# Management of Congenital Palatal Fistula Associated With Wiskott-Aldrich Syndrome

## TL;DR

This paper presents a rare case of a child with both submucous cleft palate and Wiskott-Aldrich syndrome, highlighting the need for careful, multidisciplinary management.

## Contribution

The paper reports a unique case combining two rare conditions and emphasizes the importance of coordinated care.

## Key findings

- A 19-month-old boy with Wiskott-Aldrich syndrome and submucous cleft palate was successfully managed with HSCT and surgical repair.
- Multidisciplinary care led to successful postoperative healing without complications.
- The case underscores the importance of early diagnosis and tailored treatment for rare co-occurring conditions.

## Abstract

Submucous cleft palate (SMCP) is a rare form of cleft palate involving abnormal muscle development in the soft palate. Diagnosis is often delayed, with many cases identified after age 4. Symptoms like hypernasality and velopharyngeal insufficiency (VPI) may appear, though some remain asymptomatic. SMCP diagnosis typically relies on Calnan’s triad: bifid uvula, hard palate notch, and zona pellucida. Wiskott-Aldrich syndrome (WAS) is a rare X-linked immune disorder marked by thrombocytopenia, eczema, and infections. Early diagnosis is key to effective treatment. Due to the rarity of both SMCP and WAS, we present a unique case and suggest careful management for optimal outcomes. A one-year and 10-month-old boy with WAS was referred to our cleft center for evaluation of a congenital palatal fistula. There was no history of trauma or surgery, but a 9 × 7 mm midline fistula was observed, along with a diagnosis of SMCP. After hematopoietic stem cell transplantation (HSCT) and continued immunosuppressive therapy, a palatal plate was created to assist feeding. At age 5, a cleft palate repair using a modified Bardach technique was performed. Postoperative healing was successful without complications. This case highlights multidisciplinary management in a patient with both SMCP and WAS.