|Year : 2022 | Volume
| Issue : 6 | Page : 960-963
Hip dislocation based on Developmental Dysplasia of the Hip (DDH) in a patient with Giant Congenital Melanocytic Nevus (GCMN): A rare coexistence
M Kaya, HH Muratli
Department of Orthopedics and Traumatology, Marmara University Pendik Training and Research Hospital, Istanbul, Turkey
|Date of Submission||31-Jul-2021|
|Date of Acceptance||12-Jan-2022|
|Date of Web Publication||16-Jun-2022|
Dr. M Kaya
Fevzi Çakmak, Muhsin Yazıcıoğlu Cd No: 10, Marmara University Pendik Training and Research Hospital, Department of Orthopedics and Traumatology, 34899 Pendik, İstanbul
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Giant congenital melanocytic nevus (GCMN) is a rare congenital benign neoplasm with the potential to become malignant. Various extracutaneous malformations may accompany GCMN. Acetabular dysplasia accompanying GCMN was described in previous studies. To the best of our knowledge, this case report of ours is the first reported case of GCMN diagnosed and treated with hip dislocation based on developmental dysplasia of the hip (DDH). The rare coexistence prompted us to report this case.
Keywords: Developmental dysplasia, extracutaneous malformation, giant melanocytic nevus, hip dislocation, rare congenital benign neoplasm
|How to cite this article:|
Kaya M, Muratli H H. Hip dislocation based on Developmental Dysplasia of the Hip (DDH) in a patient with Giant Congenital Melanocytic Nevus (GCMN): A rare coexistence. Niger J Clin Pract 2022;25:960-3
|How to cite this URL:|
Kaya M, Muratli H H. Hip dislocation based on Developmental Dysplasia of the Hip (DDH) in a patient with Giant Congenital Melanocytic Nevus (GCMN): A rare coexistence. Niger J Clin Pract [serial online] 2022 [cited 2022 Aug 8];25:960-3. Available from: https://www.njcponline.com/text.asp?2022/25/6/960/347604
| What is New?|| |
Unilateral hip dislocation, acetabular dysplasia, and extremity atrophy coexistence based on developmental dysplasia of the hip (DDH) were demonstrated in a patient who had a giant congenital melanocytic nevus (GCMN).
| Introduction|| |
Melanocytic nevi are benign proliferations of melanocytic cells clustered in the epidermis, dermis, or other tissues. The incidence of congenital melanocytic nevi is estimated to be 1% in newborns, the frequency of GCMN ranges between 1/20000 and 1/50000, and is much lower. The size of these nevi determines whether they are in the definition of GCMN; nevi at least 6 cm on the body and at least 9 cm on the head in the newborn are referred to as “giant” congenital melanocytic nevi.
However, DDH is the mismatch between the femoral head and the acetabulum, which appears before us in a varying range from irreducible dislocation to mild dysplasia. The worldwide incidence of DDHs ranges between 1 and 34 cases per 1000 births. The coexistence of GCMN and bilateral acetabulum dysplasia based on DDH was emphasized previously. However, the coexistence of hip dislocation that requires surgical treatment in DDH and GCMN was not reported before.
Our case is a new coexistence in which we treated the patient with GCMN for hip dislocation that developed based on DDH.
