised in the cheek and physical examination reveals a poorly defined elastic tumour that may reach a remarkable size, with overlying skin of normal colour and texture. Microscopic examination reveals non-capsulated fatty tissue formed by mature adipocytes infiltrating the neighbouring structures, such as peripheral nerves, salivary glands (parotid), muscles and vessels, thus minimising the possibility of a radical excision. The presence of mature adipocytes and the absence of lipoblastic proliferation, pleomorph-
Fig. 3 Figure 3-Histological sections of part of the excised specimen, & E x 37). (B) Higher magnification; note the parotid glandular bodies (H & E x 300).
(A) Low power view showing adipose cells and glandular tissue (H tissue with typical intranuclear and cytoplasmatic CMV inclusion
126 ism and mitoses are extremely important for the differential diagnosis from other lipomatous masses (Hoehn et al., 1984) in particular liposarcoma (Wurlitzer et al., 1973; Dionne and Seemayer, 1974). Although they are benign, treatment of these neoplasms is difficult as radical excision often implies the sacrifice of important anatomical structures. This explains the high recurrence rates, estimated at 50-60x (Dionne and Seemayer, 1974). Congenital CMV infection is transmitted transplacentally. It is a common disease [about 2% of newborns are immunologically positive (Krugman and Katz, 1981)], but fortunately in only 5% of positive subjects do clinically evident lesions arise. Symptoms frequently do not arise until several months after birth; these can vary to a great degree and can involve any anatomical area. However, the central nervous system is by far the most frequent target, with microcephaly, enlarged ventricles, convulsions, spasticity etc. Other clinical findings such as hepatosplenomegaly, jaundice, chorioretinitis, coagulation defects and skin rashes are common. Localisation in the salivary glands (parotid in particular) is typical. In such cases intranuclear and cytoplasmatic inclusion bodies, which can at times be identified in other parts of the body also, are diagnostic. In this case report most of the typical symptoms of a congenital CMV infection were present : jaundice, hepatomegaly, complex neurological alterations, and death following a respiratory infection (these patients are highly susceptible to infections). The diagnosis was confirmed by histology and CMV positive serology. Hanshaw (1970) has listed a large series of abnormalities associated with CMV congenital infection, among which lipomas are included. However, we have not traced reports in the literature relating a congenital infiltrating lipomatosis of the face to a CMV infection. The finding of glandular tissue within the lipomatosis, with the typical CMV inclusions, confirmed involvement of the region with the viral
BRITISH JOURNAL
OF PLASTIC SURGERY
infection but we cannot say whether there was a causal relationship between the two conditions. References Baker, D. C. (1984). $oft tissue tumors of the head and neck. In Serafin D. and Georgiade, N. G. Pediatric Plastic Surgery. St Louis: C. V. Mosby Co., p. 674. Diotme, G. P. and Seemayer, T. A. (1974). Infiltrating lipomas and angiolipomas revisited. Cancer, 33,732.
Gonzale-Crussi, F., Kurteking, W. F. and Arean, V. M. (1966). Infiltrating
angiolipoma.
Journal of Bone and Joint Surgery,
4SA, 1111. Hat&raw, J. B. (1970). Developmental with congenital tology, 4,64.
cytomegalovirus
abnormalities associated infection. Advances in Tera-
Hoeku, J. G., Yalamancki, B. A. and Pilon, V. (1984). Benign lipoblastomatosis: Report of a case involving the face and neck. Plastic and Reconstructive Surgery, 73,455.
Krugman, S. and Katz, S. L. (1981). Cytomegalovirus infections. In Infectious Diseases of Children. St Louis: C. V. Mosby Co., p. 1. Regan, J. M., Buckel, W. H. and Brokers, A. C. (1946). Infiltrating benign lipomas of the extremities. Western Journal of Surgery,Obstetrics and Gynecology, J4,87.
Slaviu, S. A., Baker, D. C., McCarthy, J. G. and Mufarrij, A. (1983). Congenital infiltrating lipomatosis of the face: clinicopathologic evaluation and treatment. Plasticand Reconstructive Surgery, 12, 158. Wurlitzer, F., Bedrosaian, C., Ayala, A. and McBride, C. (1973). Problems of diagnosing and treating infiltrating hpomas. American Surgeon, 39,240.
The Authors L. Donati, MD, Professor and Head of Department P. Candiaui, MD, Associate Professor S. Grappoiiai, MD, Clinical Assistant M. Klinger, MD, Clinical Assistant M. Sigaoriui, MD, Senior Registrar Department of Plastic School, Milan, Italy.
Surgery,
University
of Milan
Medical
Requests for reprints to: Professor L. Donati, Cattedra di Chirurgia Plastica Ricostruttiva, Universita degli Studi di Milano,Ospedale NiguardaCa’Granda P.zaOspedale Maggiore 3.20100 - Milano, Italy. Paper received 2 June 1988. Accepted 9 March 1989 after revision.