|Year : 2021 | Volume
| Issue : 1 | Page : 76-79
A case of congenital neurovascular hamartoma with midline cleft of the upper lip
Siddhartha Chatterjee, Debolina Pramanick, Vivek R Raythatha
Department of Cleft Lip and Palate Surgery, Shree Jain Hospital and Research Centre, Howrah, West Bengal, India
|Date of Submission||22-Sep-2020|
|Date of Acceptance||24-Nov-2020|
|Date of Web Publication||13-Jan-2021|
Dr. Debolina Pramanick
Keota Brick Field Lane, Sahaganj, Hooghly, West Bengal
Source of Support: None, Conflict of Interest: None
Developmental hamartomas are dysmorphic proliferation of native tissues which unlike neoplasms do not grow continuously and are mostly seen in infancy and childhood. Hamartoma of neurovascular origin can present itself as upper lip malformation. These lesions sometimes regress spontaneously while some require surgical intervention. Here, we report a case of congenital neurovascular hamartoma with midline cleft of the upper lip, tongue tie, and lower lip pits in a 4-month-old baby, managed by surgical intervention.
Keywords: Cleft lip, congenital, lip pits, midline hamartoma, neurovascular hamartoma, tongue tie
|How to cite this article:|
Chatterjee S, Pramanick D, Raythatha VR. A case of congenital neurovascular hamartoma with midline cleft of the upper lip. J Cleft Lip Palate Craniofac Anomal 2021;8:76-9
|How to cite this URL:|
Chatterjee S, Pramanick D, Raythatha VR. A case of congenital neurovascular hamartoma with midline cleft of the upper lip. J Cleft Lip Palate Craniofac Anomal [serial online] 2021 [cited 2021 Apr 11];8:76-9. Available from: https://www.jclpca.org/text.asp?2021/8/1/76/306773
| Introduction|| |
Hamartomas are a dysmorphic proliferation of tissues that are native to the area. Most of the benign tumors of infancy and childhood belong to the developmental hamartoma category. Few common examples are hemangiomas, pigmented nevi, odontomas, ameloblastic fibroodontomas, and squamous odontogenic tumors. As hamartomas never infiltrate surrounding tissues, they do not recur after enucleation (in case of bone) or local pericapsular excisions (in soft tissue).
The report describes a unique clinical presentation of oral neurovascular hamartoma along with midline cleft in the upper lip and tongue tie in an otherwise healthy child. The case is unique because till date we found single reference by Miyamoto Y et al in 1991 who described a case of cleft palate with lobulatedtongue and lingual hamartoma.
| Case Report|| |
A 4-month-old baby girl was brought to the OPD with a history of congenital soft-tissue growth in her maxillary alveolar ridge [Figure 1]. History revealed that she (first gravida) was born full-term by C-section with no history of neonatal intensive care unit stay. The family does not have any history of congenital anomalies. Prenatal ultrasonography at 10 weeks 1 day of average gestational age did not detect any anomaly. The mother did not have any medical condition during her pregnancy.
The patient weighed 5 kg 500 g when we examined her. There were slight head lag and no neck control at 4 months of age.
The parents complained of growth since birth on the upper lip without pain.
On inspection, approximately 4 cm × 4 cm sized reddish-black growth was seen on the premaxilla resulting in midline cleft of the upper lip. There was a similar extent of vermilion and dry mucosa on both sides of the lip and complete loss of philtrum and alar base bilaterally. Columella of the nose was divided into two halves [Figure 2]. The facial surface of the growth contained few hair follicles. On palpation, growth was soft, sessile, and attached to the alveolus of the premaxilla. There was no bleeding or discharge. The auscultation of growth was negative. Two lip pits (750.260) were present on the lower lip. Tongue-tie (#750.000) was also seen.
(A pound symbol [#] beside a disease? code indicates that the condition/defect is listed on the MACDP Exclusion List).
Two-dimensional echocardiography ruled out congenital cardiac deformity. Posteroanterior view chest and routine blood investigation were in the normal range. Computed tomography (CT) scan of the face concluded premaxillary protrusion of abnormal alveolar and gingival soft tissues.
First, growth [Figure 3] was excised using electrocautery [Figure 4]. After hemostasis, the midline cleft lip was closed in three layers using resorbable sutures by straight-line repair technique. The removed specimen was a polypoidal growth measuring 3.0 cm × 2.0 cm × 1.5 cm [Figure 5]. The color was yellowish-gray with a cartilaginous base. It was sent for histopathological examination. The postoperative period was uneventful and the patient was discharged on the 3rd postoperative day. One month follow-up [Figure 6] showed good healing of the wound.
