INTRODUCTION
Cleft lip and/or palate (CL/P) is among the most common congenital anomalies. Approximately 70% of CL/P cases are nonsyndromic, and within this group, some families exhibit a pattern of recurrence in the absence of identifiable syndromic features [1-3]. In particular, repeated bilateral cleft lip and palate within a single family is uncommon. Although such patterns are clinically notable, the underlying pathophysiology remains incompletely understood.
Mucoperiosteal flap necrosis is a rare but serious complication of palatoplasty, potentially leading to oronasal fistula (ONF), delayed healing, and long-term functional sequelae [4]. The risk is increased in patients with wide palatal defects or unfavorable anatomical features, yet such risks are difficult to predict or entirely avoid preoperatively.
Of 780 primary palatoplasties performed at our institution, including 33 in patients with bilateral cleft palate only two cases of mucoperiosteal flap necrosis were observed. Remarkably, both occurred in siblings, operated on 3 years apart, with strikingly similar timing and pattern of necrosis. Both underwent appropriate secondary procedures, leading to uneventful recov-eries and successful secondary alveolar bone grafting.
We investigated potential causes of this rare flap necrosis, but no definitive explanation was identified. Nevertheless, the identical timing and pattern of necrosis in both cases raise the possibility of a shared anatomical or genetic predisposition. This report details the surgical approach, the development of flap necrosis, and subsequent management in these siblings. By documenting this rare occurrence, we aim to contribute to the literature and provide a reference for future cases with similar presentations.
CASE REPORTS
Case 1
The first patient was a female infant born at 36 weeks and 2 days of gestation by cesarean section, with a birth weight of 2.29 kg. She was diagnosed at birth with complete bilateral cleft lip and palate. Her mother had a history of unilateral complete cleft lip, without syndromic features, and reported no systemic illness, medication use, or teratogenic exposure during pregnancy. Primary palatoplasty using a two-flap technique with a vomer flap was performed at 12 months of age. The surgery was uneventful, yet mucoperiosteal flap necrosis developed on postoperative day (POD) 5, necessitating urgent surgical debridement of necrotic tissue (Fig. 1). On POD 15, wound dehiscence was observed at the junction of the vomer flap and nasal mucosa, requiring further debridement and repair (Fig. 2). An ONF subsequently developed, though no feeding difficulties were observed. The patient was closely monitored in the outpatient clinic for speech-related issues, but no signs of velopharyngeal insufficiency (VPI) were noted. Five years later, the fistula was repaired using bilateral Veau flaps without further complications (Fig. 3).
Case 2
The second patient, the younger sibling of Case 1, was a male infant born at 37 weeks and 5 days of gestation via cesarean section, weighing 3.18 kg. He was likewise diagnosed with complete bilateral cleft lip and palate. The mother’s history was again notable for unilateral complete cleft lip, with no reported prenatal illness or teratogen exposure. Primary palatoplasty, using the same two-flap technique with a vomer flap, was performed at 13 months of age. In light of the prior flap necrosis, special care was taken during surgery, which ensured that the flap was under appropriate tension and no intraoperative complications occurred. Despite these precautions, flap necrosis appeared on POD 5 and was managed conservatively with secondary intention healing (Fig. 4). An ONF developed postoperatively, but the patient experienced no feeding or speech difficulties. Three years later, the fistula was repaired using a turnover hinge flap in combination with bilateral Veau flaps, achieving a favorable outcome without significant complications (Fig. 5).
DISCUSSION
The genetic basis of cleft palate has been extensively studied. CL/P is generally considered multifactorial, with concordance rates of 40% to 60% among monozygotic twins, underscoring a strong hereditary component [1]. Specific genetic variants, such as polymorphisms in the IRF6 gene, are linked to an increased risk of nonsyndromic CL/P at the single-nucleotide level [5]. However, even among family members with the same mutations, phenotypic expression can vary—including cleft lip, cleft palate, or tooth agenesis—reflecting the complexity and incomplete penetrance of familial nonsyndromic CL/P [6].