| Case Report|| |
A 4-year-old girl was referred to our tertiary care hospital due to right hip dislocation based on DDH. The patient presented to us with shortness in the right lower extremity, limping, and a giant nevus that covered the entire right lower extremity, including the hip. The patient had healthy and unrelated parents. The mother had a history of cesarean section (C/S) in her first pregnancy because of first live birth, preterm labor, and low birth weight. The family had no history of nevus and DDH. She was diagnosed as GCMN by the dermatology unit at birth and was then followed up. The nevus showed growth that was proportional to child growth development, but there were no changes that suggested malignant transformation such as rapid growth, ulceration, bleeding, and hardening. In newborn hip ultrasound imaging scans, the left hip was evaluated as type 1 (healthy hip joint) and the right hip as type 2b (dysplastic hip joint) according to Graf, but the treatment remained unfollowed. Conservative treatment, including plaster, was applied to the patient who developed a fracture of the right femur on the same side as the nevus and hip dislocation after falling at the age of 22 months, and the bone union was achieved. In current radiographs, increased valgus was detected in the right proximal femur compared to the left, and the acetabular index measurements were 31° on the right and 20° on the left. According to the Tonnis classification of DDH, the right hip was evaluated as stage 4 dislocation [Figure 1]. After consulting the child's parents, it was decided that surgical intervention would be a good option.
|Figure 1: Right hip dislocation and acetabular dysplasia of the patient in the anteroposterior (AP) pelvic X-ray|
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In the examination for her hip, there were limping gait and a positive Trendelenburg finding. There was limited abduction limitation (20°), right thigh atrophy (2 cm), and shortness in the right lower extremity (2 cm) in the right hip when compared to the left hip. Systemic examination revealed unremarkable results. The mucosa, hair, nails, palms, and soles of the patient were normal. No abnormalities were detected in the ophthalmological and neurological examination. In skin examination, the child had a flat melanocytic nevus with good borders, marginal satellite nevi in brown and black, covering the entire right lower extremity, hip, and lumbar region [Figure 2].
|Figure 2: Anteroposterior images of the patient's giant congenital melanocytic nevus|
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Preoperative plan and osteotomy
Early diagnosis is important in treating DDH, and conservative treatment is used with a Pavlik harness in children younger than 6 months. Surgical intervention (including closed or open reduction with/without pelvic osteotomy) and casting may be an option in late admissions. After clinical and radiological examinations of the patient, right femur proximal shortening-varization-derotation osteotomy, open reduction-capsulorrhaphy, and Salter's osteotomy were planned to provide femoral head coverage.
A lateral approach was employed for femoral shortening-varization-derotation osteotomy and fixation. Open reduction-capsulorrhaphy and anterior approaches were used for Salter's osteotomy. The amount of correction was determined according to the hip's Peri-operative clinical and fluoroscopic evaluation after the osteotomy. After the incisions were closed, pelvipedal plastering was performed under fluoroscopic image confirmation, and the surgery was completed. Concentric reduction and acetabular correction were confirmed with the radiographs obtained when the patient was still in postoperative pelvipedal plaster [Figure 3].
|Figure 3: Proximal Femoral Osteotomy-Fixation and Salter Osteotomy-Fixation images in the postoperative pelvic AP radiograph of the patient|
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Postoperative follow-up and rehabilitation
The patient was seen in the clinic in the third and sixth weeks for pelvic plaster hygiene and checked for the concentric reduction with radiography. The plaster was terminated after the sixth week, and Dennis-Brown (DB) abduction orthosis was used. The DB abduction orthosis was used for 6 weeks full time and 6 weeks at night. The sixth postoperative month follow-up radiograph is shown in [Figure 4].
|Figure 4: The sixth postoperative month follow-up of AP pelvic radiograph|
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| Discussion|| |
GCMN is a type of giant-size nevus that is present at birth. Various abnormalities associated with GCMN were reported in previous studies. Documented coexisting cases include neurocutaneous melanocytosis, spina bifida occulta, meningocele, pes equinovarus, neurofibromatosis, lipomatosis, and hypertrophy and atrophy visible in the limbs. Only two cases that had upper or lower extremity atrophy without DDH in GCMN were reported previously., Imchen et al. reported a case of bilateral DDH that accompanied lower extremity asymmetry in a 7-month-old boy with GCMN. Our case is a first coexistence case with the asymmetry in the lower extremity where the nevus is intense and hip dislocation based on the DDH on the same side.