The histopathology report revealed that the lesion was oral neurovascular hamartoma. The growth had a stratified squamous epithelium lining. The stroma contained cell clusters and cell nests with fibrillary cytoplasm, round vesicular nuclei having strong S100 positivity, and hyalinized thick-walled blood vessels. The stroma matrix also showed a fair degree of collagenosis, dispersed adipocyte lobules, smooth and skeletal muscles along with some hyaline cartilage fragments, and some osteoid with bone marrow elements.
| Discussion|| |
Albrecht coined the term “hamartoma” in 1904 to delineate developmental tumor-like malformations.
A hamartoma is a benign tumor-like lesion composed of an overgrowth of mature cells and tissues that normally occur with disorganization and often with one element predominating. Hamartomas may or may not be congenital. A failure in the normal orderly embryonic inductive process has been theorized.
The upper lip and middle portion of the nose are formed by three different embryogenic processes named as a frontonasal process, medial and lateral nasal process, and maxillary process. The medial nasal processes of both sides, together with the frontonasal process, give rise to the middle portion of the nose. The maxillary process grows medially and approaches the lateral and medial nasal processes but remains separated from them by the nasolacrimal groove and the bucconasal groove. Later, the medial nasal process is displaced toward the midline and merges with its anatomic counterpart from the opposite side, thus forming the philtrum as well as premaxilla and primary palate. Fusion occurring between the forward extent of the maxillary process and the lateral aspect of the medial nasal process obliterates the bucconasal groove and results in the formation of the lateral aspects of the upper lip.
These fusions of multiple embryogenic processes can explain the predilection of hamartoma formation in this region.
In this case, the presence of a large hamartoma might have hampered the fusion of two median nasal processes, thus forming a midline cleft. It explains hamartoma was formed much before the formation of the upper lip.
Another site for hamartoma is the tongue which is again a region of fusion of multiple embryonic processes.
They sometimes regress spontaneously. It consists of cytologically normal mature cells, native to the anatomic location. The differential diagnosis includes congenital and infantile haemangioma, vascular malformations, neurofibroma, etc. There are also few syndromes (tuberous sclerosis, Proteus syndrome, and orofacial digital syndrome) associated with hamartomas.
Our patient is nonsyndromic and the hamartoma is of mesenchymal origin as supported by the histopathological report.
CT scan delineates the extension of the lesion in all three planes. It is well circumscribed and noninfiltrative in nature. The treatment plan includes conservative surgical excision only.
There is little literature where patients were treated by photocoagulation using diode laser at 830 nm with a power set at 3.5 W.
The report imparts a key message to the clinicians about an unusual midline growth in the upper lip of a child. A vascular malformation is one of the differential diagnoses; it needs proper clinical examination, preoperative workup to lead the way toward successful management.
The patient consent has been obtained.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Marx RE, Stern D. Oral and maxillofacial pathology: a rationale for diagnosis and treatment. Hanover Park, IL: Quintessence Pub. Co.,; 2012. Page 251.
Centers for Disease Control and Prevention. Birth defects and genetic diseases branch 6-digit code. For reportable congenital anomalies. 1979 British Pediatric Association (BPA) Classification of Diseases and the World Health Organization's 1979 International Classification of Diseases,9th
Revision, Clinical Modification (ICD-9-CM).
Craig O. Hamartoma. Postgrad Med J. 1965;41:636–8.
Nanci A. Ten Cate's oral histology. Development, structure, and function. 2013;7:79-289.
Dal Vechio A, Nakajima E, Pinto D, Azevedo LH, Migliari DA. Rhabdomyomatous (mesenchymal) hamartoma presenting as haemangioma on the upper lip: a case report with immunohistochemical analysis and treatment with high-power lasers. Case reports in dentistry. 2013;2013:943953.
A. Kobayashi, T. Amagasa, N. Okada, Leiomyomatous hamartoma of the tongue:case report, Journal of Oral and Maxillofacial Surgery 59 2001;337–340.
Magro G, Di Benedetto A, Sanges G, Scalisi F, Alaggio R. Rhabdomyomatous mesenchymal hamartoma of oral cavity: an unusual location for such a rare lesion. Virchows Archiv. 2005;446:346-7.
Patil S, Rao RS, Majumdar B. Hamartomas of the oral cavity. Journal of International Society of Preventive & Community Dentistry. 2015;5:347-53.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]