In our report, both siblings were born to a mother with unilateral cleft lip and diagnosed with bilateral CL/P. Although genetic testing was not performed, a genetic predisposition likely contributed to their condition. Both children underwent primary palatoplasty using a two-flap technique with a vomer flap; intraoperative findings were unremarkable, with no evidence of pedicle injury or excessive flap tension. Nevertheless, both developed anterior mucoperiosteal flap necrosis on POD 5.
The mucoperiosteum of the hard palate receives its principal blood supply from the greater palatine artery, which exits the greater palatine foramen and courses anteriorly along the lateral palate [7]. In patients with cleft palate, supplementary vascular input arises from small arterial branches traversing from the nasal mucosa medially and toward the alveolus laterally [8]. Extensive anastomoses exist between the palatal and nasal vascu-lar networks, often occurring at the bony margins or via transosseous channels [9]. These branches are at risk of disruption during the incisions made for palatoplasty and during subperiosteal dissection.
Given this anatomical vulnerability, mucoperiosteal flap necrosis— though rare—is a serious early complication following palatoplasty that may lead to significant morbidity if not managed appropriately [10]. Its etiology is multifactorial and may involve injury to the greater palatine artery, excessive flap tension, infection or hematoma [11]. Veau type IV clefts, which are associated with wider clefts, carry a higher risk due to greater mechanical stress on the flaps [12]. Rossell-Perry et al. [13] also identified bilateral cleft palate as a risk factor, suggesting a strong relationship between anatomical complexity and the flap’s capacity to withstand vascular compromise.
Mitigating these risks requires meticulous surgical technique, including preservation of the vascular pedicle and minimizing tension throughout the procedure [14]. Techniques such as pyramidal space dissection or hamulus fracture can help decompress the greater palatine neurovascular bundle [15], and auxiliary procedures such as buccal fat pad flaps or relaxing incisions may further reduce tension [16,17]. These methods are critical for maintaining perfusion and preventing ischemia.
At our institution, these tension-reducing techniques are routinely implemented. In addition, hand-tying of sutures is performed during closure to assess mucoperiosteal flap tension, which may contribute to reducing the risk of flap necrosis. Notably, flap necrosis has been exceedingly rare in our experience. In this report, despite additional precautions during the younger sibling’s surgery, flap necrosis occurred at the same postoperative interval. These observations raise the possibility that anatomical variations potentially linked to genetic predisposition may have contributed to the development of flap necrosis, likely in combination with other factors such as increased flap tension.
After flap necrosis, one patient required debridement, while the other was managed by secondary intention. Both developed ONF, which did not interfere with feeding, and neither showed VPI during long-term follow-up. Fistula repairs were performed 5 and 3 years postoperatively using local flaps, with satisfactory healing and no recurrence.
ONF management depends on size, location, and clinical symptoms, and not all cases require immediate surgical intervention [12]. As previous literature suggests, asymptomatic, functionally stable cases can be monitored, while the presence of VPI may warrant repair regardless of age [18]. Depending on fistula characteristics, surgical options may range from local flaps to free flaps [19,20]. In this report, delayed repair was chosen due to clinical stability, and surgical results were favorable. These findings support a symptom-driven approach to ONF management and highlight the importance of individualized timing based on patient-specific considerations.
A limitation of this report is the absence of both preoperative computed tomography angiography to assess vascular variability and intraoperative indocyanine green angiography to evaluate flap viability. However, this case series presents an exceptionally rare occurrence of anterior palatal flap necrosis developing identically on POD 5 in two siblings with nonsyndromic cleft lip and palate. Based on this finding, we propose that in such patients, perioperative vascular imaging and intraoperative perfusion assessment, should be strongly considered and particular care should be taken during surgery. In conclusion, this report underscores the potential impact of anatomical and genetic predisposition on surgical complications and may provide insights into the pathogenesis and tailored management of such cases.
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