All newborns should be screened for DDH with a neonatal hip scan and physical examination. If physical examination results are suspicious or an associated risk factor, the diagnosis must be confirmed or excluded with ultrasonography in children younger than 3 months and pelvic radiographs in older children. The target of DDH treatment is to achieve and maintain a safe concentric reduction of the hip to provide an optimal setting for the femur head and acetabulum development and to avoid avascular necrosis (AVN), and also to reduce the risk of early osteoarthrosis. Although Graf type 2b hip dysplasia was detected in the right hip in the USI (Ultrasound Imaging) in our case, the patient remained unfollowed in terms of treatment. The presence of right hip Tonnis type 4 dislocation and the right acetabular dysplasia (i.e., acetabular index right 31°, left 20°) at the time of admission guided us to pelvic and femoral osteotomy, open reduction, and pelvipedal plaster treatment. We achieved concentric reduction after the treatment and provided adequate hip coverage for the patient. We did not detect any problems regarding the use of plaster and brace in the postoperative follow-ups.
Although newborn DDH screening is recommended routinely, the suspicion must be considered knowing that it is highly likely to accompany existing clinical and syndromic conditions in the baby. DDH, which is a condition in which adequate treatment will be provided by bandaging with early diagnosis, is not a desired situation to end up in surgical treatment as in our case, and the family must be informed. Compliance should be provided well in the treatment follow-ups after the diagnosis. We recommend close multidisciplinary follow-ups for malignant transformation of nevi, treatment, and follow-up of recurrence of dilated cardiomyopathy (DCM) in patients with GCMN and DDH coexistence.
| Conclusion|| |
We detected unilateral hip dislocation, acetabular dysplasia, and extremity atrophy based on DDH in our case who had GCMN and provided surgical treatment for hip dislocation. For this reason, we believe that our results can contribute to the literature of a largely unknown field.
Approval is not necessary for a case report in our locality.
Consent was taken from the patient and the family of the patient.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Arneja JS, Gosain AK. Giant congenital melanocytic nevi. Plast Reconstr Surg 2009l; 124:1e-13e.
Castilla EE, Dutra MDG, Orioli-Parreiras IM. Epidemiology of congenital pigmented naevi: I. Incidence rates and relative frequencies. Br J Dermatol 1981;104:307-15.
Graf R. Hip sonography: 20 years experience and results. Hip Int 2007;17(Suppl 5):S8-14.
Imchen S, Ghosh S, Dayal S, Marwah N, Jindal N, Sangal S. Giant congenital melanocytic nevus with developmental dysplasia of bilateral hip: A rare association. Indian J Dermatol 2013;58:475-7.
] [Full text]
Itin PH, Lautenschlager S. Lower and upper extremity atrophy associated with a giant congenital melanocytic nevus. Pediatr Dermatol 1998;15:287-9.
Lyon VB. Congenital melanocytic nevi. Pediatr Clin North Am 2010;57:1155-76.
Klisic PJ. Congenital dislocation of the hip--A misleading term: Brief report. J Bone Joint Surg Br 1989;71:136.
Noordin S, Umer M, Hafeez K, Nawaz H. Developmental dysplasia of the hip. Orthop Rev 2010;2:e19.
Sewell MD, Eastwood DM. Screening and treatment in developmental dysplasia of the hip-where do we go from here? Int Orthop 2011;35:1359-67.
Skidmore RA, Ivker RA, Resnick SD. Upper extremity atrophy associated with a giant congenital melanocytic nevus. Pediatr Dermatol 1995;12:272-4.
Tannous ZS, Mihm MC, Sober AJ, Duncan LM. Congenital melanocytic nevi: Clinical and histopathologic features, risk of melanoma, and clinical management. J Am Acad Dermatol 2005;52:197-203.
Terjesen T. Residual hip dysplasia as a risk factor for osteoarthritis in 45 years follow-up of late-detected hip dislocation. J Child Orthop 2011;5:425-31.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]